introduction to interstitial lung disease(ild) or diffuse parenchymal lung disease (dpld)
TRANSCRIPT
DR MUHAMMED ASLAMMBBS MD PULMONARY MEDICINEfacebookcommedicalppt
Interstitial Lung Disease(ILD) or
Diffuse Parenchymal Lung Disease (DPLD)
OUTLINE
bull Introduction
bull Classification
bull Epidemiology
bull Pathogenesis
bull Clinical features
bull Investigation
bull Treatment
INTRODUCTION
bull Interstitial Lung Disease refers to a broad range of conditions that have common clinical physiological and radiological features
bull ILD is not one disease but several diseases that do not necessarily share a common histopathological or pathophysiological basis
INTRODUCTIONbull By strict definition Interstitial lung disease involves
abnormalities of the interstitium ndash ldquothe potential space between the epithelium and capillary endothelial basement membrane within the alveolusrdquo HOWEVERhelliphelliphelliphellip
INTRODUCTION
bull Interstitial is a misleading terminology because most of these disorders are associated with extensive alteration of airway and alveolar architecture in addition to changes in interstitial compartment
bull For this reason Diffuse Parenchymal Lung Disease or DPLD is the better term
CLASSIFICATION OF DPLD
bull DPLD Two large groups
(1)Idiopathic DPLD ( no known cause )
(2) Those with identifiable cause or occurs secondary to other diseases
IDIOPATHIC ILD
1Idiopathic pulmonary fibrosis (IPF) ndash most common2Acute interstitial pneumonia (AIP)3Cryptogenic Organizing Pneumonia ( COP) Bronchiolitis
obliterans organizing pneumonia (BOOP)4Desquamative interstitial pneumonia (DIP)5Lymphocytic interstitial Pneumonia ( LIP)6Non specific interstitial Pneumonia (NSIP)7Respiratory bronchiolitis associated Interstitial lung disease
(RBILD)
THOSE WITH IDENTIFIABLE CAUSE
1Autoimmune- rheumatoid arthritis SLE Sarcoidosis Scleroderma
2Certain infections3Medications4Radiation5Occupational exposure asbestos coal cotton silica
CLASSIFICATION
EPIDEMIOLOGY
bull Incidence ranges from 3-26100000 per yearbull Prevalence of preclinical and undiagnosed ILD is
estimated to be 10 times that of clinical recognized disease
bull IPF is the most common form representing at least 30 percent of the incident cases
IPF - PATHOGENESIS VIDEO
NORMAL DPLD
RESPIRATORY SYMPTOMS Breathlessness (most common) Initially dyspnea on exertionrarr later at rest Nonproductive cough
Pleuritic chest pain Wheeing Hemoptysis
NON RESPIRATORY SYMPTOMS ASSOCIATED WITH DIFFERENT DPLDS
bull Arthritisbull Ocular bull Skin and musclebull GERD bull Lower GI symptoms bull Recurrent sinusitis
bull Neurological symptoms bull Epilepsy amp mental retardation bull Diabetes inspidus
ONSET OF SYMPTOMS
1 Acute presentation (days to weeks)- eg Acute idiopathic interstitial pneumoniHypersensitive pneumonitis
2 Sub-acute presentation (weeks to months)- eg SarcoidosisDrug induced ILD Alveolar hemorrhage syndromes
3 Chronic presentation (months to years) ndash eg IPF
ILD-HISTORY
bullSmokingbullMedication history- Amiodorane Methotrexate
bullOccupational historybullEnvironmental exposure history
OCCUPATIONAL HISTORY1048766 Pneumoconioses ndash miners1048766 Silicosis ndash sand blasters amp granite workers1048766 Asbestosis ndash welders electricians mechanics workers withbrakes shipyard workers1048766 Berylliosis ndash aerospace nuclear computer amp electronic
industries1048766 Hypersensitive pneumonitis ndash farm workers poultry workersbird breeders1048766 The degree of exposure duration latency of exposure and
theuse of protective devices should be elicited
ENVIRONMENTAL EXPOSURE HISTORY
bull 1048766 Exposures to pets (especially birds)bull 1048766 Air conditionersbull 1048766 Humidifiersbull 1048766 Hot tubsbull 1048766 Evaporative cooling systems
ILD-SIGNS
bull Crackles - Dry velcro end inspiratory predominantly bibasilar
bull Inspiratory squeaks- Mid inspiratory high pitched- Seen in Airway centred pathologies
bull Cor pulmonale featuresbull Clubbing ndash IPF
EXTRA PULMONARY -SIGNS
bull Skin abnormalitiesbull Lymphadenopathybull Hepatosplenomegalybull Maculopapular skin rashes bull Erythema nodosum
bull Subcutaneous nodules bull Proximal muscle
weakness bull Arthritis
INVESTIGATIONS
CHEST X RAY
bull Typically small lung volumes with Reticular Nodular or RETICULONODULAR shadow
HIGH-RESOLUTION COMPUTED TOPOGRAPHY (HRCT)
bull HRCT is more sensitivebull Combinations of ground
glass changes reticulonodular shadowing honeycomb cysts and traction
bronchiectasis
PULMONARY FUNCTION TESTING
bull Objective assessment of Resp Symptoms
bull Grading the severity
bull Monitoring the response to therapy
PULMONARY FUNCTION TESTING
bull Restrictive Defect bull darr Lung volumes (TLC FRCRV lt80)bull darr FEV1 FVC With Normal or uarrFEV1FVC
bull Reduced diffusing capacity (DLCO)
LABORATORY INVESTIGATIONS
bull Full blood count lymphopenia in sarcoid eosinophilia in pulmonary eosinophilias and drug reactions neutrophilia in hypersensitivity pneumonitis
bull Ca2+ may be elevated in sarcoidbull Serum angiotensin-converting enzyme non-specific indicator of disease
activity in sarcoidbull ESR and CRP non-specifically raisedbull Autoimmune screen autoantibodies may suggest connectivetissue disease
OTHER INVESTIGATIONS
bull ARTERIAL BLOOD GAS ANALYSIS bull Tuberculin test ndash negative in 23 of sarcoidosis patientsbull BRONCHOALVEOLAR LAVAGE- cellular profile special
stains or studiesbull Lung biopsy- TRANS BRONCHIAL BIOPSY OPEN LUNG
BIOPSY
MANEGEMENTPRINCIPAL AIMS (1) to remove exposure to injurious agents
(2) to suppress inflammation to prevent further destruction of the pulmonary parenchyma
(3) to palliate the manifestations of these diseases
CORTICOSTERIODS
bull Prednisone 1 mgkgbull Gradually tapered (5 mgweek) over several months
to a maintenance dose
IMMUNO SUPRISSIVE AGENTS
Cytotoxic agents (Cyclophosphamide)or immunosuppressive agents (Azathioprine) may be used in patients who do not improve on steroid therapy or who cannot tolerate corticosteroids
N-ACETYL CYSTEIN
bullAntioxidant
bull600 mg PO tid added to prednisone and azathioprine preserves vital capacity and FVC and DLCO
PIRFENIDONE
bullAntifibroticbullReduces acute exacerbations and reduction in FVC
OXYGEN THERAPY
bull F or pa tients w i th d o c u m e nted h y p ox i a ndash S p O 2 lt89 P a O 2 lt55m m H g
bull Improves exerc i se to le rance
CONCLUSION
bull DPLD is the better term not a single disease bull IPF is the most common DPLDbull Restrictive Airway Diseasebull Main treatment aim is to suppress inflammation to prevent
further destruction of the pulmonary parenchyma
Thank you
- Outline
- introduction
- Introduction
- Introduction (2)
- Classification of dpld
- Idiopathic ILD
- Those with identifiable cause
- classification
- Epidemiology
- Ipf - PATHOgenesis VIDEO
- Slide 12
- Respiratory SYMPTOMS
- Non Respiratory symptoms associated with different dplds
- ONSET OF SYMPTOMS
- ILD-HISTORY
- Occupational history
- Environmental exposure history
- ILD-SIGNS
- Extra Pulmonary -SIGNS
- INVESTIGATIONS
- Chest X Ray
- High-resolution Computed topography (HRCT)
- PULMONARY FUNCTION TESTING
- PULMONARY FUNCTION TESTING (2)
- Laboratory investigations
- OTHER INVESTIGATIONS
- Slide 28
- MANEGEMENT
- CORTICOSTERIODS
- IMMUNO SUPRISSIVE AGENTS
- N-acetyl cystein
- Pirfenidone
- Oxygen therapy
- Conclusion
- Slide 36
-
OUTLINE
bull Introduction
bull Classification
bull Epidemiology
bull Pathogenesis
bull Clinical features
bull Investigation
bull Treatment
INTRODUCTION
bull Interstitial Lung Disease refers to a broad range of conditions that have common clinical physiological and radiological features
bull ILD is not one disease but several diseases that do not necessarily share a common histopathological or pathophysiological basis
INTRODUCTIONbull By strict definition Interstitial lung disease involves
abnormalities of the interstitium ndash ldquothe potential space between the epithelium and capillary endothelial basement membrane within the alveolusrdquo HOWEVERhelliphelliphelliphellip
INTRODUCTION
bull Interstitial is a misleading terminology because most of these disorders are associated with extensive alteration of airway and alveolar architecture in addition to changes in interstitial compartment
bull For this reason Diffuse Parenchymal Lung Disease or DPLD is the better term
CLASSIFICATION OF DPLD
bull DPLD Two large groups
(1)Idiopathic DPLD ( no known cause )
(2) Those with identifiable cause or occurs secondary to other diseases
IDIOPATHIC ILD
1Idiopathic pulmonary fibrosis (IPF) ndash most common2Acute interstitial pneumonia (AIP)3Cryptogenic Organizing Pneumonia ( COP) Bronchiolitis
obliterans organizing pneumonia (BOOP)4Desquamative interstitial pneumonia (DIP)5Lymphocytic interstitial Pneumonia ( LIP)6Non specific interstitial Pneumonia (NSIP)7Respiratory bronchiolitis associated Interstitial lung disease
(RBILD)
THOSE WITH IDENTIFIABLE CAUSE
1Autoimmune- rheumatoid arthritis SLE Sarcoidosis Scleroderma
2Certain infections3Medications4Radiation5Occupational exposure asbestos coal cotton silica
CLASSIFICATION
EPIDEMIOLOGY
bull Incidence ranges from 3-26100000 per yearbull Prevalence of preclinical and undiagnosed ILD is
estimated to be 10 times that of clinical recognized disease
bull IPF is the most common form representing at least 30 percent of the incident cases
IPF - PATHOGENESIS VIDEO
NORMAL DPLD
RESPIRATORY SYMPTOMS Breathlessness (most common) Initially dyspnea on exertionrarr later at rest Nonproductive cough
Pleuritic chest pain Wheeing Hemoptysis
NON RESPIRATORY SYMPTOMS ASSOCIATED WITH DIFFERENT DPLDS
bull Arthritisbull Ocular bull Skin and musclebull GERD bull Lower GI symptoms bull Recurrent sinusitis
bull Neurological symptoms bull Epilepsy amp mental retardation bull Diabetes inspidus
ONSET OF SYMPTOMS
1 Acute presentation (days to weeks)- eg Acute idiopathic interstitial pneumoniHypersensitive pneumonitis
2 Sub-acute presentation (weeks to months)- eg SarcoidosisDrug induced ILD Alveolar hemorrhage syndromes
3 Chronic presentation (months to years) ndash eg IPF
ILD-HISTORY
bullSmokingbullMedication history- Amiodorane Methotrexate
bullOccupational historybullEnvironmental exposure history
OCCUPATIONAL HISTORY1048766 Pneumoconioses ndash miners1048766 Silicosis ndash sand blasters amp granite workers1048766 Asbestosis ndash welders electricians mechanics workers withbrakes shipyard workers1048766 Berylliosis ndash aerospace nuclear computer amp electronic
industries1048766 Hypersensitive pneumonitis ndash farm workers poultry workersbird breeders1048766 The degree of exposure duration latency of exposure and
theuse of protective devices should be elicited
ENVIRONMENTAL EXPOSURE HISTORY
bull 1048766 Exposures to pets (especially birds)bull 1048766 Air conditionersbull 1048766 Humidifiersbull 1048766 Hot tubsbull 1048766 Evaporative cooling systems
ILD-SIGNS
bull Crackles - Dry velcro end inspiratory predominantly bibasilar
bull Inspiratory squeaks- Mid inspiratory high pitched- Seen in Airway centred pathologies
bull Cor pulmonale featuresbull Clubbing ndash IPF
EXTRA PULMONARY -SIGNS
bull Skin abnormalitiesbull Lymphadenopathybull Hepatosplenomegalybull Maculopapular skin rashes bull Erythema nodosum
bull Subcutaneous nodules bull Proximal muscle
weakness bull Arthritis
INVESTIGATIONS
CHEST X RAY
bull Typically small lung volumes with Reticular Nodular or RETICULONODULAR shadow
HIGH-RESOLUTION COMPUTED TOPOGRAPHY (HRCT)
bull HRCT is more sensitivebull Combinations of ground
glass changes reticulonodular shadowing honeycomb cysts and traction
bronchiectasis
PULMONARY FUNCTION TESTING
bull Objective assessment of Resp Symptoms
bull Grading the severity
bull Monitoring the response to therapy
PULMONARY FUNCTION TESTING
bull Restrictive Defect bull darr Lung volumes (TLC FRCRV lt80)bull darr FEV1 FVC With Normal or uarrFEV1FVC
bull Reduced diffusing capacity (DLCO)
LABORATORY INVESTIGATIONS
bull Full blood count lymphopenia in sarcoid eosinophilia in pulmonary eosinophilias and drug reactions neutrophilia in hypersensitivity pneumonitis
bull Ca2+ may be elevated in sarcoidbull Serum angiotensin-converting enzyme non-specific indicator of disease
activity in sarcoidbull ESR and CRP non-specifically raisedbull Autoimmune screen autoantibodies may suggest connectivetissue disease
OTHER INVESTIGATIONS
bull ARTERIAL BLOOD GAS ANALYSIS bull Tuberculin test ndash negative in 23 of sarcoidosis patientsbull BRONCHOALVEOLAR LAVAGE- cellular profile special
stains or studiesbull Lung biopsy- TRANS BRONCHIAL BIOPSY OPEN LUNG
BIOPSY
MANEGEMENTPRINCIPAL AIMS (1) to remove exposure to injurious agents
(2) to suppress inflammation to prevent further destruction of the pulmonary parenchyma
(3) to palliate the manifestations of these diseases
CORTICOSTERIODS
bull Prednisone 1 mgkgbull Gradually tapered (5 mgweek) over several months
to a maintenance dose
IMMUNO SUPRISSIVE AGENTS
Cytotoxic agents (Cyclophosphamide)or immunosuppressive agents (Azathioprine) may be used in patients who do not improve on steroid therapy or who cannot tolerate corticosteroids
N-ACETYL CYSTEIN
bullAntioxidant
bull600 mg PO tid added to prednisone and azathioprine preserves vital capacity and FVC and DLCO
PIRFENIDONE
bullAntifibroticbullReduces acute exacerbations and reduction in FVC
OXYGEN THERAPY
bull F or pa tients w i th d o c u m e nted h y p ox i a ndash S p O 2 lt89 P a O 2 lt55m m H g
bull Improves exerc i se to le rance
CONCLUSION
bull DPLD is the better term not a single disease bull IPF is the most common DPLDbull Restrictive Airway Diseasebull Main treatment aim is to suppress inflammation to prevent
further destruction of the pulmonary parenchyma
Thank you
- Outline
- introduction
- Introduction
- Introduction (2)
- Classification of dpld
- Idiopathic ILD
- Those with identifiable cause
- classification
- Epidemiology
- Ipf - PATHOgenesis VIDEO
- Slide 12
- Respiratory SYMPTOMS
- Non Respiratory symptoms associated with different dplds
- ONSET OF SYMPTOMS
- ILD-HISTORY
- Occupational history
- Environmental exposure history
- ILD-SIGNS
- Extra Pulmonary -SIGNS
- INVESTIGATIONS
- Chest X Ray
- High-resolution Computed topography (HRCT)
- PULMONARY FUNCTION TESTING
- PULMONARY FUNCTION TESTING (2)
- Laboratory investigations
- OTHER INVESTIGATIONS
- Slide 28
- MANEGEMENT
- CORTICOSTERIODS
- IMMUNO SUPRISSIVE AGENTS
- N-acetyl cystein
- Pirfenidone
- Oxygen therapy
- Conclusion
- Slide 36
-
INTRODUCTION
bull Interstitial Lung Disease refers to a broad range of conditions that have common clinical physiological and radiological features
bull ILD is not one disease but several diseases that do not necessarily share a common histopathological or pathophysiological basis
INTRODUCTIONbull By strict definition Interstitial lung disease involves
abnormalities of the interstitium ndash ldquothe potential space between the epithelium and capillary endothelial basement membrane within the alveolusrdquo HOWEVERhelliphelliphelliphellip
INTRODUCTION
bull Interstitial is a misleading terminology because most of these disorders are associated with extensive alteration of airway and alveolar architecture in addition to changes in interstitial compartment
bull For this reason Diffuse Parenchymal Lung Disease or DPLD is the better term
CLASSIFICATION OF DPLD
bull DPLD Two large groups
(1)Idiopathic DPLD ( no known cause )
(2) Those with identifiable cause or occurs secondary to other diseases
IDIOPATHIC ILD
1Idiopathic pulmonary fibrosis (IPF) ndash most common2Acute interstitial pneumonia (AIP)3Cryptogenic Organizing Pneumonia ( COP) Bronchiolitis
obliterans organizing pneumonia (BOOP)4Desquamative interstitial pneumonia (DIP)5Lymphocytic interstitial Pneumonia ( LIP)6Non specific interstitial Pneumonia (NSIP)7Respiratory bronchiolitis associated Interstitial lung disease
(RBILD)
THOSE WITH IDENTIFIABLE CAUSE
1Autoimmune- rheumatoid arthritis SLE Sarcoidosis Scleroderma
2Certain infections3Medications4Radiation5Occupational exposure asbestos coal cotton silica
CLASSIFICATION
EPIDEMIOLOGY
bull Incidence ranges from 3-26100000 per yearbull Prevalence of preclinical and undiagnosed ILD is
estimated to be 10 times that of clinical recognized disease
bull IPF is the most common form representing at least 30 percent of the incident cases
IPF - PATHOGENESIS VIDEO
NORMAL DPLD
RESPIRATORY SYMPTOMS Breathlessness (most common) Initially dyspnea on exertionrarr later at rest Nonproductive cough
Pleuritic chest pain Wheeing Hemoptysis
NON RESPIRATORY SYMPTOMS ASSOCIATED WITH DIFFERENT DPLDS
bull Arthritisbull Ocular bull Skin and musclebull GERD bull Lower GI symptoms bull Recurrent sinusitis
bull Neurological symptoms bull Epilepsy amp mental retardation bull Diabetes inspidus
ONSET OF SYMPTOMS
1 Acute presentation (days to weeks)- eg Acute idiopathic interstitial pneumoniHypersensitive pneumonitis
2 Sub-acute presentation (weeks to months)- eg SarcoidosisDrug induced ILD Alveolar hemorrhage syndromes
3 Chronic presentation (months to years) ndash eg IPF
ILD-HISTORY
bullSmokingbullMedication history- Amiodorane Methotrexate
bullOccupational historybullEnvironmental exposure history
OCCUPATIONAL HISTORY1048766 Pneumoconioses ndash miners1048766 Silicosis ndash sand blasters amp granite workers1048766 Asbestosis ndash welders electricians mechanics workers withbrakes shipyard workers1048766 Berylliosis ndash aerospace nuclear computer amp electronic
industries1048766 Hypersensitive pneumonitis ndash farm workers poultry workersbird breeders1048766 The degree of exposure duration latency of exposure and
theuse of protective devices should be elicited
ENVIRONMENTAL EXPOSURE HISTORY
bull 1048766 Exposures to pets (especially birds)bull 1048766 Air conditionersbull 1048766 Humidifiersbull 1048766 Hot tubsbull 1048766 Evaporative cooling systems
ILD-SIGNS
bull Crackles - Dry velcro end inspiratory predominantly bibasilar
bull Inspiratory squeaks- Mid inspiratory high pitched- Seen in Airway centred pathologies
bull Cor pulmonale featuresbull Clubbing ndash IPF
EXTRA PULMONARY -SIGNS
bull Skin abnormalitiesbull Lymphadenopathybull Hepatosplenomegalybull Maculopapular skin rashes bull Erythema nodosum
bull Subcutaneous nodules bull Proximal muscle
weakness bull Arthritis
INVESTIGATIONS
CHEST X RAY
bull Typically small lung volumes with Reticular Nodular or RETICULONODULAR shadow
HIGH-RESOLUTION COMPUTED TOPOGRAPHY (HRCT)
bull HRCT is more sensitivebull Combinations of ground
glass changes reticulonodular shadowing honeycomb cysts and traction
bronchiectasis
PULMONARY FUNCTION TESTING
bull Objective assessment of Resp Symptoms
bull Grading the severity
bull Monitoring the response to therapy
PULMONARY FUNCTION TESTING
bull Restrictive Defect bull darr Lung volumes (TLC FRCRV lt80)bull darr FEV1 FVC With Normal or uarrFEV1FVC
bull Reduced diffusing capacity (DLCO)
LABORATORY INVESTIGATIONS
bull Full blood count lymphopenia in sarcoid eosinophilia in pulmonary eosinophilias and drug reactions neutrophilia in hypersensitivity pneumonitis
bull Ca2+ may be elevated in sarcoidbull Serum angiotensin-converting enzyme non-specific indicator of disease
activity in sarcoidbull ESR and CRP non-specifically raisedbull Autoimmune screen autoantibodies may suggest connectivetissue disease
OTHER INVESTIGATIONS
bull ARTERIAL BLOOD GAS ANALYSIS bull Tuberculin test ndash negative in 23 of sarcoidosis patientsbull BRONCHOALVEOLAR LAVAGE- cellular profile special
stains or studiesbull Lung biopsy- TRANS BRONCHIAL BIOPSY OPEN LUNG
BIOPSY
MANEGEMENTPRINCIPAL AIMS (1) to remove exposure to injurious agents
(2) to suppress inflammation to prevent further destruction of the pulmonary parenchyma
(3) to palliate the manifestations of these diseases
CORTICOSTERIODS
bull Prednisone 1 mgkgbull Gradually tapered (5 mgweek) over several months
to a maintenance dose
IMMUNO SUPRISSIVE AGENTS
Cytotoxic agents (Cyclophosphamide)or immunosuppressive agents (Azathioprine) may be used in patients who do not improve on steroid therapy or who cannot tolerate corticosteroids
N-ACETYL CYSTEIN
bullAntioxidant
bull600 mg PO tid added to prednisone and azathioprine preserves vital capacity and FVC and DLCO
PIRFENIDONE
bullAntifibroticbullReduces acute exacerbations and reduction in FVC
OXYGEN THERAPY
bull F or pa tients w i th d o c u m e nted h y p ox i a ndash S p O 2 lt89 P a O 2 lt55m m H g
bull Improves exerc i se to le rance
CONCLUSION
bull DPLD is the better term not a single disease bull IPF is the most common DPLDbull Restrictive Airway Diseasebull Main treatment aim is to suppress inflammation to prevent
further destruction of the pulmonary parenchyma
Thank you
- Outline
- introduction
- Introduction
- Introduction (2)
- Classification of dpld
- Idiopathic ILD
- Those with identifiable cause
- classification
- Epidemiology
- Ipf - PATHOgenesis VIDEO
- Slide 12
- Respiratory SYMPTOMS
- Non Respiratory symptoms associated with different dplds
- ONSET OF SYMPTOMS
- ILD-HISTORY
- Occupational history
- Environmental exposure history
- ILD-SIGNS
- Extra Pulmonary -SIGNS
- INVESTIGATIONS
- Chest X Ray
- High-resolution Computed topography (HRCT)
- PULMONARY FUNCTION TESTING
- PULMONARY FUNCTION TESTING (2)
- Laboratory investigations
- OTHER INVESTIGATIONS
- Slide 28
- MANEGEMENT
- CORTICOSTERIODS
- IMMUNO SUPRISSIVE AGENTS
- N-acetyl cystein
- Pirfenidone
- Oxygen therapy
- Conclusion
- Slide 36
-
INTRODUCTIONbull By strict definition Interstitial lung disease involves
abnormalities of the interstitium ndash ldquothe potential space between the epithelium and capillary endothelial basement membrane within the alveolusrdquo HOWEVERhelliphelliphelliphellip
INTRODUCTION
bull Interstitial is a misleading terminology because most of these disorders are associated with extensive alteration of airway and alveolar architecture in addition to changes in interstitial compartment
bull For this reason Diffuse Parenchymal Lung Disease or DPLD is the better term
CLASSIFICATION OF DPLD
bull DPLD Two large groups
(1)Idiopathic DPLD ( no known cause )
(2) Those with identifiable cause or occurs secondary to other diseases
IDIOPATHIC ILD
1Idiopathic pulmonary fibrosis (IPF) ndash most common2Acute interstitial pneumonia (AIP)3Cryptogenic Organizing Pneumonia ( COP) Bronchiolitis
obliterans organizing pneumonia (BOOP)4Desquamative interstitial pneumonia (DIP)5Lymphocytic interstitial Pneumonia ( LIP)6Non specific interstitial Pneumonia (NSIP)7Respiratory bronchiolitis associated Interstitial lung disease
(RBILD)
THOSE WITH IDENTIFIABLE CAUSE
1Autoimmune- rheumatoid arthritis SLE Sarcoidosis Scleroderma
2Certain infections3Medications4Radiation5Occupational exposure asbestos coal cotton silica
CLASSIFICATION
EPIDEMIOLOGY
bull Incidence ranges from 3-26100000 per yearbull Prevalence of preclinical and undiagnosed ILD is
estimated to be 10 times that of clinical recognized disease
bull IPF is the most common form representing at least 30 percent of the incident cases
IPF - PATHOGENESIS VIDEO
NORMAL DPLD
RESPIRATORY SYMPTOMS Breathlessness (most common) Initially dyspnea on exertionrarr later at rest Nonproductive cough
Pleuritic chest pain Wheeing Hemoptysis
NON RESPIRATORY SYMPTOMS ASSOCIATED WITH DIFFERENT DPLDS
bull Arthritisbull Ocular bull Skin and musclebull GERD bull Lower GI symptoms bull Recurrent sinusitis
bull Neurological symptoms bull Epilepsy amp mental retardation bull Diabetes inspidus
ONSET OF SYMPTOMS
1 Acute presentation (days to weeks)- eg Acute idiopathic interstitial pneumoniHypersensitive pneumonitis
2 Sub-acute presentation (weeks to months)- eg SarcoidosisDrug induced ILD Alveolar hemorrhage syndromes
3 Chronic presentation (months to years) ndash eg IPF
ILD-HISTORY
bullSmokingbullMedication history- Amiodorane Methotrexate
bullOccupational historybullEnvironmental exposure history
OCCUPATIONAL HISTORY1048766 Pneumoconioses ndash miners1048766 Silicosis ndash sand blasters amp granite workers1048766 Asbestosis ndash welders electricians mechanics workers withbrakes shipyard workers1048766 Berylliosis ndash aerospace nuclear computer amp electronic
industries1048766 Hypersensitive pneumonitis ndash farm workers poultry workersbird breeders1048766 The degree of exposure duration latency of exposure and
theuse of protective devices should be elicited
ENVIRONMENTAL EXPOSURE HISTORY
bull 1048766 Exposures to pets (especially birds)bull 1048766 Air conditionersbull 1048766 Humidifiersbull 1048766 Hot tubsbull 1048766 Evaporative cooling systems
ILD-SIGNS
bull Crackles - Dry velcro end inspiratory predominantly bibasilar
bull Inspiratory squeaks- Mid inspiratory high pitched- Seen in Airway centred pathologies
bull Cor pulmonale featuresbull Clubbing ndash IPF
EXTRA PULMONARY -SIGNS
bull Skin abnormalitiesbull Lymphadenopathybull Hepatosplenomegalybull Maculopapular skin rashes bull Erythema nodosum
bull Subcutaneous nodules bull Proximal muscle
weakness bull Arthritis
INVESTIGATIONS
CHEST X RAY
bull Typically small lung volumes with Reticular Nodular or RETICULONODULAR shadow
HIGH-RESOLUTION COMPUTED TOPOGRAPHY (HRCT)
bull HRCT is more sensitivebull Combinations of ground
glass changes reticulonodular shadowing honeycomb cysts and traction
bronchiectasis
PULMONARY FUNCTION TESTING
bull Objective assessment of Resp Symptoms
bull Grading the severity
bull Monitoring the response to therapy
PULMONARY FUNCTION TESTING
bull Restrictive Defect bull darr Lung volumes (TLC FRCRV lt80)bull darr FEV1 FVC With Normal or uarrFEV1FVC
bull Reduced diffusing capacity (DLCO)
LABORATORY INVESTIGATIONS
bull Full blood count lymphopenia in sarcoid eosinophilia in pulmonary eosinophilias and drug reactions neutrophilia in hypersensitivity pneumonitis
bull Ca2+ may be elevated in sarcoidbull Serum angiotensin-converting enzyme non-specific indicator of disease
activity in sarcoidbull ESR and CRP non-specifically raisedbull Autoimmune screen autoantibodies may suggest connectivetissue disease
OTHER INVESTIGATIONS
bull ARTERIAL BLOOD GAS ANALYSIS bull Tuberculin test ndash negative in 23 of sarcoidosis patientsbull BRONCHOALVEOLAR LAVAGE- cellular profile special
stains or studiesbull Lung biopsy- TRANS BRONCHIAL BIOPSY OPEN LUNG
BIOPSY
MANEGEMENTPRINCIPAL AIMS (1) to remove exposure to injurious agents
(2) to suppress inflammation to prevent further destruction of the pulmonary parenchyma
(3) to palliate the manifestations of these diseases
CORTICOSTERIODS
bull Prednisone 1 mgkgbull Gradually tapered (5 mgweek) over several months
to a maintenance dose
IMMUNO SUPRISSIVE AGENTS
Cytotoxic agents (Cyclophosphamide)or immunosuppressive agents (Azathioprine) may be used in patients who do not improve on steroid therapy or who cannot tolerate corticosteroids
N-ACETYL CYSTEIN
bullAntioxidant
bull600 mg PO tid added to prednisone and azathioprine preserves vital capacity and FVC and DLCO
PIRFENIDONE
bullAntifibroticbullReduces acute exacerbations and reduction in FVC
OXYGEN THERAPY
bull F or pa tients w i th d o c u m e nted h y p ox i a ndash S p O 2 lt89 P a O 2 lt55m m H g
bull Improves exerc i se to le rance
CONCLUSION
bull DPLD is the better term not a single disease bull IPF is the most common DPLDbull Restrictive Airway Diseasebull Main treatment aim is to suppress inflammation to prevent
further destruction of the pulmonary parenchyma
Thank you
- Outline
- introduction
- Introduction
- Introduction (2)
- Classification of dpld
- Idiopathic ILD
- Those with identifiable cause
- classification
- Epidemiology
- Ipf - PATHOgenesis VIDEO
- Slide 12
- Respiratory SYMPTOMS
- Non Respiratory symptoms associated with different dplds
- ONSET OF SYMPTOMS
- ILD-HISTORY
- Occupational history
- Environmental exposure history
- ILD-SIGNS
- Extra Pulmonary -SIGNS
- INVESTIGATIONS
- Chest X Ray
- High-resolution Computed topography (HRCT)
- PULMONARY FUNCTION TESTING
- PULMONARY FUNCTION TESTING (2)
- Laboratory investigations
- OTHER INVESTIGATIONS
- Slide 28
- MANEGEMENT
- CORTICOSTERIODS
- IMMUNO SUPRISSIVE AGENTS
- N-acetyl cystein
- Pirfenidone
- Oxygen therapy
- Conclusion
- Slide 36
-
INTRODUCTION
bull Interstitial is a misleading terminology because most of these disorders are associated with extensive alteration of airway and alveolar architecture in addition to changes in interstitial compartment
bull For this reason Diffuse Parenchymal Lung Disease or DPLD is the better term
CLASSIFICATION OF DPLD
bull DPLD Two large groups
(1)Idiopathic DPLD ( no known cause )
(2) Those with identifiable cause or occurs secondary to other diseases
IDIOPATHIC ILD
1Idiopathic pulmonary fibrosis (IPF) ndash most common2Acute interstitial pneumonia (AIP)3Cryptogenic Organizing Pneumonia ( COP) Bronchiolitis
obliterans organizing pneumonia (BOOP)4Desquamative interstitial pneumonia (DIP)5Lymphocytic interstitial Pneumonia ( LIP)6Non specific interstitial Pneumonia (NSIP)7Respiratory bronchiolitis associated Interstitial lung disease
(RBILD)
THOSE WITH IDENTIFIABLE CAUSE
1Autoimmune- rheumatoid arthritis SLE Sarcoidosis Scleroderma
2Certain infections3Medications4Radiation5Occupational exposure asbestos coal cotton silica
CLASSIFICATION
EPIDEMIOLOGY
bull Incidence ranges from 3-26100000 per yearbull Prevalence of preclinical and undiagnosed ILD is
estimated to be 10 times that of clinical recognized disease
bull IPF is the most common form representing at least 30 percent of the incident cases
IPF - PATHOGENESIS VIDEO
NORMAL DPLD
RESPIRATORY SYMPTOMS Breathlessness (most common) Initially dyspnea on exertionrarr later at rest Nonproductive cough
Pleuritic chest pain Wheeing Hemoptysis
NON RESPIRATORY SYMPTOMS ASSOCIATED WITH DIFFERENT DPLDS
bull Arthritisbull Ocular bull Skin and musclebull GERD bull Lower GI symptoms bull Recurrent sinusitis
bull Neurological symptoms bull Epilepsy amp mental retardation bull Diabetes inspidus
ONSET OF SYMPTOMS
1 Acute presentation (days to weeks)- eg Acute idiopathic interstitial pneumoniHypersensitive pneumonitis
2 Sub-acute presentation (weeks to months)- eg SarcoidosisDrug induced ILD Alveolar hemorrhage syndromes
3 Chronic presentation (months to years) ndash eg IPF
ILD-HISTORY
bullSmokingbullMedication history- Amiodorane Methotrexate
bullOccupational historybullEnvironmental exposure history
OCCUPATIONAL HISTORY1048766 Pneumoconioses ndash miners1048766 Silicosis ndash sand blasters amp granite workers1048766 Asbestosis ndash welders electricians mechanics workers withbrakes shipyard workers1048766 Berylliosis ndash aerospace nuclear computer amp electronic
industries1048766 Hypersensitive pneumonitis ndash farm workers poultry workersbird breeders1048766 The degree of exposure duration latency of exposure and
theuse of protective devices should be elicited
ENVIRONMENTAL EXPOSURE HISTORY
bull 1048766 Exposures to pets (especially birds)bull 1048766 Air conditionersbull 1048766 Humidifiersbull 1048766 Hot tubsbull 1048766 Evaporative cooling systems
ILD-SIGNS
bull Crackles - Dry velcro end inspiratory predominantly bibasilar
bull Inspiratory squeaks- Mid inspiratory high pitched- Seen in Airway centred pathologies
bull Cor pulmonale featuresbull Clubbing ndash IPF
EXTRA PULMONARY -SIGNS
bull Skin abnormalitiesbull Lymphadenopathybull Hepatosplenomegalybull Maculopapular skin rashes bull Erythema nodosum
bull Subcutaneous nodules bull Proximal muscle
weakness bull Arthritis
INVESTIGATIONS
CHEST X RAY
bull Typically small lung volumes with Reticular Nodular or RETICULONODULAR shadow
HIGH-RESOLUTION COMPUTED TOPOGRAPHY (HRCT)
bull HRCT is more sensitivebull Combinations of ground
glass changes reticulonodular shadowing honeycomb cysts and traction
bronchiectasis
PULMONARY FUNCTION TESTING
bull Objective assessment of Resp Symptoms
bull Grading the severity
bull Monitoring the response to therapy
PULMONARY FUNCTION TESTING
bull Restrictive Defect bull darr Lung volumes (TLC FRCRV lt80)bull darr FEV1 FVC With Normal or uarrFEV1FVC
bull Reduced diffusing capacity (DLCO)
LABORATORY INVESTIGATIONS
bull Full blood count lymphopenia in sarcoid eosinophilia in pulmonary eosinophilias and drug reactions neutrophilia in hypersensitivity pneumonitis
bull Ca2+ may be elevated in sarcoidbull Serum angiotensin-converting enzyme non-specific indicator of disease
activity in sarcoidbull ESR and CRP non-specifically raisedbull Autoimmune screen autoantibodies may suggest connectivetissue disease
OTHER INVESTIGATIONS
bull ARTERIAL BLOOD GAS ANALYSIS bull Tuberculin test ndash negative in 23 of sarcoidosis patientsbull BRONCHOALVEOLAR LAVAGE- cellular profile special
stains or studiesbull Lung biopsy- TRANS BRONCHIAL BIOPSY OPEN LUNG
BIOPSY
MANEGEMENTPRINCIPAL AIMS (1) to remove exposure to injurious agents
(2) to suppress inflammation to prevent further destruction of the pulmonary parenchyma
(3) to palliate the manifestations of these diseases
CORTICOSTERIODS
bull Prednisone 1 mgkgbull Gradually tapered (5 mgweek) over several months
to a maintenance dose
IMMUNO SUPRISSIVE AGENTS
Cytotoxic agents (Cyclophosphamide)or immunosuppressive agents (Azathioprine) may be used in patients who do not improve on steroid therapy or who cannot tolerate corticosteroids
N-ACETYL CYSTEIN
bullAntioxidant
bull600 mg PO tid added to prednisone and azathioprine preserves vital capacity and FVC and DLCO
PIRFENIDONE
bullAntifibroticbullReduces acute exacerbations and reduction in FVC
OXYGEN THERAPY
bull F or pa tients w i th d o c u m e nted h y p ox i a ndash S p O 2 lt89 P a O 2 lt55m m H g
bull Improves exerc i se to le rance
CONCLUSION
bull DPLD is the better term not a single disease bull IPF is the most common DPLDbull Restrictive Airway Diseasebull Main treatment aim is to suppress inflammation to prevent
further destruction of the pulmonary parenchyma
Thank you
- Outline
- introduction
- Introduction
- Introduction (2)
- Classification of dpld
- Idiopathic ILD
- Those with identifiable cause
- classification
- Epidemiology
- Ipf - PATHOgenesis VIDEO
- Slide 12
- Respiratory SYMPTOMS
- Non Respiratory symptoms associated with different dplds
- ONSET OF SYMPTOMS
- ILD-HISTORY
- Occupational history
- Environmental exposure history
- ILD-SIGNS
- Extra Pulmonary -SIGNS
- INVESTIGATIONS
- Chest X Ray
- High-resolution Computed topography (HRCT)
- PULMONARY FUNCTION TESTING
- PULMONARY FUNCTION TESTING (2)
- Laboratory investigations
- OTHER INVESTIGATIONS
- Slide 28
- MANEGEMENT
- CORTICOSTERIODS
- IMMUNO SUPRISSIVE AGENTS
- N-acetyl cystein
- Pirfenidone
- Oxygen therapy
- Conclusion
- Slide 36
-
CLASSIFICATION OF DPLD
bull DPLD Two large groups
(1)Idiopathic DPLD ( no known cause )
(2) Those with identifiable cause or occurs secondary to other diseases
IDIOPATHIC ILD
1Idiopathic pulmonary fibrosis (IPF) ndash most common2Acute interstitial pneumonia (AIP)3Cryptogenic Organizing Pneumonia ( COP) Bronchiolitis
obliterans organizing pneumonia (BOOP)4Desquamative interstitial pneumonia (DIP)5Lymphocytic interstitial Pneumonia ( LIP)6Non specific interstitial Pneumonia (NSIP)7Respiratory bronchiolitis associated Interstitial lung disease
(RBILD)
THOSE WITH IDENTIFIABLE CAUSE
1Autoimmune- rheumatoid arthritis SLE Sarcoidosis Scleroderma
2Certain infections3Medications4Radiation5Occupational exposure asbestos coal cotton silica
CLASSIFICATION
EPIDEMIOLOGY
bull Incidence ranges from 3-26100000 per yearbull Prevalence of preclinical and undiagnosed ILD is
estimated to be 10 times that of clinical recognized disease
bull IPF is the most common form representing at least 30 percent of the incident cases
IPF - PATHOGENESIS VIDEO
NORMAL DPLD
RESPIRATORY SYMPTOMS Breathlessness (most common) Initially dyspnea on exertionrarr later at rest Nonproductive cough
Pleuritic chest pain Wheeing Hemoptysis
NON RESPIRATORY SYMPTOMS ASSOCIATED WITH DIFFERENT DPLDS
bull Arthritisbull Ocular bull Skin and musclebull GERD bull Lower GI symptoms bull Recurrent sinusitis
bull Neurological symptoms bull Epilepsy amp mental retardation bull Diabetes inspidus
ONSET OF SYMPTOMS
1 Acute presentation (days to weeks)- eg Acute idiopathic interstitial pneumoniHypersensitive pneumonitis
2 Sub-acute presentation (weeks to months)- eg SarcoidosisDrug induced ILD Alveolar hemorrhage syndromes
3 Chronic presentation (months to years) ndash eg IPF
ILD-HISTORY
bullSmokingbullMedication history- Amiodorane Methotrexate
bullOccupational historybullEnvironmental exposure history
OCCUPATIONAL HISTORY1048766 Pneumoconioses ndash miners1048766 Silicosis ndash sand blasters amp granite workers1048766 Asbestosis ndash welders electricians mechanics workers withbrakes shipyard workers1048766 Berylliosis ndash aerospace nuclear computer amp electronic
industries1048766 Hypersensitive pneumonitis ndash farm workers poultry workersbird breeders1048766 The degree of exposure duration latency of exposure and
theuse of protective devices should be elicited
ENVIRONMENTAL EXPOSURE HISTORY
bull 1048766 Exposures to pets (especially birds)bull 1048766 Air conditionersbull 1048766 Humidifiersbull 1048766 Hot tubsbull 1048766 Evaporative cooling systems
ILD-SIGNS
bull Crackles - Dry velcro end inspiratory predominantly bibasilar
bull Inspiratory squeaks- Mid inspiratory high pitched- Seen in Airway centred pathologies
bull Cor pulmonale featuresbull Clubbing ndash IPF
EXTRA PULMONARY -SIGNS
bull Skin abnormalitiesbull Lymphadenopathybull Hepatosplenomegalybull Maculopapular skin rashes bull Erythema nodosum
bull Subcutaneous nodules bull Proximal muscle
weakness bull Arthritis
INVESTIGATIONS
CHEST X RAY
bull Typically small lung volumes with Reticular Nodular or RETICULONODULAR shadow
HIGH-RESOLUTION COMPUTED TOPOGRAPHY (HRCT)
bull HRCT is more sensitivebull Combinations of ground
glass changes reticulonodular shadowing honeycomb cysts and traction
bronchiectasis
PULMONARY FUNCTION TESTING
bull Objective assessment of Resp Symptoms
bull Grading the severity
bull Monitoring the response to therapy
PULMONARY FUNCTION TESTING
bull Restrictive Defect bull darr Lung volumes (TLC FRCRV lt80)bull darr FEV1 FVC With Normal or uarrFEV1FVC
bull Reduced diffusing capacity (DLCO)
LABORATORY INVESTIGATIONS
bull Full blood count lymphopenia in sarcoid eosinophilia in pulmonary eosinophilias and drug reactions neutrophilia in hypersensitivity pneumonitis
bull Ca2+ may be elevated in sarcoidbull Serum angiotensin-converting enzyme non-specific indicator of disease
activity in sarcoidbull ESR and CRP non-specifically raisedbull Autoimmune screen autoantibodies may suggest connectivetissue disease
OTHER INVESTIGATIONS
bull ARTERIAL BLOOD GAS ANALYSIS bull Tuberculin test ndash negative in 23 of sarcoidosis patientsbull BRONCHOALVEOLAR LAVAGE- cellular profile special
stains or studiesbull Lung biopsy- TRANS BRONCHIAL BIOPSY OPEN LUNG
BIOPSY
MANEGEMENTPRINCIPAL AIMS (1) to remove exposure to injurious agents
(2) to suppress inflammation to prevent further destruction of the pulmonary parenchyma
(3) to palliate the manifestations of these diseases
CORTICOSTERIODS
bull Prednisone 1 mgkgbull Gradually tapered (5 mgweek) over several months
to a maintenance dose
IMMUNO SUPRISSIVE AGENTS
Cytotoxic agents (Cyclophosphamide)or immunosuppressive agents (Azathioprine) may be used in patients who do not improve on steroid therapy or who cannot tolerate corticosteroids
N-ACETYL CYSTEIN
bullAntioxidant
bull600 mg PO tid added to prednisone and azathioprine preserves vital capacity and FVC and DLCO
PIRFENIDONE
bullAntifibroticbullReduces acute exacerbations and reduction in FVC
OXYGEN THERAPY
bull F or pa tients w i th d o c u m e nted h y p ox i a ndash S p O 2 lt89 P a O 2 lt55m m H g
bull Improves exerc i se to le rance
CONCLUSION
bull DPLD is the better term not a single disease bull IPF is the most common DPLDbull Restrictive Airway Diseasebull Main treatment aim is to suppress inflammation to prevent
further destruction of the pulmonary parenchyma
Thank you
- Outline
- introduction
- Introduction
- Introduction (2)
- Classification of dpld
- Idiopathic ILD
- Those with identifiable cause
- classification
- Epidemiology
- Ipf - PATHOgenesis VIDEO
- Slide 12
- Respiratory SYMPTOMS
- Non Respiratory symptoms associated with different dplds
- ONSET OF SYMPTOMS
- ILD-HISTORY
- Occupational history
- Environmental exposure history
- ILD-SIGNS
- Extra Pulmonary -SIGNS
- INVESTIGATIONS
- Chest X Ray
- High-resolution Computed topography (HRCT)
- PULMONARY FUNCTION TESTING
- PULMONARY FUNCTION TESTING (2)
- Laboratory investigations
- OTHER INVESTIGATIONS
- Slide 28
- MANEGEMENT
- CORTICOSTERIODS
- IMMUNO SUPRISSIVE AGENTS
- N-acetyl cystein
- Pirfenidone
- Oxygen therapy
- Conclusion
- Slide 36
-
IDIOPATHIC ILD
1Idiopathic pulmonary fibrosis (IPF) ndash most common2Acute interstitial pneumonia (AIP)3Cryptogenic Organizing Pneumonia ( COP) Bronchiolitis
obliterans organizing pneumonia (BOOP)4Desquamative interstitial pneumonia (DIP)5Lymphocytic interstitial Pneumonia ( LIP)6Non specific interstitial Pneumonia (NSIP)7Respiratory bronchiolitis associated Interstitial lung disease
(RBILD)
THOSE WITH IDENTIFIABLE CAUSE
1Autoimmune- rheumatoid arthritis SLE Sarcoidosis Scleroderma
2Certain infections3Medications4Radiation5Occupational exposure asbestos coal cotton silica
CLASSIFICATION
EPIDEMIOLOGY
bull Incidence ranges from 3-26100000 per yearbull Prevalence of preclinical and undiagnosed ILD is
estimated to be 10 times that of clinical recognized disease
bull IPF is the most common form representing at least 30 percent of the incident cases
IPF - PATHOGENESIS VIDEO
NORMAL DPLD
RESPIRATORY SYMPTOMS Breathlessness (most common) Initially dyspnea on exertionrarr later at rest Nonproductive cough
Pleuritic chest pain Wheeing Hemoptysis
NON RESPIRATORY SYMPTOMS ASSOCIATED WITH DIFFERENT DPLDS
bull Arthritisbull Ocular bull Skin and musclebull GERD bull Lower GI symptoms bull Recurrent sinusitis
bull Neurological symptoms bull Epilepsy amp mental retardation bull Diabetes inspidus
ONSET OF SYMPTOMS
1 Acute presentation (days to weeks)- eg Acute idiopathic interstitial pneumoniHypersensitive pneumonitis
2 Sub-acute presentation (weeks to months)- eg SarcoidosisDrug induced ILD Alveolar hemorrhage syndromes
3 Chronic presentation (months to years) ndash eg IPF
ILD-HISTORY
bullSmokingbullMedication history- Amiodorane Methotrexate
bullOccupational historybullEnvironmental exposure history
OCCUPATIONAL HISTORY1048766 Pneumoconioses ndash miners1048766 Silicosis ndash sand blasters amp granite workers1048766 Asbestosis ndash welders electricians mechanics workers withbrakes shipyard workers1048766 Berylliosis ndash aerospace nuclear computer amp electronic
industries1048766 Hypersensitive pneumonitis ndash farm workers poultry workersbird breeders1048766 The degree of exposure duration latency of exposure and
theuse of protective devices should be elicited
ENVIRONMENTAL EXPOSURE HISTORY
bull 1048766 Exposures to pets (especially birds)bull 1048766 Air conditionersbull 1048766 Humidifiersbull 1048766 Hot tubsbull 1048766 Evaporative cooling systems
ILD-SIGNS
bull Crackles - Dry velcro end inspiratory predominantly bibasilar
bull Inspiratory squeaks- Mid inspiratory high pitched- Seen in Airway centred pathologies
bull Cor pulmonale featuresbull Clubbing ndash IPF
EXTRA PULMONARY -SIGNS
bull Skin abnormalitiesbull Lymphadenopathybull Hepatosplenomegalybull Maculopapular skin rashes bull Erythema nodosum
bull Subcutaneous nodules bull Proximal muscle
weakness bull Arthritis
INVESTIGATIONS
CHEST X RAY
bull Typically small lung volumes with Reticular Nodular or RETICULONODULAR shadow
HIGH-RESOLUTION COMPUTED TOPOGRAPHY (HRCT)
bull HRCT is more sensitivebull Combinations of ground
glass changes reticulonodular shadowing honeycomb cysts and traction
bronchiectasis
PULMONARY FUNCTION TESTING
bull Objective assessment of Resp Symptoms
bull Grading the severity
bull Monitoring the response to therapy
PULMONARY FUNCTION TESTING
bull Restrictive Defect bull darr Lung volumes (TLC FRCRV lt80)bull darr FEV1 FVC With Normal or uarrFEV1FVC
bull Reduced diffusing capacity (DLCO)
LABORATORY INVESTIGATIONS
bull Full blood count lymphopenia in sarcoid eosinophilia in pulmonary eosinophilias and drug reactions neutrophilia in hypersensitivity pneumonitis
bull Ca2+ may be elevated in sarcoidbull Serum angiotensin-converting enzyme non-specific indicator of disease
activity in sarcoidbull ESR and CRP non-specifically raisedbull Autoimmune screen autoantibodies may suggest connectivetissue disease
OTHER INVESTIGATIONS
bull ARTERIAL BLOOD GAS ANALYSIS bull Tuberculin test ndash negative in 23 of sarcoidosis patientsbull BRONCHOALVEOLAR LAVAGE- cellular profile special
stains or studiesbull Lung biopsy- TRANS BRONCHIAL BIOPSY OPEN LUNG
BIOPSY
MANEGEMENTPRINCIPAL AIMS (1) to remove exposure to injurious agents
(2) to suppress inflammation to prevent further destruction of the pulmonary parenchyma
(3) to palliate the manifestations of these diseases
CORTICOSTERIODS
bull Prednisone 1 mgkgbull Gradually tapered (5 mgweek) over several months
to a maintenance dose
IMMUNO SUPRISSIVE AGENTS
Cytotoxic agents (Cyclophosphamide)or immunosuppressive agents (Azathioprine) may be used in patients who do not improve on steroid therapy or who cannot tolerate corticosteroids
N-ACETYL CYSTEIN
bullAntioxidant
bull600 mg PO tid added to prednisone and azathioprine preserves vital capacity and FVC and DLCO
PIRFENIDONE
bullAntifibroticbullReduces acute exacerbations and reduction in FVC
OXYGEN THERAPY
bull F or pa tients w i th d o c u m e nted h y p ox i a ndash S p O 2 lt89 P a O 2 lt55m m H g
bull Improves exerc i se to le rance
CONCLUSION
bull DPLD is the better term not a single disease bull IPF is the most common DPLDbull Restrictive Airway Diseasebull Main treatment aim is to suppress inflammation to prevent
further destruction of the pulmonary parenchyma
Thank you
- Outline
- introduction
- Introduction
- Introduction (2)
- Classification of dpld
- Idiopathic ILD
- Those with identifiable cause
- classification
- Epidemiology
- Ipf - PATHOgenesis VIDEO
- Slide 12
- Respiratory SYMPTOMS
- Non Respiratory symptoms associated with different dplds
- ONSET OF SYMPTOMS
- ILD-HISTORY
- Occupational history
- Environmental exposure history
- ILD-SIGNS
- Extra Pulmonary -SIGNS
- INVESTIGATIONS
- Chest X Ray
- High-resolution Computed topography (HRCT)
- PULMONARY FUNCTION TESTING
- PULMONARY FUNCTION TESTING (2)
- Laboratory investigations
- OTHER INVESTIGATIONS
- Slide 28
- MANEGEMENT
- CORTICOSTERIODS
- IMMUNO SUPRISSIVE AGENTS
- N-acetyl cystein
- Pirfenidone
- Oxygen therapy
- Conclusion
- Slide 36
-
THOSE WITH IDENTIFIABLE CAUSE
1Autoimmune- rheumatoid arthritis SLE Sarcoidosis Scleroderma
2Certain infections3Medications4Radiation5Occupational exposure asbestos coal cotton silica
CLASSIFICATION
EPIDEMIOLOGY
bull Incidence ranges from 3-26100000 per yearbull Prevalence of preclinical and undiagnosed ILD is
estimated to be 10 times that of clinical recognized disease
bull IPF is the most common form representing at least 30 percent of the incident cases
IPF - PATHOGENESIS VIDEO
NORMAL DPLD
RESPIRATORY SYMPTOMS Breathlessness (most common) Initially dyspnea on exertionrarr later at rest Nonproductive cough
Pleuritic chest pain Wheeing Hemoptysis
NON RESPIRATORY SYMPTOMS ASSOCIATED WITH DIFFERENT DPLDS
bull Arthritisbull Ocular bull Skin and musclebull GERD bull Lower GI symptoms bull Recurrent sinusitis
bull Neurological symptoms bull Epilepsy amp mental retardation bull Diabetes inspidus
ONSET OF SYMPTOMS
1 Acute presentation (days to weeks)- eg Acute idiopathic interstitial pneumoniHypersensitive pneumonitis
2 Sub-acute presentation (weeks to months)- eg SarcoidosisDrug induced ILD Alveolar hemorrhage syndromes
3 Chronic presentation (months to years) ndash eg IPF
ILD-HISTORY
bullSmokingbullMedication history- Amiodorane Methotrexate
bullOccupational historybullEnvironmental exposure history
OCCUPATIONAL HISTORY1048766 Pneumoconioses ndash miners1048766 Silicosis ndash sand blasters amp granite workers1048766 Asbestosis ndash welders electricians mechanics workers withbrakes shipyard workers1048766 Berylliosis ndash aerospace nuclear computer amp electronic
industries1048766 Hypersensitive pneumonitis ndash farm workers poultry workersbird breeders1048766 The degree of exposure duration latency of exposure and
theuse of protective devices should be elicited
ENVIRONMENTAL EXPOSURE HISTORY
bull 1048766 Exposures to pets (especially birds)bull 1048766 Air conditionersbull 1048766 Humidifiersbull 1048766 Hot tubsbull 1048766 Evaporative cooling systems
ILD-SIGNS
bull Crackles - Dry velcro end inspiratory predominantly bibasilar
bull Inspiratory squeaks- Mid inspiratory high pitched- Seen in Airway centred pathologies
bull Cor pulmonale featuresbull Clubbing ndash IPF
EXTRA PULMONARY -SIGNS
bull Skin abnormalitiesbull Lymphadenopathybull Hepatosplenomegalybull Maculopapular skin rashes bull Erythema nodosum
bull Subcutaneous nodules bull Proximal muscle
weakness bull Arthritis
INVESTIGATIONS
CHEST X RAY
bull Typically small lung volumes with Reticular Nodular or RETICULONODULAR shadow
HIGH-RESOLUTION COMPUTED TOPOGRAPHY (HRCT)
bull HRCT is more sensitivebull Combinations of ground
glass changes reticulonodular shadowing honeycomb cysts and traction
bronchiectasis
PULMONARY FUNCTION TESTING
bull Objective assessment of Resp Symptoms
bull Grading the severity
bull Monitoring the response to therapy
PULMONARY FUNCTION TESTING
bull Restrictive Defect bull darr Lung volumes (TLC FRCRV lt80)bull darr FEV1 FVC With Normal or uarrFEV1FVC
bull Reduced diffusing capacity (DLCO)
LABORATORY INVESTIGATIONS
bull Full blood count lymphopenia in sarcoid eosinophilia in pulmonary eosinophilias and drug reactions neutrophilia in hypersensitivity pneumonitis
bull Ca2+ may be elevated in sarcoidbull Serum angiotensin-converting enzyme non-specific indicator of disease
activity in sarcoidbull ESR and CRP non-specifically raisedbull Autoimmune screen autoantibodies may suggest connectivetissue disease
OTHER INVESTIGATIONS
bull ARTERIAL BLOOD GAS ANALYSIS bull Tuberculin test ndash negative in 23 of sarcoidosis patientsbull BRONCHOALVEOLAR LAVAGE- cellular profile special
stains or studiesbull Lung biopsy- TRANS BRONCHIAL BIOPSY OPEN LUNG
BIOPSY
MANEGEMENTPRINCIPAL AIMS (1) to remove exposure to injurious agents
(2) to suppress inflammation to prevent further destruction of the pulmonary parenchyma
(3) to palliate the manifestations of these diseases
CORTICOSTERIODS
bull Prednisone 1 mgkgbull Gradually tapered (5 mgweek) over several months
to a maintenance dose
IMMUNO SUPRISSIVE AGENTS
Cytotoxic agents (Cyclophosphamide)or immunosuppressive agents (Azathioprine) may be used in patients who do not improve on steroid therapy or who cannot tolerate corticosteroids
N-ACETYL CYSTEIN
bullAntioxidant
bull600 mg PO tid added to prednisone and azathioprine preserves vital capacity and FVC and DLCO
PIRFENIDONE
bullAntifibroticbullReduces acute exacerbations and reduction in FVC
OXYGEN THERAPY
bull F or pa tients w i th d o c u m e nted h y p ox i a ndash S p O 2 lt89 P a O 2 lt55m m H g
bull Improves exerc i se to le rance
CONCLUSION
bull DPLD is the better term not a single disease bull IPF is the most common DPLDbull Restrictive Airway Diseasebull Main treatment aim is to suppress inflammation to prevent
further destruction of the pulmonary parenchyma
Thank you
- Outline
- introduction
- Introduction
- Introduction (2)
- Classification of dpld
- Idiopathic ILD
- Those with identifiable cause
- classification
- Epidemiology
- Ipf - PATHOgenesis VIDEO
- Slide 12
- Respiratory SYMPTOMS
- Non Respiratory symptoms associated with different dplds
- ONSET OF SYMPTOMS
- ILD-HISTORY
- Occupational history
- Environmental exposure history
- ILD-SIGNS
- Extra Pulmonary -SIGNS
- INVESTIGATIONS
- Chest X Ray
- High-resolution Computed topography (HRCT)
- PULMONARY FUNCTION TESTING
- PULMONARY FUNCTION TESTING (2)
- Laboratory investigations
- OTHER INVESTIGATIONS
- Slide 28
- MANEGEMENT
- CORTICOSTERIODS
- IMMUNO SUPRISSIVE AGENTS
- N-acetyl cystein
- Pirfenidone
- Oxygen therapy
- Conclusion
- Slide 36
-
CLASSIFICATION
EPIDEMIOLOGY
bull Incidence ranges from 3-26100000 per yearbull Prevalence of preclinical and undiagnosed ILD is
estimated to be 10 times that of clinical recognized disease
bull IPF is the most common form representing at least 30 percent of the incident cases
IPF - PATHOGENESIS VIDEO
NORMAL DPLD
RESPIRATORY SYMPTOMS Breathlessness (most common) Initially dyspnea on exertionrarr later at rest Nonproductive cough
Pleuritic chest pain Wheeing Hemoptysis
NON RESPIRATORY SYMPTOMS ASSOCIATED WITH DIFFERENT DPLDS
bull Arthritisbull Ocular bull Skin and musclebull GERD bull Lower GI symptoms bull Recurrent sinusitis
bull Neurological symptoms bull Epilepsy amp mental retardation bull Diabetes inspidus
ONSET OF SYMPTOMS
1 Acute presentation (days to weeks)- eg Acute idiopathic interstitial pneumoniHypersensitive pneumonitis
2 Sub-acute presentation (weeks to months)- eg SarcoidosisDrug induced ILD Alveolar hemorrhage syndromes
3 Chronic presentation (months to years) ndash eg IPF
ILD-HISTORY
bullSmokingbullMedication history- Amiodorane Methotrexate
bullOccupational historybullEnvironmental exposure history
OCCUPATIONAL HISTORY1048766 Pneumoconioses ndash miners1048766 Silicosis ndash sand blasters amp granite workers1048766 Asbestosis ndash welders electricians mechanics workers withbrakes shipyard workers1048766 Berylliosis ndash aerospace nuclear computer amp electronic
industries1048766 Hypersensitive pneumonitis ndash farm workers poultry workersbird breeders1048766 The degree of exposure duration latency of exposure and
theuse of protective devices should be elicited
ENVIRONMENTAL EXPOSURE HISTORY
bull 1048766 Exposures to pets (especially birds)bull 1048766 Air conditionersbull 1048766 Humidifiersbull 1048766 Hot tubsbull 1048766 Evaporative cooling systems
ILD-SIGNS
bull Crackles - Dry velcro end inspiratory predominantly bibasilar
bull Inspiratory squeaks- Mid inspiratory high pitched- Seen in Airway centred pathologies
bull Cor pulmonale featuresbull Clubbing ndash IPF
EXTRA PULMONARY -SIGNS
bull Skin abnormalitiesbull Lymphadenopathybull Hepatosplenomegalybull Maculopapular skin rashes bull Erythema nodosum
bull Subcutaneous nodules bull Proximal muscle
weakness bull Arthritis
INVESTIGATIONS
CHEST X RAY
bull Typically small lung volumes with Reticular Nodular or RETICULONODULAR shadow
HIGH-RESOLUTION COMPUTED TOPOGRAPHY (HRCT)
bull HRCT is more sensitivebull Combinations of ground
glass changes reticulonodular shadowing honeycomb cysts and traction
bronchiectasis
PULMONARY FUNCTION TESTING
bull Objective assessment of Resp Symptoms
bull Grading the severity
bull Monitoring the response to therapy
PULMONARY FUNCTION TESTING
bull Restrictive Defect bull darr Lung volumes (TLC FRCRV lt80)bull darr FEV1 FVC With Normal or uarrFEV1FVC
bull Reduced diffusing capacity (DLCO)
LABORATORY INVESTIGATIONS
bull Full blood count lymphopenia in sarcoid eosinophilia in pulmonary eosinophilias and drug reactions neutrophilia in hypersensitivity pneumonitis
bull Ca2+ may be elevated in sarcoidbull Serum angiotensin-converting enzyme non-specific indicator of disease
activity in sarcoidbull ESR and CRP non-specifically raisedbull Autoimmune screen autoantibodies may suggest connectivetissue disease
OTHER INVESTIGATIONS
bull ARTERIAL BLOOD GAS ANALYSIS bull Tuberculin test ndash negative in 23 of sarcoidosis patientsbull BRONCHOALVEOLAR LAVAGE- cellular profile special
stains or studiesbull Lung biopsy- TRANS BRONCHIAL BIOPSY OPEN LUNG
BIOPSY
MANEGEMENTPRINCIPAL AIMS (1) to remove exposure to injurious agents
(2) to suppress inflammation to prevent further destruction of the pulmonary parenchyma
(3) to palliate the manifestations of these diseases
CORTICOSTERIODS
bull Prednisone 1 mgkgbull Gradually tapered (5 mgweek) over several months
to a maintenance dose
IMMUNO SUPRISSIVE AGENTS
Cytotoxic agents (Cyclophosphamide)or immunosuppressive agents (Azathioprine) may be used in patients who do not improve on steroid therapy or who cannot tolerate corticosteroids
N-ACETYL CYSTEIN
bullAntioxidant
bull600 mg PO tid added to prednisone and azathioprine preserves vital capacity and FVC and DLCO
PIRFENIDONE
bullAntifibroticbullReduces acute exacerbations and reduction in FVC
OXYGEN THERAPY
bull F or pa tients w i th d o c u m e nted h y p ox i a ndash S p O 2 lt89 P a O 2 lt55m m H g
bull Improves exerc i se to le rance
CONCLUSION
bull DPLD is the better term not a single disease bull IPF is the most common DPLDbull Restrictive Airway Diseasebull Main treatment aim is to suppress inflammation to prevent
further destruction of the pulmonary parenchyma
Thank you
- Outline
- introduction
- Introduction
- Introduction (2)
- Classification of dpld
- Idiopathic ILD
- Those with identifiable cause
- classification
- Epidemiology
- Ipf - PATHOgenesis VIDEO
- Slide 12
- Respiratory SYMPTOMS
- Non Respiratory symptoms associated with different dplds
- ONSET OF SYMPTOMS
- ILD-HISTORY
- Occupational history
- Environmental exposure history
- ILD-SIGNS
- Extra Pulmonary -SIGNS
- INVESTIGATIONS
- Chest X Ray
- High-resolution Computed topography (HRCT)
- PULMONARY FUNCTION TESTING
- PULMONARY FUNCTION TESTING (2)
- Laboratory investigations
- OTHER INVESTIGATIONS
- Slide 28
- MANEGEMENT
- CORTICOSTERIODS
- IMMUNO SUPRISSIVE AGENTS
- N-acetyl cystein
- Pirfenidone
- Oxygen therapy
- Conclusion
- Slide 36
-
EPIDEMIOLOGY
bull Incidence ranges from 3-26100000 per yearbull Prevalence of preclinical and undiagnosed ILD is
estimated to be 10 times that of clinical recognized disease
bull IPF is the most common form representing at least 30 percent of the incident cases
IPF - PATHOGENESIS VIDEO
NORMAL DPLD
RESPIRATORY SYMPTOMS Breathlessness (most common) Initially dyspnea on exertionrarr later at rest Nonproductive cough
Pleuritic chest pain Wheeing Hemoptysis
NON RESPIRATORY SYMPTOMS ASSOCIATED WITH DIFFERENT DPLDS
bull Arthritisbull Ocular bull Skin and musclebull GERD bull Lower GI symptoms bull Recurrent sinusitis
bull Neurological symptoms bull Epilepsy amp mental retardation bull Diabetes inspidus
ONSET OF SYMPTOMS
1 Acute presentation (days to weeks)- eg Acute idiopathic interstitial pneumoniHypersensitive pneumonitis
2 Sub-acute presentation (weeks to months)- eg SarcoidosisDrug induced ILD Alveolar hemorrhage syndromes
3 Chronic presentation (months to years) ndash eg IPF
ILD-HISTORY
bullSmokingbullMedication history- Amiodorane Methotrexate
bullOccupational historybullEnvironmental exposure history
OCCUPATIONAL HISTORY1048766 Pneumoconioses ndash miners1048766 Silicosis ndash sand blasters amp granite workers1048766 Asbestosis ndash welders electricians mechanics workers withbrakes shipyard workers1048766 Berylliosis ndash aerospace nuclear computer amp electronic
industries1048766 Hypersensitive pneumonitis ndash farm workers poultry workersbird breeders1048766 The degree of exposure duration latency of exposure and
theuse of protective devices should be elicited
ENVIRONMENTAL EXPOSURE HISTORY
bull 1048766 Exposures to pets (especially birds)bull 1048766 Air conditionersbull 1048766 Humidifiersbull 1048766 Hot tubsbull 1048766 Evaporative cooling systems
ILD-SIGNS
bull Crackles - Dry velcro end inspiratory predominantly bibasilar
bull Inspiratory squeaks- Mid inspiratory high pitched- Seen in Airway centred pathologies
bull Cor pulmonale featuresbull Clubbing ndash IPF
EXTRA PULMONARY -SIGNS
bull Skin abnormalitiesbull Lymphadenopathybull Hepatosplenomegalybull Maculopapular skin rashes bull Erythema nodosum
bull Subcutaneous nodules bull Proximal muscle
weakness bull Arthritis
INVESTIGATIONS
CHEST X RAY
bull Typically small lung volumes with Reticular Nodular or RETICULONODULAR shadow
HIGH-RESOLUTION COMPUTED TOPOGRAPHY (HRCT)
bull HRCT is more sensitivebull Combinations of ground
glass changes reticulonodular shadowing honeycomb cysts and traction
bronchiectasis
PULMONARY FUNCTION TESTING
bull Objective assessment of Resp Symptoms
bull Grading the severity
bull Monitoring the response to therapy
PULMONARY FUNCTION TESTING
bull Restrictive Defect bull darr Lung volumes (TLC FRCRV lt80)bull darr FEV1 FVC With Normal or uarrFEV1FVC
bull Reduced diffusing capacity (DLCO)
LABORATORY INVESTIGATIONS
bull Full blood count lymphopenia in sarcoid eosinophilia in pulmonary eosinophilias and drug reactions neutrophilia in hypersensitivity pneumonitis
bull Ca2+ may be elevated in sarcoidbull Serum angiotensin-converting enzyme non-specific indicator of disease
activity in sarcoidbull ESR and CRP non-specifically raisedbull Autoimmune screen autoantibodies may suggest connectivetissue disease
OTHER INVESTIGATIONS
bull ARTERIAL BLOOD GAS ANALYSIS bull Tuberculin test ndash negative in 23 of sarcoidosis patientsbull BRONCHOALVEOLAR LAVAGE- cellular profile special
stains or studiesbull Lung biopsy- TRANS BRONCHIAL BIOPSY OPEN LUNG
BIOPSY
MANEGEMENTPRINCIPAL AIMS (1) to remove exposure to injurious agents
(2) to suppress inflammation to prevent further destruction of the pulmonary parenchyma
(3) to palliate the manifestations of these diseases
CORTICOSTERIODS
bull Prednisone 1 mgkgbull Gradually tapered (5 mgweek) over several months
to a maintenance dose
IMMUNO SUPRISSIVE AGENTS
Cytotoxic agents (Cyclophosphamide)or immunosuppressive agents (Azathioprine) may be used in patients who do not improve on steroid therapy or who cannot tolerate corticosteroids
N-ACETYL CYSTEIN
bullAntioxidant
bull600 mg PO tid added to prednisone and azathioprine preserves vital capacity and FVC and DLCO
PIRFENIDONE
bullAntifibroticbullReduces acute exacerbations and reduction in FVC
OXYGEN THERAPY
bull F or pa tients w i th d o c u m e nted h y p ox i a ndash S p O 2 lt89 P a O 2 lt55m m H g
bull Improves exerc i se to le rance
CONCLUSION
bull DPLD is the better term not a single disease bull IPF is the most common DPLDbull Restrictive Airway Diseasebull Main treatment aim is to suppress inflammation to prevent
further destruction of the pulmonary parenchyma
Thank you
- Outline
- introduction
- Introduction
- Introduction (2)
- Classification of dpld
- Idiopathic ILD
- Those with identifiable cause
- classification
- Epidemiology
- Ipf - PATHOgenesis VIDEO
- Slide 12
- Respiratory SYMPTOMS
- Non Respiratory symptoms associated with different dplds
- ONSET OF SYMPTOMS
- ILD-HISTORY
- Occupational history
- Environmental exposure history
- ILD-SIGNS
- Extra Pulmonary -SIGNS
- INVESTIGATIONS
- Chest X Ray
- High-resolution Computed topography (HRCT)
- PULMONARY FUNCTION TESTING
- PULMONARY FUNCTION TESTING (2)
- Laboratory investigations
- OTHER INVESTIGATIONS
- Slide 28
- MANEGEMENT
- CORTICOSTERIODS
- IMMUNO SUPRISSIVE AGENTS
- N-acetyl cystein
- Pirfenidone
- Oxygen therapy
- Conclusion
- Slide 36
-
IPF - PATHOGENESIS VIDEO
NORMAL DPLD
RESPIRATORY SYMPTOMS Breathlessness (most common) Initially dyspnea on exertionrarr later at rest Nonproductive cough
Pleuritic chest pain Wheeing Hemoptysis
NON RESPIRATORY SYMPTOMS ASSOCIATED WITH DIFFERENT DPLDS
bull Arthritisbull Ocular bull Skin and musclebull GERD bull Lower GI symptoms bull Recurrent sinusitis
bull Neurological symptoms bull Epilepsy amp mental retardation bull Diabetes inspidus
ONSET OF SYMPTOMS
1 Acute presentation (days to weeks)- eg Acute idiopathic interstitial pneumoniHypersensitive pneumonitis
2 Sub-acute presentation (weeks to months)- eg SarcoidosisDrug induced ILD Alveolar hemorrhage syndromes
3 Chronic presentation (months to years) ndash eg IPF
ILD-HISTORY
bullSmokingbullMedication history- Amiodorane Methotrexate
bullOccupational historybullEnvironmental exposure history
OCCUPATIONAL HISTORY1048766 Pneumoconioses ndash miners1048766 Silicosis ndash sand blasters amp granite workers1048766 Asbestosis ndash welders electricians mechanics workers withbrakes shipyard workers1048766 Berylliosis ndash aerospace nuclear computer amp electronic
industries1048766 Hypersensitive pneumonitis ndash farm workers poultry workersbird breeders1048766 The degree of exposure duration latency of exposure and
theuse of protective devices should be elicited
ENVIRONMENTAL EXPOSURE HISTORY
bull 1048766 Exposures to pets (especially birds)bull 1048766 Air conditionersbull 1048766 Humidifiersbull 1048766 Hot tubsbull 1048766 Evaporative cooling systems
ILD-SIGNS
bull Crackles - Dry velcro end inspiratory predominantly bibasilar
bull Inspiratory squeaks- Mid inspiratory high pitched- Seen in Airway centred pathologies
bull Cor pulmonale featuresbull Clubbing ndash IPF
EXTRA PULMONARY -SIGNS
bull Skin abnormalitiesbull Lymphadenopathybull Hepatosplenomegalybull Maculopapular skin rashes bull Erythema nodosum
bull Subcutaneous nodules bull Proximal muscle
weakness bull Arthritis
INVESTIGATIONS
CHEST X RAY
bull Typically small lung volumes with Reticular Nodular or RETICULONODULAR shadow
HIGH-RESOLUTION COMPUTED TOPOGRAPHY (HRCT)
bull HRCT is more sensitivebull Combinations of ground
glass changes reticulonodular shadowing honeycomb cysts and traction
bronchiectasis
PULMONARY FUNCTION TESTING
bull Objective assessment of Resp Symptoms
bull Grading the severity
bull Monitoring the response to therapy
PULMONARY FUNCTION TESTING
bull Restrictive Defect bull darr Lung volumes (TLC FRCRV lt80)bull darr FEV1 FVC With Normal or uarrFEV1FVC
bull Reduced diffusing capacity (DLCO)
LABORATORY INVESTIGATIONS
bull Full blood count lymphopenia in sarcoid eosinophilia in pulmonary eosinophilias and drug reactions neutrophilia in hypersensitivity pneumonitis
bull Ca2+ may be elevated in sarcoidbull Serum angiotensin-converting enzyme non-specific indicator of disease
activity in sarcoidbull ESR and CRP non-specifically raisedbull Autoimmune screen autoantibodies may suggest connectivetissue disease
OTHER INVESTIGATIONS
bull ARTERIAL BLOOD GAS ANALYSIS bull Tuberculin test ndash negative in 23 of sarcoidosis patientsbull BRONCHOALVEOLAR LAVAGE- cellular profile special
stains or studiesbull Lung biopsy- TRANS BRONCHIAL BIOPSY OPEN LUNG
BIOPSY
MANEGEMENTPRINCIPAL AIMS (1) to remove exposure to injurious agents
(2) to suppress inflammation to prevent further destruction of the pulmonary parenchyma
(3) to palliate the manifestations of these diseases
CORTICOSTERIODS
bull Prednisone 1 mgkgbull Gradually tapered (5 mgweek) over several months
to a maintenance dose
IMMUNO SUPRISSIVE AGENTS
Cytotoxic agents (Cyclophosphamide)or immunosuppressive agents (Azathioprine) may be used in patients who do not improve on steroid therapy or who cannot tolerate corticosteroids
N-ACETYL CYSTEIN
bullAntioxidant
bull600 mg PO tid added to prednisone and azathioprine preserves vital capacity and FVC and DLCO
PIRFENIDONE
bullAntifibroticbullReduces acute exacerbations and reduction in FVC
OXYGEN THERAPY
bull F or pa tients w i th d o c u m e nted h y p ox i a ndash S p O 2 lt89 P a O 2 lt55m m H g
bull Improves exerc i se to le rance
CONCLUSION
bull DPLD is the better term not a single disease bull IPF is the most common DPLDbull Restrictive Airway Diseasebull Main treatment aim is to suppress inflammation to prevent
further destruction of the pulmonary parenchyma
Thank you
- Outline
- introduction
- Introduction
- Introduction (2)
- Classification of dpld
- Idiopathic ILD
- Those with identifiable cause
- classification
- Epidemiology
- Ipf - PATHOgenesis VIDEO
- Slide 12
- Respiratory SYMPTOMS
- Non Respiratory symptoms associated with different dplds
- ONSET OF SYMPTOMS
- ILD-HISTORY
- Occupational history
- Environmental exposure history
- ILD-SIGNS
- Extra Pulmonary -SIGNS
- INVESTIGATIONS
- Chest X Ray
- High-resolution Computed topography (HRCT)
- PULMONARY FUNCTION TESTING
- PULMONARY FUNCTION TESTING (2)
- Laboratory investigations
- OTHER INVESTIGATIONS
- Slide 28
- MANEGEMENT
- CORTICOSTERIODS
- IMMUNO SUPRISSIVE AGENTS
- N-acetyl cystein
- Pirfenidone
- Oxygen therapy
- Conclusion
- Slide 36
-
NORMAL DPLD
RESPIRATORY SYMPTOMS Breathlessness (most common) Initially dyspnea on exertionrarr later at rest Nonproductive cough
Pleuritic chest pain Wheeing Hemoptysis
NON RESPIRATORY SYMPTOMS ASSOCIATED WITH DIFFERENT DPLDS
bull Arthritisbull Ocular bull Skin and musclebull GERD bull Lower GI symptoms bull Recurrent sinusitis
bull Neurological symptoms bull Epilepsy amp mental retardation bull Diabetes inspidus
ONSET OF SYMPTOMS
1 Acute presentation (days to weeks)- eg Acute idiopathic interstitial pneumoniHypersensitive pneumonitis
2 Sub-acute presentation (weeks to months)- eg SarcoidosisDrug induced ILD Alveolar hemorrhage syndromes
3 Chronic presentation (months to years) ndash eg IPF
ILD-HISTORY
bullSmokingbullMedication history- Amiodorane Methotrexate
bullOccupational historybullEnvironmental exposure history
OCCUPATIONAL HISTORY1048766 Pneumoconioses ndash miners1048766 Silicosis ndash sand blasters amp granite workers1048766 Asbestosis ndash welders electricians mechanics workers withbrakes shipyard workers1048766 Berylliosis ndash aerospace nuclear computer amp electronic
industries1048766 Hypersensitive pneumonitis ndash farm workers poultry workersbird breeders1048766 The degree of exposure duration latency of exposure and
theuse of protective devices should be elicited
ENVIRONMENTAL EXPOSURE HISTORY
bull 1048766 Exposures to pets (especially birds)bull 1048766 Air conditionersbull 1048766 Humidifiersbull 1048766 Hot tubsbull 1048766 Evaporative cooling systems
ILD-SIGNS
bull Crackles - Dry velcro end inspiratory predominantly bibasilar
bull Inspiratory squeaks- Mid inspiratory high pitched- Seen in Airway centred pathologies
bull Cor pulmonale featuresbull Clubbing ndash IPF
EXTRA PULMONARY -SIGNS
bull Skin abnormalitiesbull Lymphadenopathybull Hepatosplenomegalybull Maculopapular skin rashes bull Erythema nodosum
bull Subcutaneous nodules bull Proximal muscle
weakness bull Arthritis
INVESTIGATIONS
CHEST X RAY
bull Typically small lung volumes with Reticular Nodular or RETICULONODULAR shadow
HIGH-RESOLUTION COMPUTED TOPOGRAPHY (HRCT)
bull HRCT is more sensitivebull Combinations of ground
glass changes reticulonodular shadowing honeycomb cysts and traction
bronchiectasis
PULMONARY FUNCTION TESTING
bull Objective assessment of Resp Symptoms
bull Grading the severity
bull Monitoring the response to therapy
PULMONARY FUNCTION TESTING
bull Restrictive Defect bull darr Lung volumes (TLC FRCRV lt80)bull darr FEV1 FVC With Normal or uarrFEV1FVC
bull Reduced diffusing capacity (DLCO)
LABORATORY INVESTIGATIONS
bull Full blood count lymphopenia in sarcoid eosinophilia in pulmonary eosinophilias and drug reactions neutrophilia in hypersensitivity pneumonitis
bull Ca2+ may be elevated in sarcoidbull Serum angiotensin-converting enzyme non-specific indicator of disease
activity in sarcoidbull ESR and CRP non-specifically raisedbull Autoimmune screen autoantibodies may suggest connectivetissue disease
OTHER INVESTIGATIONS
bull ARTERIAL BLOOD GAS ANALYSIS bull Tuberculin test ndash negative in 23 of sarcoidosis patientsbull BRONCHOALVEOLAR LAVAGE- cellular profile special
stains or studiesbull Lung biopsy- TRANS BRONCHIAL BIOPSY OPEN LUNG
BIOPSY
MANEGEMENTPRINCIPAL AIMS (1) to remove exposure to injurious agents
(2) to suppress inflammation to prevent further destruction of the pulmonary parenchyma
(3) to palliate the manifestations of these diseases
CORTICOSTERIODS
bull Prednisone 1 mgkgbull Gradually tapered (5 mgweek) over several months
to a maintenance dose
IMMUNO SUPRISSIVE AGENTS
Cytotoxic agents (Cyclophosphamide)or immunosuppressive agents (Azathioprine) may be used in patients who do not improve on steroid therapy or who cannot tolerate corticosteroids
N-ACETYL CYSTEIN
bullAntioxidant
bull600 mg PO tid added to prednisone and azathioprine preserves vital capacity and FVC and DLCO
PIRFENIDONE
bullAntifibroticbullReduces acute exacerbations and reduction in FVC
OXYGEN THERAPY
bull F or pa tients w i th d o c u m e nted h y p ox i a ndash S p O 2 lt89 P a O 2 lt55m m H g
bull Improves exerc i se to le rance
CONCLUSION
bull DPLD is the better term not a single disease bull IPF is the most common DPLDbull Restrictive Airway Diseasebull Main treatment aim is to suppress inflammation to prevent
further destruction of the pulmonary parenchyma
Thank you
- Outline
- introduction
- Introduction
- Introduction (2)
- Classification of dpld
- Idiopathic ILD
- Those with identifiable cause
- classification
- Epidemiology
- Ipf - PATHOgenesis VIDEO
- Slide 12
- Respiratory SYMPTOMS
- Non Respiratory symptoms associated with different dplds
- ONSET OF SYMPTOMS
- ILD-HISTORY
- Occupational history
- Environmental exposure history
- ILD-SIGNS
- Extra Pulmonary -SIGNS
- INVESTIGATIONS
- Chest X Ray
- High-resolution Computed topography (HRCT)
- PULMONARY FUNCTION TESTING
- PULMONARY FUNCTION TESTING (2)
- Laboratory investigations
- OTHER INVESTIGATIONS
- Slide 28
- MANEGEMENT
- CORTICOSTERIODS
- IMMUNO SUPRISSIVE AGENTS
- N-acetyl cystein
- Pirfenidone
- Oxygen therapy
- Conclusion
- Slide 36
-
RESPIRATORY SYMPTOMS Breathlessness (most common) Initially dyspnea on exertionrarr later at rest Nonproductive cough
Pleuritic chest pain Wheeing Hemoptysis
NON RESPIRATORY SYMPTOMS ASSOCIATED WITH DIFFERENT DPLDS
bull Arthritisbull Ocular bull Skin and musclebull GERD bull Lower GI symptoms bull Recurrent sinusitis
bull Neurological symptoms bull Epilepsy amp mental retardation bull Diabetes inspidus
ONSET OF SYMPTOMS
1 Acute presentation (days to weeks)- eg Acute idiopathic interstitial pneumoniHypersensitive pneumonitis
2 Sub-acute presentation (weeks to months)- eg SarcoidosisDrug induced ILD Alveolar hemorrhage syndromes
3 Chronic presentation (months to years) ndash eg IPF
ILD-HISTORY
bullSmokingbullMedication history- Amiodorane Methotrexate
bullOccupational historybullEnvironmental exposure history
OCCUPATIONAL HISTORY1048766 Pneumoconioses ndash miners1048766 Silicosis ndash sand blasters amp granite workers1048766 Asbestosis ndash welders electricians mechanics workers withbrakes shipyard workers1048766 Berylliosis ndash aerospace nuclear computer amp electronic
industries1048766 Hypersensitive pneumonitis ndash farm workers poultry workersbird breeders1048766 The degree of exposure duration latency of exposure and
theuse of protective devices should be elicited
ENVIRONMENTAL EXPOSURE HISTORY
bull 1048766 Exposures to pets (especially birds)bull 1048766 Air conditionersbull 1048766 Humidifiersbull 1048766 Hot tubsbull 1048766 Evaporative cooling systems
ILD-SIGNS
bull Crackles - Dry velcro end inspiratory predominantly bibasilar
bull Inspiratory squeaks- Mid inspiratory high pitched- Seen in Airway centred pathologies
bull Cor pulmonale featuresbull Clubbing ndash IPF
EXTRA PULMONARY -SIGNS
bull Skin abnormalitiesbull Lymphadenopathybull Hepatosplenomegalybull Maculopapular skin rashes bull Erythema nodosum
bull Subcutaneous nodules bull Proximal muscle
weakness bull Arthritis
INVESTIGATIONS
CHEST X RAY
bull Typically small lung volumes with Reticular Nodular or RETICULONODULAR shadow
HIGH-RESOLUTION COMPUTED TOPOGRAPHY (HRCT)
bull HRCT is more sensitivebull Combinations of ground
glass changes reticulonodular shadowing honeycomb cysts and traction
bronchiectasis
PULMONARY FUNCTION TESTING
bull Objective assessment of Resp Symptoms
bull Grading the severity
bull Monitoring the response to therapy
PULMONARY FUNCTION TESTING
bull Restrictive Defect bull darr Lung volumes (TLC FRCRV lt80)bull darr FEV1 FVC With Normal or uarrFEV1FVC
bull Reduced diffusing capacity (DLCO)
LABORATORY INVESTIGATIONS
bull Full blood count lymphopenia in sarcoid eosinophilia in pulmonary eosinophilias and drug reactions neutrophilia in hypersensitivity pneumonitis
bull Ca2+ may be elevated in sarcoidbull Serum angiotensin-converting enzyme non-specific indicator of disease
activity in sarcoidbull ESR and CRP non-specifically raisedbull Autoimmune screen autoantibodies may suggest connectivetissue disease
OTHER INVESTIGATIONS
bull ARTERIAL BLOOD GAS ANALYSIS bull Tuberculin test ndash negative in 23 of sarcoidosis patientsbull BRONCHOALVEOLAR LAVAGE- cellular profile special
stains or studiesbull Lung biopsy- TRANS BRONCHIAL BIOPSY OPEN LUNG
BIOPSY
MANEGEMENTPRINCIPAL AIMS (1) to remove exposure to injurious agents
(2) to suppress inflammation to prevent further destruction of the pulmonary parenchyma
(3) to palliate the manifestations of these diseases
CORTICOSTERIODS
bull Prednisone 1 mgkgbull Gradually tapered (5 mgweek) over several months
to a maintenance dose
IMMUNO SUPRISSIVE AGENTS
Cytotoxic agents (Cyclophosphamide)or immunosuppressive agents (Azathioprine) may be used in patients who do not improve on steroid therapy or who cannot tolerate corticosteroids
N-ACETYL CYSTEIN
bullAntioxidant
bull600 mg PO tid added to prednisone and azathioprine preserves vital capacity and FVC and DLCO
PIRFENIDONE
bullAntifibroticbullReduces acute exacerbations and reduction in FVC
OXYGEN THERAPY
bull F or pa tients w i th d o c u m e nted h y p ox i a ndash S p O 2 lt89 P a O 2 lt55m m H g
bull Improves exerc i se to le rance
CONCLUSION
bull DPLD is the better term not a single disease bull IPF is the most common DPLDbull Restrictive Airway Diseasebull Main treatment aim is to suppress inflammation to prevent
further destruction of the pulmonary parenchyma
Thank you
- Outline
- introduction
- Introduction
- Introduction (2)
- Classification of dpld
- Idiopathic ILD
- Those with identifiable cause
- classification
- Epidemiology
- Ipf - PATHOgenesis VIDEO
- Slide 12
- Respiratory SYMPTOMS
- Non Respiratory symptoms associated with different dplds
- ONSET OF SYMPTOMS
- ILD-HISTORY
- Occupational history
- Environmental exposure history
- ILD-SIGNS
- Extra Pulmonary -SIGNS
- INVESTIGATIONS
- Chest X Ray
- High-resolution Computed topography (HRCT)
- PULMONARY FUNCTION TESTING
- PULMONARY FUNCTION TESTING (2)
- Laboratory investigations
- OTHER INVESTIGATIONS
- Slide 28
- MANEGEMENT
- CORTICOSTERIODS
- IMMUNO SUPRISSIVE AGENTS
- N-acetyl cystein
- Pirfenidone
- Oxygen therapy
- Conclusion
- Slide 36
-
NON RESPIRATORY SYMPTOMS ASSOCIATED WITH DIFFERENT DPLDS
bull Arthritisbull Ocular bull Skin and musclebull GERD bull Lower GI symptoms bull Recurrent sinusitis
bull Neurological symptoms bull Epilepsy amp mental retardation bull Diabetes inspidus
ONSET OF SYMPTOMS
1 Acute presentation (days to weeks)- eg Acute idiopathic interstitial pneumoniHypersensitive pneumonitis
2 Sub-acute presentation (weeks to months)- eg SarcoidosisDrug induced ILD Alveolar hemorrhage syndromes
3 Chronic presentation (months to years) ndash eg IPF
ILD-HISTORY
bullSmokingbullMedication history- Amiodorane Methotrexate
bullOccupational historybullEnvironmental exposure history
OCCUPATIONAL HISTORY1048766 Pneumoconioses ndash miners1048766 Silicosis ndash sand blasters amp granite workers1048766 Asbestosis ndash welders electricians mechanics workers withbrakes shipyard workers1048766 Berylliosis ndash aerospace nuclear computer amp electronic
industries1048766 Hypersensitive pneumonitis ndash farm workers poultry workersbird breeders1048766 The degree of exposure duration latency of exposure and
theuse of protective devices should be elicited
ENVIRONMENTAL EXPOSURE HISTORY
bull 1048766 Exposures to pets (especially birds)bull 1048766 Air conditionersbull 1048766 Humidifiersbull 1048766 Hot tubsbull 1048766 Evaporative cooling systems
ILD-SIGNS
bull Crackles - Dry velcro end inspiratory predominantly bibasilar
bull Inspiratory squeaks- Mid inspiratory high pitched- Seen in Airway centred pathologies
bull Cor pulmonale featuresbull Clubbing ndash IPF
EXTRA PULMONARY -SIGNS
bull Skin abnormalitiesbull Lymphadenopathybull Hepatosplenomegalybull Maculopapular skin rashes bull Erythema nodosum
bull Subcutaneous nodules bull Proximal muscle
weakness bull Arthritis
INVESTIGATIONS
CHEST X RAY
bull Typically small lung volumes with Reticular Nodular or RETICULONODULAR shadow
HIGH-RESOLUTION COMPUTED TOPOGRAPHY (HRCT)
bull HRCT is more sensitivebull Combinations of ground
glass changes reticulonodular shadowing honeycomb cysts and traction
bronchiectasis
PULMONARY FUNCTION TESTING
bull Objective assessment of Resp Symptoms
bull Grading the severity
bull Monitoring the response to therapy
PULMONARY FUNCTION TESTING
bull Restrictive Defect bull darr Lung volumes (TLC FRCRV lt80)bull darr FEV1 FVC With Normal or uarrFEV1FVC
bull Reduced diffusing capacity (DLCO)
LABORATORY INVESTIGATIONS
bull Full blood count lymphopenia in sarcoid eosinophilia in pulmonary eosinophilias and drug reactions neutrophilia in hypersensitivity pneumonitis
bull Ca2+ may be elevated in sarcoidbull Serum angiotensin-converting enzyme non-specific indicator of disease
activity in sarcoidbull ESR and CRP non-specifically raisedbull Autoimmune screen autoantibodies may suggest connectivetissue disease
OTHER INVESTIGATIONS
bull ARTERIAL BLOOD GAS ANALYSIS bull Tuberculin test ndash negative in 23 of sarcoidosis patientsbull BRONCHOALVEOLAR LAVAGE- cellular profile special
stains or studiesbull Lung biopsy- TRANS BRONCHIAL BIOPSY OPEN LUNG
BIOPSY
MANEGEMENTPRINCIPAL AIMS (1) to remove exposure to injurious agents
(2) to suppress inflammation to prevent further destruction of the pulmonary parenchyma
(3) to palliate the manifestations of these diseases
CORTICOSTERIODS
bull Prednisone 1 mgkgbull Gradually tapered (5 mgweek) over several months
to a maintenance dose
IMMUNO SUPRISSIVE AGENTS
Cytotoxic agents (Cyclophosphamide)or immunosuppressive agents (Azathioprine) may be used in patients who do not improve on steroid therapy or who cannot tolerate corticosteroids
N-ACETYL CYSTEIN
bullAntioxidant
bull600 mg PO tid added to prednisone and azathioprine preserves vital capacity and FVC and DLCO
PIRFENIDONE
bullAntifibroticbullReduces acute exacerbations and reduction in FVC
OXYGEN THERAPY
bull F or pa tients w i th d o c u m e nted h y p ox i a ndash S p O 2 lt89 P a O 2 lt55m m H g
bull Improves exerc i se to le rance
CONCLUSION
bull DPLD is the better term not a single disease bull IPF is the most common DPLDbull Restrictive Airway Diseasebull Main treatment aim is to suppress inflammation to prevent
further destruction of the pulmonary parenchyma
Thank you
- Outline
- introduction
- Introduction
- Introduction (2)
- Classification of dpld
- Idiopathic ILD
- Those with identifiable cause
- classification
- Epidemiology
- Ipf - PATHOgenesis VIDEO
- Slide 12
- Respiratory SYMPTOMS
- Non Respiratory symptoms associated with different dplds
- ONSET OF SYMPTOMS
- ILD-HISTORY
- Occupational history
- Environmental exposure history
- ILD-SIGNS
- Extra Pulmonary -SIGNS
- INVESTIGATIONS
- Chest X Ray
- High-resolution Computed topography (HRCT)
- PULMONARY FUNCTION TESTING
- PULMONARY FUNCTION TESTING (2)
- Laboratory investigations
- OTHER INVESTIGATIONS
- Slide 28
- MANEGEMENT
- CORTICOSTERIODS
- IMMUNO SUPRISSIVE AGENTS
- N-acetyl cystein
- Pirfenidone
- Oxygen therapy
- Conclusion
- Slide 36
-
ONSET OF SYMPTOMS
1 Acute presentation (days to weeks)- eg Acute idiopathic interstitial pneumoniHypersensitive pneumonitis
2 Sub-acute presentation (weeks to months)- eg SarcoidosisDrug induced ILD Alveolar hemorrhage syndromes
3 Chronic presentation (months to years) ndash eg IPF
ILD-HISTORY
bullSmokingbullMedication history- Amiodorane Methotrexate
bullOccupational historybullEnvironmental exposure history
OCCUPATIONAL HISTORY1048766 Pneumoconioses ndash miners1048766 Silicosis ndash sand blasters amp granite workers1048766 Asbestosis ndash welders electricians mechanics workers withbrakes shipyard workers1048766 Berylliosis ndash aerospace nuclear computer amp electronic
industries1048766 Hypersensitive pneumonitis ndash farm workers poultry workersbird breeders1048766 The degree of exposure duration latency of exposure and
theuse of protective devices should be elicited
ENVIRONMENTAL EXPOSURE HISTORY
bull 1048766 Exposures to pets (especially birds)bull 1048766 Air conditionersbull 1048766 Humidifiersbull 1048766 Hot tubsbull 1048766 Evaporative cooling systems
ILD-SIGNS
bull Crackles - Dry velcro end inspiratory predominantly bibasilar
bull Inspiratory squeaks- Mid inspiratory high pitched- Seen in Airway centred pathologies
bull Cor pulmonale featuresbull Clubbing ndash IPF
EXTRA PULMONARY -SIGNS
bull Skin abnormalitiesbull Lymphadenopathybull Hepatosplenomegalybull Maculopapular skin rashes bull Erythema nodosum
bull Subcutaneous nodules bull Proximal muscle
weakness bull Arthritis
INVESTIGATIONS
CHEST X RAY
bull Typically small lung volumes with Reticular Nodular or RETICULONODULAR shadow
HIGH-RESOLUTION COMPUTED TOPOGRAPHY (HRCT)
bull HRCT is more sensitivebull Combinations of ground
glass changes reticulonodular shadowing honeycomb cysts and traction
bronchiectasis
PULMONARY FUNCTION TESTING
bull Objective assessment of Resp Symptoms
bull Grading the severity
bull Monitoring the response to therapy
PULMONARY FUNCTION TESTING
bull Restrictive Defect bull darr Lung volumes (TLC FRCRV lt80)bull darr FEV1 FVC With Normal or uarrFEV1FVC
bull Reduced diffusing capacity (DLCO)
LABORATORY INVESTIGATIONS
bull Full blood count lymphopenia in sarcoid eosinophilia in pulmonary eosinophilias and drug reactions neutrophilia in hypersensitivity pneumonitis
bull Ca2+ may be elevated in sarcoidbull Serum angiotensin-converting enzyme non-specific indicator of disease
activity in sarcoidbull ESR and CRP non-specifically raisedbull Autoimmune screen autoantibodies may suggest connectivetissue disease
OTHER INVESTIGATIONS
bull ARTERIAL BLOOD GAS ANALYSIS bull Tuberculin test ndash negative in 23 of sarcoidosis patientsbull BRONCHOALVEOLAR LAVAGE- cellular profile special
stains or studiesbull Lung biopsy- TRANS BRONCHIAL BIOPSY OPEN LUNG
BIOPSY
MANEGEMENTPRINCIPAL AIMS (1) to remove exposure to injurious agents
(2) to suppress inflammation to prevent further destruction of the pulmonary parenchyma
(3) to palliate the manifestations of these diseases
CORTICOSTERIODS
bull Prednisone 1 mgkgbull Gradually tapered (5 mgweek) over several months
to a maintenance dose
IMMUNO SUPRISSIVE AGENTS
Cytotoxic agents (Cyclophosphamide)or immunosuppressive agents (Azathioprine) may be used in patients who do not improve on steroid therapy or who cannot tolerate corticosteroids
N-ACETYL CYSTEIN
bullAntioxidant
bull600 mg PO tid added to prednisone and azathioprine preserves vital capacity and FVC and DLCO
PIRFENIDONE
bullAntifibroticbullReduces acute exacerbations and reduction in FVC
OXYGEN THERAPY
bull F or pa tients w i th d o c u m e nted h y p ox i a ndash S p O 2 lt89 P a O 2 lt55m m H g
bull Improves exerc i se to le rance
CONCLUSION
bull DPLD is the better term not a single disease bull IPF is the most common DPLDbull Restrictive Airway Diseasebull Main treatment aim is to suppress inflammation to prevent
further destruction of the pulmonary parenchyma
Thank you
- Outline
- introduction
- Introduction
- Introduction (2)
- Classification of dpld
- Idiopathic ILD
- Those with identifiable cause
- classification
- Epidemiology
- Ipf - PATHOgenesis VIDEO
- Slide 12
- Respiratory SYMPTOMS
- Non Respiratory symptoms associated with different dplds
- ONSET OF SYMPTOMS
- ILD-HISTORY
- Occupational history
- Environmental exposure history
- ILD-SIGNS
- Extra Pulmonary -SIGNS
- INVESTIGATIONS
- Chest X Ray
- High-resolution Computed topography (HRCT)
- PULMONARY FUNCTION TESTING
- PULMONARY FUNCTION TESTING (2)
- Laboratory investigations
- OTHER INVESTIGATIONS
- Slide 28
- MANEGEMENT
- CORTICOSTERIODS
- IMMUNO SUPRISSIVE AGENTS
- N-acetyl cystein
- Pirfenidone
- Oxygen therapy
- Conclusion
- Slide 36
-
ILD-HISTORY
bullSmokingbullMedication history- Amiodorane Methotrexate
bullOccupational historybullEnvironmental exposure history
OCCUPATIONAL HISTORY1048766 Pneumoconioses ndash miners1048766 Silicosis ndash sand blasters amp granite workers1048766 Asbestosis ndash welders electricians mechanics workers withbrakes shipyard workers1048766 Berylliosis ndash aerospace nuclear computer amp electronic
industries1048766 Hypersensitive pneumonitis ndash farm workers poultry workersbird breeders1048766 The degree of exposure duration latency of exposure and
theuse of protective devices should be elicited
ENVIRONMENTAL EXPOSURE HISTORY
bull 1048766 Exposures to pets (especially birds)bull 1048766 Air conditionersbull 1048766 Humidifiersbull 1048766 Hot tubsbull 1048766 Evaporative cooling systems
ILD-SIGNS
bull Crackles - Dry velcro end inspiratory predominantly bibasilar
bull Inspiratory squeaks- Mid inspiratory high pitched- Seen in Airway centred pathologies
bull Cor pulmonale featuresbull Clubbing ndash IPF
EXTRA PULMONARY -SIGNS
bull Skin abnormalitiesbull Lymphadenopathybull Hepatosplenomegalybull Maculopapular skin rashes bull Erythema nodosum
bull Subcutaneous nodules bull Proximal muscle
weakness bull Arthritis
INVESTIGATIONS
CHEST X RAY
bull Typically small lung volumes with Reticular Nodular or RETICULONODULAR shadow
HIGH-RESOLUTION COMPUTED TOPOGRAPHY (HRCT)
bull HRCT is more sensitivebull Combinations of ground
glass changes reticulonodular shadowing honeycomb cysts and traction
bronchiectasis
PULMONARY FUNCTION TESTING
bull Objective assessment of Resp Symptoms
bull Grading the severity
bull Monitoring the response to therapy
PULMONARY FUNCTION TESTING
bull Restrictive Defect bull darr Lung volumes (TLC FRCRV lt80)bull darr FEV1 FVC With Normal or uarrFEV1FVC
bull Reduced diffusing capacity (DLCO)
LABORATORY INVESTIGATIONS
bull Full blood count lymphopenia in sarcoid eosinophilia in pulmonary eosinophilias and drug reactions neutrophilia in hypersensitivity pneumonitis
bull Ca2+ may be elevated in sarcoidbull Serum angiotensin-converting enzyme non-specific indicator of disease
activity in sarcoidbull ESR and CRP non-specifically raisedbull Autoimmune screen autoantibodies may suggest connectivetissue disease
OTHER INVESTIGATIONS
bull ARTERIAL BLOOD GAS ANALYSIS bull Tuberculin test ndash negative in 23 of sarcoidosis patientsbull BRONCHOALVEOLAR LAVAGE- cellular profile special
stains or studiesbull Lung biopsy- TRANS BRONCHIAL BIOPSY OPEN LUNG
BIOPSY
MANEGEMENTPRINCIPAL AIMS (1) to remove exposure to injurious agents
(2) to suppress inflammation to prevent further destruction of the pulmonary parenchyma
(3) to palliate the manifestations of these diseases
CORTICOSTERIODS
bull Prednisone 1 mgkgbull Gradually tapered (5 mgweek) over several months
to a maintenance dose
IMMUNO SUPRISSIVE AGENTS
Cytotoxic agents (Cyclophosphamide)or immunosuppressive agents (Azathioprine) may be used in patients who do not improve on steroid therapy or who cannot tolerate corticosteroids
N-ACETYL CYSTEIN
bullAntioxidant
bull600 mg PO tid added to prednisone and azathioprine preserves vital capacity and FVC and DLCO
PIRFENIDONE
bullAntifibroticbullReduces acute exacerbations and reduction in FVC
OXYGEN THERAPY
bull F or pa tients w i th d o c u m e nted h y p ox i a ndash S p O 2 lt89 P a O 2 lt55m m H g
bull Improves exerc i se to le rance
CONCLUSION
bull DPLD is the better term not a single disease bull IPF is the most common DPLDbull Restrictive Airway Diseasebull Main treatment aim is to suppress inflammation to prevent
further destruction of the pulmonary parenchyma
Thank you
- Outline
- introduction
- Introduction
- Introduction (2)
- Classification of dpld
- Idiopathic ILD
- Those with identifiable cause
- classification
- Epidemiology
- Ipf - PATHOgenesis VIDEO
- Slide 12
- Respiratory SYMPTOMS
- Non Respiratory symptoms associated with different dplds
- ONSET OF SYMPTOMS
- ILD-HISTORY
- Occupational history
- Environmental exposure history
- ILD-SIGNS
- Extra Pulmonary -SIGNS
- INVESTIGATIONS
- Chest X Ray
- High-resolution Computed topography (HRCT)
- PULMONARY FUNCTION TESTING
- PULMONARY FUNCTION TESTING (2)
- Laboratory investigations
- OTHER INVESTIGATIONS
- Slide 28
- MANEGEMENT
- CORTICOSTERIODS
- IMMUNO SUPRISSIVE AGENTS
- N-acetyl cystein
- Pirfenidone
- Oxygen therapy
- Conclusion
- Slide 36
-
OCCUPATIONAL HISTORY1048766 Pneumoconioses ndash miners1048766 Silicosis ndash sand blasters amp granite workers1048766 Asbestosis ndash welders electricians mechanics workers withbrakes shipyard workers1048766 Berylliosis ndash aerospace nuclear computer amp electronic
industries1048766 Hypersensitive pneumonitis ndash farm workers poultry workersbird breeders1048766 The degree of exposure duration latency of exposure and
theuse of protective devices should be elicited
ENVIRONMENTAL EXPOSURE HISTORY
bull 1048766 Exposures to pets (especially birds)bull 1048766 Air conditionersbull 1048766 Humidifiersbull 1048766 Hot tubsbull 1048766 Evaporative cooling systems
ILD-SIGNS
bull Crackles - Dry velcro end inspiratory predominantly bibasilar
bull Inspiratory squeaks- Mid inspiratory high pitched- Seen in Airway centred pathologies
bull Cor pulmonale featuresbull Clubbing ndash IPF
EXTRA PULMONARY -SIGNS
bull Skin abnormalitiesbull Lymphadenopathybull Hepatosplenomegalybull Maculopapular skin rashes bull Erythema nodosum
bull Subcutaneous nodules bull Proximal muscle
weakness bull Arthritis
INVESTIGATIONS
CHEST X RAY
bull Typically small lung volumes with Reticular Nodular or RETICULONODULAR shadow
HIGH-RESOLUTION COMPUTED TOPOGRAPHY (HRCT)
bull HRCT is more sensitivebull Combinations of ground
glass changes reticulonodular shadowing honeycomb cysts and traction
bronchiectasis
PULMONARY FUNCTION TESTING
bull Objective assessment of Resp Symptoms
bull Grading the severity
bull Monitoring the response to therapy
PULMONARY FUNCTION TESTING
bull Restrictive Defect bull darr Lung volumes (TLC FRCRV lt80)bull darr FEV1 FVC With Normal or uarrFEV1FVC
bull Reduced diffusing capacity (DLCO)
LABORATORY INVESTIGATIONS
bull Full blood count lymphopenia in sarcoid eosinophilia in pulmonary eosinophilias and drug reactions neutrophilia in hypersensitivity pneumonitis
bull Ca2+ may be elevated in sarcoidbull Serum angiotensin-converting enzyme non-specific indicator of disease
activity in sarcoidbull ESR and CRP non-specifically raisedbull Autoimmune screen autoantibodies may suggest connectivetissue disease
OTHER INVESTIGATIONS
bull ARTERIAL BLOOD GAS ANALYSIS bull Tuberculin test ndash negative in 23 of sarcoidosis patientsbull BRONCHOALVEOLAR LAVAGE- cellular profile special
stains or studiesbull Lung biopsy- TRANS BRONCHIAL BIOPSY OPEN LUNG
BIOPSY
MANEGEMENTPRINCIPAL AIMS (1) to remove exposure to injurious agents
(2) to suppress inflammation to prevent further destruction of the pulmonary parenchyma
(3) to palliate the manifestations of these diseases
CORTICOSTERIODS
bull Prednisone 1 mgkgbull Gradually tapered (5 mgweek) over several months
to a maintenance dose
IMMUNO SUPRISSIVE AGENTS
Cytotoxic agents (Cyclophosphamide)or immunosuppressive agents (Azathioprine) may be used in patients who do not improve on steroid therapy or who cannot tolerate corticosteroids
N-ACETYL CYSTEIN
bullAntioxidant
bull600 mg PO tid added to prednisone and azathioprine preserves vital capacity and FVC and DLCO
PIRFENIDONE
bullAntifibroticbullReduces acute exacerbations and reduction in FVC
OXYGEN THERAPY
bull F or pa tients w i th d o c u m e nted h y p ox i a ndash S p O 2 lt89 P a O 2 lt55m m H g
bull Improves exerc i se to le rance
CONCLUSION
bull DPLD is the better term not a single disease bull IPF is the most common DPLDbull Restrictive Airway Diseasebull Main treatment aim is to suppress inflammation to prevent
further destruction of the pulmonary parenchyma
Thank you
- Outline
- introduction
- Introduction
- Introduction (2)
- Classification of dpld
- Idiopathic ILD
- Those with identifiable cause
- classification
- Epidemiology
- Ipf - PATHOgenesis VIDEO
- Slide 12
- Respiratory SYMPTOMS
- Non Respiratory symptoms associated with different dplds
- ONSET OF SYMPTOMS
- ILD-HISTORY
- Occupational history
- Environmental exposure history
- ILD-SIGNS
- Extra Pulmonary -SIGNS
- INVESTIGATIONS
- Chest X Ray
- High-resolution Computed topography (HRCT)
- PULMONARY FUNCTION TESTING
- PULMONARY FUNCTION TESTING (2)
- Laboratory investigations
- OTHER INVESTIGATIONS
- Slide 28
- MANEGEMENT
- CORTICOSTERIODS
- IMMUNO SUPRISSIVE AGENTS
- N-acetyl cystein
- Pirfenidone
- Oxygen therapy
- Conclusion
- Slide 36
-
ENVIRONMENTAL EXPOSURE HISTORY
bull 1048766 Exposures to pets (especially birds)bull 1048766 Air conditionersbull 1048766 Humidifiersbull 1048766 Hot tubsbull 1048766 Evaporative cooling systems
ILD-SIGNS
bull Crackles - Dry velcro end inspiratory predominantly bibasilar
bull Inspiratory squeaks- Mid inspiratory high pitched- Seen in Airway centred pathologies
bull Cor pulmonale featuresbull Clubbing ndash IPF
EXTRA PULMONARY -SIGNS
bull Skin abnormalitiesbull Lymphadenopathybull Hepatosplenomegalybull Maculopapular skin rashes bull Erythema nodosum
bull Subcutaneous nodules bull Proximal muscle
weakness bull Arthritis
INVESTIGATIONS
CHEST X RAY
bull Typically small lung volumes with Reticular Nodular or RETICULONODULAR shadow
HIGH-RESOLUTION COMPUTED TOPOGRAPHY (HRCT)
bull HRCT is more sensitivebull Combinations of ground
glass changes reticulonodular shadowing honeycomb cysts and traction
bronchiectasis
PULMONARY FUNCTION TESTING
bull Objective assessment of Resp Symptoms
bull Grading the severity
bull Monitoring the response to therapy
PULMONARY FUNCTION TESTING
bull Restrictive Defect bull darr Lung volumes (TLC FRCRV lt80)bull darr FEV1 FVC With Normal or uarrFEV1FVC
bull Reduced diffusing capacity (DLCO)
LABORATORY INVESTIGATIONS
bull Full blood count lymphopenia in sarcoid eosinophilia in pulmonary eosinophilias and drug reactions neutrophilia in hypersensitivity pneumonitis
bull Ca2+ may be elevated in sarcoidbull Serum angiotensin-converting enzyme non-specific indicator of disease
activity in sarcoidbull ESR and CRP non-specifically raisedbull Autoimmune screen autoantibodies may suggest connectivetissue disease
OTHER INVESTIGATIONS
bull ARTERIAL BLOOD GAS ANALYSIS bull Tuberculin test ndash negative in 23 of sarcoidosis patientsbull BRONCHOALVEOLAR LAVAGE- cellular profile special
stains or studiesbull Lung biopsy- TRANS BRONCHIAL BIOPSY OPEN LUNG
BIOPSY
MANEGEMENTPRINCIPAL AIMS (1) to remove exposure to injurious agents
(2) to suppress inflammation to prevent further destruction of the pulmonary parenchyma
(3) to palliate the manifestations of these diseases
CORTICOSTERIODS
bull Prednisone 1 mgkgbull Gradually tapered (5 mgweek) over several months
to a maintenance dose
IMMUNO SUPRISSIVE AGENTS
Cytotoxic agents (Cyclophosphamide)or immunosuppressive agents (Azathioprine) may be used in patients who do not improve on steroid therapy or who cannot tolerate corticosteroids
N-ACETYL CYSTEIN
bullAntioxidant
bull600 mg PO tid added to prednisone and azathioprine preserves vital capacity and FVC and DLCO
PIRFENIDONE
bullAntifibroticbullReduces acute exacerbations and reduction in FVC
OXYGEN THERAPY
bull F or pa tients w i th d o c u m e nted h y p ox i a ndash S p O 2 lt89 P a O 2 lt55m m H g
bull Improves exerc i se to le rance
CONCLUSION
bull DPLD is the better term not a single disease bull IPF is the most common DPLDbull Restrictive Airway Diseasebull Main treatment aim is to suppress inflammation to prevent
further destruction of the pulmonary parenchyma
Thank you
- Outline
- introduction
- Introduction
- Introduction (2)
- Classification of dpld
- Idiopathic ILD
- Those with identifiable cause
- classification
- Epidemiology
- Ipf - PATHOgenesis VIDEO
- Slide 12
- Respiratory SYMPTOMS
- Non Respiratory symptoms associated with different dplds
- ONSET OF SYMPTOMS
- ILD-HISTORY
- Occupational history
- Environmental exposure history
- ILD-SIGNS
- Extra Pulmonary -SIGNS
- INVESTIGATIONS
- Chest X Ray
- High-resolution Computed topography (HRCT)
- PULMONARY FUNCTION TESTING
- PULMONARY FUNCTION TESTING (2)
- Laboratory investigations
- OTHER INVESTIGATIONS
- Slide 28
- MANEGEMENT
- CORTICOSTERIODS
- IMMUNO SUPRISSIVE AGENTS
- N-acetyl cystein
- Pirfenidone
- Oxygen therapy
- Conclusion
- Slide 36
-
ILD-SIGNS
bull Crackles - Dry velcro end inspiratory predominantly bibasilar
bull Inspiratory squeaks- Mid inspiratory high pitched- Seen in Airway centred pathologies
bull Cor pulmonale featuresbull Clubbing ndash IPF
EXTRA PULMONARY -SIGNS
bull Skin abnormalitiesbull Lymphadenopathybull Hepatosplenomegalybull Maculopapular skin rashes bull Erythema nodosum
bull Subcutaneous nodules bull Proximal muscle
weakness bull Arthritis
INVESTIGATIONS
CHEST X RAY
bull Typically small lung volumes with Reticular Nodular or RETICULONODULAR shadow
HIGH-RESOLUTION COMPUTED TOPOGRAPHY (HRCT)
bull HRCT is more sensitivebull Combinations of ground
glass changes reticulonodular shadowing honeycomb cysts and traction
bronchiectasis
PULMONARY FUNCTION TESTING
bull Objective assessment of Resp Symptoms
bull Grading the severity
bull Monitoring the response to therapy
PULMONARY FUNCTION TESTING
bull Restrictive Defect bull darr Lung volumes (TLC FRCRV lt80)bull darr FEV1 FVC With Normal or uarrFEV1FVC
bull Reduced diffusing capacity (DLCO)
LABORATORY INVESTIGATIONS
bull Full blood count lymphopenia in sarcoid eosinophilia in pulmonary eosinophilias and drug reactions neutrophilia in hypersensitivity pneumonitis
bull Ca2+ may be elevated in sarcoidbull Serum angiotensin-converting enzyme non-specific indicator of disease
activity in sarcoidbull ESR and CRP non-specifically raisedbull Autoimmune screen autoantibodies may suggest connectivetissue disease
OTHER INVESTIGATIONS
bull ARTERIAL BLOOD GAS ANALYSIS bull Tuberculin test ndash negative in 23 of sarcoidosis patientsbull BRONCHOALVEOLAR LAVAGE- cellular profile special
stains or studiesbull Lung biopsy- TRANS BRONCHIAL BIOPSY OPEN LUNG
BIOPSY
MANEGEMENTPRINCIPAL AIMS (1) to remove exposure to injurious agents
(2) to suppress inflammation to prevent further destruction of the pulmonary parenchyma
(3) to palliate the manifestations of these diseases
CORTICOSTERIODS
bull Prednisone 1 mgkgbull Gradually tapered (5 mgweek) over several months
to a maintenance dose
IMMUNO SUPRISSIVE AGENTS
Cytotoxic agents (Cyclophosphamide)or immunosuppressive agents (Azathioprine) may be used in patients who do not improve on steroid therapy or who cannot tolerate corticosteroids
N-ACETYL CYSTEIN
bullAntioxidant
bull600 mg PO tid added to prednisone and azathioprine preserves vital capacity and FVC and DLCO
PIRFENIDONE
bullAntifibroticbullReduces acute exacerbations and reduction in FVC
OXYGEN THERAPY
bull F or pa tients w i th d o c u m e nted h y p ox i a ndash S p O 2 lt89 P a O 2 lt55m m H g
bull Improves exerc i se to le rance
CONCLUSION
bull DPLD is the better term not a single disease bull IPF is the most common DPLDbull Restrictive Airway Diseasebull Main treatment aim is to suppress inflammation to prevent
further destruction of the pulmonary parenchyma
Thank you
- Outline
- introduction
- Introduction
- Introduction (2)
- Classification of dpld
- Idiopathic ILD
- Those with identifiable cause
- classification
- Epidemiology
- Ipf - PATHOgenesis VIDEO
- Slide 12
- Respiratory SYMPTOMS
- Non Respiratory symptoms associated with different dplds
- ONSET OF SYMPTOMS
- ILD-HISTORY
- Occupational history
- Environmental exposure history
- ILD-SIGNS
- Extra Pulmonary -SIGNS
- INVESTIGATIONS
- Chest X Ray
- High-resolution Computed topography (HRCT)
- PULMONARY FUNCTION TESTING
- PULMONARY FUNCTION TESTING (2)
- Laboratory investigations
- OTHER INVESTIGATIONS
- Slide 28
- MANEGEMENT
- CORTICOSTERIODS
- IMMUNO SUPRISSIVE AGENTS
- N-acetyl cystein
- Pirfenidone
- Oxygen therapy
- Conclusion
- Slide 36
-
EXTRA PULMONARY -SIGNS
bull Skin abnormalitiesbull Lymphadenopathybull Hepatosplenomegalybull Maculopapular skin rashes bull Erythema nodosum
bull Subcutaneous nodules bull Proximal muscle
weakness bull Arthritis
INVESTIGATIONS
CHEST X RAY
bull Typically small lung volumes with Reticular Nodular or RETICULONODULAR shadow
HIGH-RESOLUTION COMPUTED TOPOGRAPHY (HRCT)
bull HRCT is more sensitivebull Combinations of ground
glass changes reticulonodular shadowing honeycomb cysts and traction
bronchiectasis
PULMONARY FUNCTION TESTING
bull Objective assessment of Resp Symptoms
bull Grading the severity
bull Monitoring the response to therapy
PULMONARY FUNCTION TESTING
bull Restrictive Defect bull darr Lung volumes (TLC FRCRV lt80)bull darr FEV1 FVC With Normal or uarrFEV1FVC
bull Reduced diffusing capacity (DLCO)
LABORATORY INVESTIGATIONS
bull Full blood count lymphopenia in sarcoid eosinophilia in pulmonary eosinophilias and drug reactions neutrophilia in hypersensitivity pneumonitis
bull Ca2+ may be elevated in sarcoidbull Serum angiotensin-converting enzyme non-specific indicator of disease
activity in sarcoidbull ESR and CRP non-specifically raisedbull Autoimmune screen autoantibodies may suggest connectivetissue disease
OTHER INVESTIGATIONS
bull ARTERIAL BLOOD GAS ANALYSIS bull Tuberculin test ndash negative in 23 of sarcoidosis patientsbull BRONCHOALVEOLAR LAVAGE- cellular profile special
stains or studiesbull Lung biopsy- TRANS BRONCHIAL BIOPSY OPEN LUNG
BIOPSY
MANEGEMENTPRINCIPAL AIMS (1) to remove exposure to injurious agents
(2) to suppress inflammation to prevent further destruction of the pulmonary parenchyma
(3) to palliate the manifestations of these diseases
CORTICOSTERIODS
bull Prednisone 1 mgkgbull Gradually tapered (5 mgweek) over several months
to a maintenance dose
IMMUNO SUPRISSIVE AGENTS
Cytotoxic agents (Cyclophosphamide)or immunosuppressive agents (Azathioprine) may be used in patients who do not improve on steroid therapy or who cannot tolerate corticosteroids
N-ACETYL CYSTEIN
bullAntioxidant
bull600 mg PO tid added to prednisone and azathioprine preserves vital capacity and FVC and DLCO
PIRFENIDONE
bullAntifibroticbullReduces acute exacerbations and reduction in FVC
OXYGEN THERAPY
bull F or pa tients w i th d o c u m e nted h y p ox i a ndash S p O 2 lt89 P a O 2 lt55m m H g
bull Improves exerc i se to le rance
CONCLUSION
bull DPLD is the better term not a single disease bull IPF is the most common DPLDbull Restrictive Airway Diseasebull Main treatment aim is to suppress inflammation to prevent
further destruction of the pulmonary parenchyma
Thank you
- Outline
- introduction
- Introduction
- Introduction (2)
- Classification of dpld
- Idiopathic ILD
- Those with identifiable cause
- classification
- Epidemiology
- Ipf - PATHOgenesis VIDEO
- Slide 12
- Respiratory SYMPTOMS
- Non Respiratory symptoms associated with different dplds
- ONSET OF SYMPTOMS
- ILD-HISTORY
- Occupational history
- Environmental exposure history
- ILD-SIGNS
- Extra Pulmonary -SIGNS
- INVESTIGATIONS
- Chest X Ray
- High-resolution Computed topography (HRCT)
- PULMONARY FUNCTION TESTING
- PULMONARY FUNCTION TESTING (2)
- Laboratory investigations
- OTHER INVESTIGATIONS
- Slide 28
- MANEGEMENT
- CORTICOSTERIODS
- IMMUNO SUPRISSIVE AGENTS
- N-acetyl cystein
- Pirfenidone
- Oxygen therapy
- Conclusion
- Slide 36
-
INVESTIGATIONS
CHEST X RAY
bull Typically small lung volumes with Reticular Nodular or RETICULONODULAR shadow
HIGH-RESOLUTION COMPUTED TOPOGRAPHY (HRCT)
bull HRCT is more sensitivebull Combinations of ground
glass changes reticulonodular shadowing honeycomb cysts and traction
bronchiectasis
PULMONARY FUNCTION TESTING
bull Objective assessment of Resp Symptoms
bull Grading the severity
bull Monitoring the response to therapy
PULMONARY FUNCTION TESTING
bull Restrictive Defect bull darr Lung volumes (TLC FRCRV lt80)bull darr FEV1 FVC With Normal or uarrFEV1FVC
bull Reduced diffusing capacity (DLCO)
LABORATORY INVESTIGATIONS
bull Full blood count lymphopenia in sarcoid eosinophilia in pulmonary eosinophilias and drug reactions neutrophilia in hypersensitivity pneumonitis
bull Ca2+ may be elevated in sarcoidbull Serum angiotensin-converting enzyme non-specific indicator of disease
activity in sarcoidbull ESR and CRP non-specifically raisedbull Autoimmune screen autoantibodies may suggest connectivetissue disease
OTHER INVESTIGATIONS
bull ARTERIAL BLOOD GAS ANALYSIS bull Tuberculin test ndash negative in 23 of sarcoidosis patientsbull BRONCHOALVEOLAR LAVAGE- cellular profile special
stains or studiesbull Lung biopsy- TRANS BRONCHIAL BIOPSY OPEN LUNG
BIOPSY
MANEGEMENTPRINCIPAL AIMS (1) to remove exposure to injurious agents
(2) to suppress inflammation to prevent further destruction of the pulmonary parenchyma
(3) to palliate the manifestations of these diseases
CORTICOSTERIODS
bull Prednisone 1 mgkgbull Gradually tapered (5 mgweek) over several months
to a maintenance dose
IMMUNO SUPRISSIVE AGENTS
Cytotoxic agents (Cyclophosphamide)or immunosuppressive agents (Azathioprine) may be used in patients who do not improve on steroid therapy or who cannot tolerate corticosteroids
N-ACETYL CYSTEIN
bullAntioxidant
bull600 mg PO tid added to prednisone and azathioprine preserves vital capacity and FVC and DLCO
PIRFENIDONE
bullAntifibroticbullReduces acute exacerbations and reduction in FVC
OXYGEN THERAPY
bull F or pa tients w i th d o c u m e nted h y p ox i a ndash S p O 2 lt89 P a O 2 lt55m m H g
bull Improves exerc i se to le rance
CONCLUSION
bull DPLD is the better term not a single disease bull IPF is the most common DPLDbull Restrictive Airway Diseasebull Main treatment aim is to suppress inflammation to prevent
further destruction of the pulmonary parenchyma
Thank you
- Outline
- introduction
- Introduction
- Introduction (2)
- Classification of dpld
- Idiopathic ILD
- Those with identifiable cause
- classification
- Epidemiology
- Ipf - PATHOgenesis VIDEO
- Slide 12
- Respiratory SYMPTOMS
- Non Respiratory symptoms associated with different dplds
- ONSET OF SYMPTOMS
- ILD-HISTORY
- Occupational history
- Environmental exposure history
- ILD-SIGNS
- Extra Pulmonary -SIGNS
- INVESTIGATIONS
- Chest X Ray
- High-resolution Computed topography (HRCT)
- PULMONARY FUNCTION TESTING
- PULMONARY FUNCTION TESTING (2)
- Laboratory investigations
- OTHER INVESTIGATIONS
- Slide 28
- MANEGEMENT
- CORTICOSTERIODS
- IMMUNO SUPRISSIVE AGENTS
- N-acetyl cystein
- Pirfenidone
- Oxygen therapy
- Conclusion
- Slide 36
-
CHEST X RAY
bull Typically small lung volumes with Reticular Nodular or RETICULONODULAR shadow
HIGH-RESOLUTION COMPUTED TOPOGRAPHY (HRCT)
bull HRCT is more sensitivebull Combinations of ground
glass changes reticulonodular shadowing honeycomb cysts and traction
bronchiectasis
PULMONARY FUNCTION TESTING
bull Objective assessment of Resp Symptoms
bull Grading the severity
bull Monitoring the response to therapy
PULMONARY FUNCTION TESTING
bull Restrictive Defect bull darr Lung volumes (TLC FRCRV lt80)bull darr FEV1 FVC With Normal or uarrFEV1FVC
bull Reduced diffusing capacity (DLCO)
LABORATORY INVESTIGATIONS
bull Full blood count lymphopenia in sarcoid eosinophilia in pulmonary eosinophilias and drug reactions neutrophilia in hypersensitivity pneumonitis
bull Ca2+ may be elevated in sarcoidbull Serum angiotensin-converting enzyme non-specific indicator of disease
activity in sarcoidbull ESR and CRP non-specifically raisedbull Autoimmune screen autoantibodies may suggest connectivetissue disease
OTHER INVESTIGATIONS
bull ARTERIAL BLOOD GAS ANALYSIS bull Tuberculin test ndash negative in 23 of sarcoidosis patientsbull BRONCHOALVEOLAR LAVAGE- cellular profile special
stains or studiesbull Lung biopsy- TRANS BRONCHIAL BIOPSY OPEN LUNG
BIOPSY
MANEGEMENTPRINCIPAL AIMS (1) to remove exposure to injurious agents
(2) to suppress inflammation to prevent further destruction of the pulmonary parenchyma
(3) to palliate the manifestations of these diseases
CORTICOSTERIODS
bull Prednisone 1 mgkgbull Gradually tapered (5 mgweek) over several months
to a maintenance dose
IMMUNO SUPRISSIVE AGENTS
Cytotoxic agents (Cyclophosphamide)or immunosuppressive agents (Azathioprine) may be used in patients who do not improve on steroid therapy or who cannot tolerate corticosteroids
N-ACETYL CYSTEIN
bullAntioxidant
bull600 mg PO tid added to prednisone and azathioprine preserves vital capacity and FVC and DLCO
PIRFENIDONE
bullAntifibroticbullReduces acute exacerbations and reduction in FVC
OXYGEN THERAPY
bull F or pa tients w i th d o c u m e nted h y p ox i a ndash S p O 2 lt89 P a O 2 lt55m m H g
bull Improves exerc i se to le rance
CONCLUSION
bull DPLD is the better term not a single disease bull IPF is the most common DPLDbull Restrictive Airway Diseasebull Main treatment aim is to suppress inflammation to prevent
further destruction of the pulmonary parenchyma
Thank you
- Outline
- introduction
- Introduction
- Introduction (2)
- Classification of dpld
- Idiopathic ILD
- Those with identifiable cause
- classification
- Epidemiology
- Ipf - PATHOgenesis VIDEO
- Slide 12
- Respiratory SYMPTOMS
- Non Respiratory symptoms associated with different dplds
- ONSET OF SYMPTOMS
- ILD-HISTORY
- Occupational history
- Environmental exposure history
- ILD-SIGNS
- Extra Pulmonary -SIGNS
- INVESTIGATIONS
- Chest X Ray
- High-resolution Computed topography (HRCT)
- PULMONARY FUNCTION TESTING
- PULMONARY FUNCTION TESTING (2)
- Laboratory investigations
- OTHER INVESTIGATIONS
- Slide 28
- MANEGEMENT
- CORTICOSTERIODS
- IMMUNO SUPRISSIVE AGENTS
- N-acetyl cystein
- Pirfenidone
- Oxygen therapy
- Conclusion
- Slide 36
-
HIGH-RESOLUTION COMPUTED TOPOGRAPHY (HRCT)
bull HRCT is more sensitivebull Combinations of ground
glass changes reticulonodular shadowing honeycomb cysts and traction
bronchiectasis
PULMONARY FUNCTION TESTING
bull Objective assessment of Resp Symptoms
bull Grading the severity
bull Monitoring the response to therapy
PULMONARY FUNCTION TESTING
bull Restrictive Defect bull darr Lung volumes (TLC FRCRV lt80)bull darr FEV1 FVC With Normal or uarrFEV1FVC
bull Reduced diffusing capacity (DLCO)
LABORATORY INVESTIGATIONS
bull Full blood count lymphopenia in sarcoid eosinophilia in pulmonary eosinophilias and drug reactions neutrophilia in hypersensitivity pneumonitis
bull Ca2+ may be elevated in sarcoidbull Serum angiotensin-converting enzyme non-specific indicator of disease
activity in sarcoidbull ESR and CRP non-specifically raisedbull Autoimmune screen autoantibodies may suggest connectivetissue disease
OTHER INVESTIGATIONS
bull ARTERIAL BLOOD GAS ANALYSIS bull Tuberculin test ndash negative in 23 of sarcoidosis patientsbull BRONCHOALVEOLAR LAVAGE- cellular profile special
stains or studiesbull Lung biopsy- TRANS BRONCHIAL BIOPSY OPEN LUNG
BIOPSY
MANEGEMENTPRINCIPAL AIMS (1) to remove exposure to injurious agents
(2) to suppress inflammation to prevent further destruction of the pulmonary parenchyma
(3) to palliate the manifestations of these diseases
CORTICOSTERIODS
bull Prednisone 1 mgkgbull Gradually tapered (5 mgweek) over several months
to a maintenance dose
IMMUNO SUPRISSIVE AGENTS
Cytotoxic agents (Cyclophosphamide)or immunosuppressive agents (Azathioprine) may be used in patients who do not improve on steroid therapy or who cannot tolerate corticosteroids
N-ACETYL CYSTEIN
bullAntioxidant
bull600 mg PO tid added to prednisone and azathioprine preserves vital capacity and FVC and DLCO
PIRFENIDONE
bullAntifibroticbullReduces acute exacerbations and reduction in FVC
OXYGEN THERAPY
bull F or pa tients w i th d o c u m e nted h y p ox i a ndash S p O 2 lt89 P a O 2 lt55m m H g
bull Improves exerc i se to le rance
CONCLUSION
bull DPLD is the better term not a single disease bull IPF is the most common DPLDbull Restrictive Airway Diseasebull Main treatment aim is to suppress inflammation to prevent
further destruction of the pulmonary parenchyma
Thank you
- Outline
- introduction
- Introduction
- Introduction (2)
- Classification of dpld
- Idiopathic ILD
- Those with identifiable cause
- classification
- Epidemiology
- Ipf - PATHOgenesis VIDEO
- Slide 12
- Respiratory SYMPTOMS
- Non Respiratory symptoms associated with different dplds
- ONSET OF SYMPTOMS
- ILD-HISTORY
- Occupational history
- Environmental exposure history
- ILD-SIGNS
- Extra Pulmonary -SIGNS
- INVESTIGATIONS
- Chest X Ray
- High-resolution Computed topography (HRCT)
- PULMONARY FUNCTION TESTING
- PULMONARY FUNCTION TESTING (2)
- Laboratory investigations
- OTHER INVESTIGATIONS
- Slide 28
- MANEGEMENT
- CORTICOSTERIODS
- IMMUNO SUPRISSIVE AGENTS
- N-acetyl cystein
- Pirfenidone
- Oxygen therapy
- Conclusion
- Slide 36
-
PULMONARY FUNCTION TESTING
bull Objective assessment of Resp Symptoms
bull Grading the severity
bull Monitoring the response to therapy
PULMONARY FUNCTION TESTING
bull Restrictive Defect bull darr Lung volumes (TLC FRCRV lt80)bull darr FEV1 FVC With Normal or uarrFEV1FVC
bull Reduced diffusing capacity (DLCO)
LABORATORY INVESTIGATIONS
bull Full blood count lymphopenia in sarcoid eosinophilia in pulmonary eosinophilias and drug reactions neutrophilia in hypersensitivity pneumonitis
bull Ca2+ may be elevated in sarcoidbull Serum angiotensin-converting enzyme non-specific indicator of disease
activity in sarcoidbull ESR and CRP non-specifically raisedbull Autoimmune screen autoantibodies may suggest connectivetissue disease
OTHER INVESTIGATIONS
bull ARTERIAL BLOOD GAS ANALYSIS bull Tuberculin test ndash negative in 23 of sarcoidosis patientsbull BRONCHOALVEOLAR LAVAGE- cellular profile special
stains or studiesbull Lung biopsy- TRANS BRONCHIAL BIOPSY OPEN LUNG
BIOPSY
MANEGEMENTPRINCIPAL AIMS (1) to remove exposure to injurious agents
(2) to suppress inflammation to prevent further destruction of the pulmonary parenchyma
(3) to palliate the manifestations of these diseases
CORTICOSTERIODS
bull Prednisone 1 mgkgbull Gradually tapered (5 mgweek) over several months
to a maintenance dose
IMMUNO SUPRISSIVE AGENTS
Cytotoxic agents (Cyclophosphamide)or immunosuppressive agents (Azathioprine) may be used in patients who do not improve on steroid therapy or who cannot tolerate corticosteroids
N-ACETYL CYSTEIN
bullAntioxidant
bull600 mg PO tid added to prednisone and azathioprine preserves vital capacity and FVC and DLCO
PIRFENIDONE
bullAntifibroticbullReduces acute exacerbations and reduction in FVC
OXYGEN THERAPY
bull F or pa tients w i th d o c u m e nted h y p ox i a ndash S p O 2 lt89 P a O 2 lt55m m H g
bull Improves exerc i se to le rance
CONCLUSION
bull DPLD is the better term not a single disease bull IPF is the most common DPLDbull Restrictive Airway Diseasebull Main treatment aim is to suppress inflammation to prevent
further destruction of the pulmonary parenchyma
Thank you
- Outline
- introduction
- Introduction
- Introduction (2)
- Classification of dpld
- Idiopathic ILD
- Those with identifiable cause
- classification
- Epidemiology
- Ipf - PATHOgenesis VIDEO
- Slide 12
- Respiratory SYMPTOMS
- Non Respiratory symptoms associated with different dplds
- ONSET OF SYMPTOMS
- ILD-HISTORY
- Occupational history
- Environmental exposure history
- ILD-SIGNS
- Extra Pulmonary -SIGNS
- INVESTIGATIONS
- Chest X Ray
- High-resolution Computed topography (HRCT)
- PULMONARY FUNCTION TESTING
- PULMONARY FUNCTION TESTING (2)
- Laboratory investigations
- OTHER INVESTIGATIONS
- Slide 28
- MANEGEMENT
- CORTICOSTERIODS
- IMMUNO SUPRISSIVE AGENTS
- N-acetyl cystein
- Pirfenidone
- Oxygen therapy
- Conclusion
- Slide 36
-
PULMONARY FUNCTION TESTING
bull Restrictive Defect bull darr Lung volumes (TLC FRCRV lt80)bull darr FEV1 FVC With Normal or uarrFEV1FVC
bull Reduced diffusing capacity (DLCO)
LABORATORY INVESTIGATIONS
bull Full blood count lymphopenia in sarcoid eosinophilia in pulmonary eosinophilias and drug reactions neutrophilia in hypersensitivity pneumonitis
bull Ca2+ may be elevated in sarcoidbull Serum angiotensin-converting enzyme non-specific indicator of disease
activity in sarcoidbull ESR and CRP non-specifically raisedbull Autoimmune screen autoantibodies may suggest connectivetissue disease
OTHER INVESTIGATIONS
bull ARTERIAL BLOOD GAS ANALYSIS bull Tuberculin test ndash negative in 23 of sarcoidosis patientsbull BRONCHOALVEOLAR LAVAGE- cellular profile special
stains or studiesbull Lung biopsy- TRANS BRONCHIAL BIOPSY OPEN LUNG
BIOPSY
MANEGEMENTPRINCIPAL AIMS (1) to remove exposure to injurious agents
(2) to suppress inflammation to prevent further destruction of the pulmonary parenchyma
(3) to palliate the manifestations of these diseases
CORTICOSTERIODS
bull Prednisone 1 mgkgbull Gradually tapered (5 mgweek) over several months
to a maintenance dose
IMMUNO SUPRISSIVE AGENTS
Cytotoxic agents (Cyclophosphamide)or immunosuppressive agents (Azathioprine) may be used in patients who do not improve on steroid therapy or who cannot tolerate corticosteroids
N-ACETYL CYSTEIN
bullAntioxidant
bull600 mg PO tid added to prednisone and azathioprine preserves vital capacity and FVC and DLCO
PIRFENIDONE
bullAntifibroticbullReduces acute exacerbations and reduction in FVC
OXYGEN THERAPY
bull F or pa tients w i th d o c u m e nted h y p ox i a ndash S p O 2 lt89 P a O 2 lt55m m H g
bull Improves exerc i se to le rance
CONCLUSION
bull DPLD is the better term not a single disease bull IPF is the most common DPLDbull Restrictive Airway Diseasebull Main treatment aim is to suppress inflammation to prevent
further destruction of the pulmonary parenchyma
Thank you
- Outline
- introduction
- Introduction
- Introduction (2)
- Classification of dpld
- Idiopathic ILD
- Those with identifiable cause
- classification
- Epidemiology
- Ipf - PATHOgenesis VIDEO
- Slide 12
- Respiratory SYMPTOMS
- Non Respiratory symptoms associated with different dplds
- ONSET OF SYMPTOMS
- ILD-HISTORY
- Occupational history
- Environmental exposure history
- ILD-SIGNS
- Extra Pulmonary -SIGNS
- INVESTIGATIONS
- Chest X Ray
- High-resolution Computed topography (HRCT)
- PULMONARY FUNCTION TESTING
- PULMONARY FUNCTION TESTING (2)
- Laboratory investigations
- OTHER INVESTIGATIONS
- Slide 28
- MANEGEMENT
- CORTICOSTERIODS
- IMMUNO SUPRISSIVE AGENTS
- N-acetyl cystein
- Pirfenidone
- Oxygen therapy
- Conclusion
- Slide 36
-
LABORATORY INVESTIGATIONS
bull Full blood count lymphopenia in sarcoid eosinophilia in pulmonary eosinophilias and drug reactions neutrophilia in hypersensitivity pneumonitis
bull Ca2+ may be elevated in sarcoidbull Serum angiotensin-converting enzyme non-specific indicator of disease
activity in sarcoidbull ESR and CRP non-specifically raisedbull Autoimmune screen autoantibodies may suggest connectivetissue disease
OTHER INVESTIGATIONS
bull ARTERIAL BLOOD GAS ANALYSIS bull Tuberculin test ndash negative in 23 of sarcoidosis patientsbull BRONCHOALVEOLAR LAVAGE- cellular profile special
stains or studiesbull Lung biopsy- TRANS BRONCHIAL BIOPSY OPEN LUNG
BIOPSY
MANEGEMENTPRINCIPAL AIMS (1) to remove exposure to injurious agents
(2) to suppress inflammation to prevent further destruction of the pulmonary parenchyma
(3) to palliate the manifestations of these diseases
CORTICOSTERIODS
bull Prednisone 1 mgkgbull Gradually tapered (5 mgweek) over several months
to a maintenance dose
IMMUNO SUPRISSIVE AGENTS
Cytotoxic agents (Cyclophosphamide)or immunosuppressive agents (Azathioprine) may be used in patients who do not improve on steroid therapy or who cannot tolerate corticosteroids
N-ACETYL CYSTEIN
bullAntioxidant
bull600 mg PO tid added to prednisone and azathioprine preserves vital capacity and FVC and DLCO
PIRFENIDONE
bullAntifibroticbullReduces acute exacerbations and reduction in FVC
OXYGEN THERAPY
bull F or pa tients w i th d o c u m e nted h y p ox i a ndash S p O 2 lt89 P a O 2 lt55m m H g
bull Improves exerc i se to le rance
CONCLUSION
bull DPLD is the better term not a single disease bull IPF is the most common DPLDbull Restrictive Airway Diseasebull Main treatment aim is to suppress inflammation to prevent
further destruction of the pulmonary parenchyma
Thank you
- Outline
- introduction
- Introduction
- Introduction (2)
- Classification of dpld
- Idiopathic ILD
- Those with identifiable cause
- classification
- Epidemiology
- Ipf - PATHOgenesis VIDEO
- Slide 12
- Respiratory SYMPTOMS
- Non Respiratory symptoms associated with different dplds
- ONSET OF SYMPTOMS
- ILD-HISTORY
- Occupational history
- Environmental exposure history
- ILD-SIGNS
- Extra Pulmonary -SIGNS
- INVESTIGATIONS
- Chest X Ray
- High-resolution Computed topography (HRCT)
- PULMONARY FUNCTION TESTING
- PULMONARY FUNCTION TESTING (2)
- Laboratory investigations
- OTHER INVESTIGATIONS
- Slide 28
- MANEGEMENT
- CORTICOSTERIODS
- IMMUNO SUPRISSIVE AGENTS
- N-acetyl cystein
- Pirfenidone
- Oxygen therapy
- Conclusion
- Slide 36
-
OTHER INVESTIGATIONS
bull ARTERIAL BLOOD GAS ANALYSIS bull Tuberculin test ndash negative in 23 of sarcoidosis patientsbull BRONCHOALVEOLAR LAVAGE- cellular profile special
stains or studiesbull Lung biopsy- TRANS BRONCHIAL BIOPSY OPEN LUNG
BIOPSY
MANEGEMENTPRINCIPAL AIMS (1) to remove exposure to injurious agents
(2) to suppress inflammation to prevent further destruction of the pulmonary parenchyma
(3) to palliate the manifestations of these diseases
CORTICOSTERIODS
bull Prednisone 1 mgkgbull Gradually tapered (5 mgweek) over several months
to a maintenance dose
IMMUNO SUPRISSIVE AGENTS
Cytotoxic agents (Cyclophosphamide)or immunosuppressive agents (Azathioprine) may be used in patients who do not improve on steroid therapy or who cannot tolerate corticosteroids
N-ACETYL CYSTEIN
bullAntioxidant
bull600 mg PO tid added to prednisone and azathioprine preserves vital capacity and FVC and DLCO
PIRFENIDONE
bullAntifibroticbullReduces acute exacerbations and reduction in FVC
OXYGEN THERAPY
bull F or pa tients w i th d o c u m e nted h y p ox i a ndash S p O 2 lt89 P a O 2 lt55m m H g
bull Improves exerc i se to le rance
CONCLUSION
bull DPLD is the better term not a single disease bull IPF is the most common DPLDbull Restrictive Airway Diseasebull Main treatment aim is to suppress inflammation to prevent
further destruction of the pulmonary parenchyma
Thank you
- Outline
- introduction
- Introduction
- Introduction (2)
- Classification of dpld
- Idiopathic ILD
- Those with identifiable cause
- classification
- Epidemiology
- Ipf - PATHOgenesis VIDEO
- Slide 12
- Respiratory SYMPTOMS
- Non Respiratory symptoms associated with different dplds
- ONSET OF SYMPTOMS
- ILD-HISTORY
- Occupational history
- Environmental exposure history
- ILD-SIGNS
- Extra Pulmonary -SIGNS
- INVESTIGATIONS
- Chest X Ray
- High-resolution Computed topography (HRCT)
- PULMONARY FUNCTION TESTING
- PULMONARY FUNCTION TESTING (2)
- Laboratory investigations
- OTHER INVESTIGATIONS
- Slide 28
- MANEGEMENT
- CORTICOSTERIODS
- IMMUNO SUPRISSIVE AGENTS
- N-acetyl cystein
- Pirfenidone
- Oxygen therapy
- Conclusion
- Slide 36
-
MANEGEMENTPRINCIPAL AIMS (1) to remove exposure to injurious agents
(2) to suppress inflammation to prevent further destruction of the pulmonary parenchyma
(3) to palliate the manifestations of these diseases
CORTICOSTERIODS
bull Prednisone 1 mgkgbull Gradually tapered (5 mgweek) over several months
to a maintenance dose
IMMUNO SUPRISSIVE AGENTS
Cytotoxic agents (Cyclophosphamide)or immunosuppressive agents (Azathioprine) may be used in patients who do not improve on steroid therapy or who cannot tolerate corticosteroids
N-ACETYL CYSTEIN
bullAntioxidant
bull600 mg PO tid added to prednisone and azathioprine preserves vital capacity and FVC and DLCO
PIRFENIDONE
bullAntifibroticbullReduces acute exacerbations and reduction in FVC
OXYGEN THERAPY
bull F or pa tients w i th d o c u m e nted h y p ox i a ndash S p O 2 lt89 P a O 2 lt55m m H g
bull Improves exerc i se to le rance
CONCLUSION
bull DPLD is the better term not a single disease bull IPF is the most common DPLDbull Restrictive Airway Diseasebull Main treatment aim is to suppress inflammation to prevent
further destruction of the pulmonary parenchyma
Thank you
- Outline
- introduction
- Introduction
- Introduction (2)
- Classification of dpld
- Idiopathic ILD
- Those with identifiable cause
- classification
- Epidemiology
- Ipf - PATHOgenesis VIDEO
- Slide 12
- Respiratory SYMPTOMS
- Non Respiratory symptoms associated with different dplds
- ONSET OF SYMPTOMS
- ILD-HISTORY
- Occupational history
- Environmental exposure history
- ILD-SIGNS
- Extra Pulmonary -SIGNS
- INVESTIGATIONS
- Chest X Ray
- High-resolution Computed topography (HRCT)
- PULMONARY FUNCTION TESTING
- PULMONARY FUNCTION TESTING (2)
- Laboratory investigations
- OTHER INVESTIGATIONS
- Slide 28
- MANEGEMENT
- CORTICOSTERIODS
- IMMUNO SUPRISSIVE AGENTS
- N-acetyl cystein
- Pirfenidone
- Oxygen therapy
- Conclusion
- Slide 36
-
CORTICOSTERIODS
bull Prednisone 1 mgkgbull Gradually tapered (5 mgweek) over several months
to a maintenance dose
IMMUNO SUPRISSIVE AGENTS
Cytotoxic agents (Cyclophosphamide)or immunosuppressive agents (Azathioprine) may be used in patients who do not improve on steroid therapy or who cannot tolerate corticosteroids
N-ACETYL CYSTEIN
bullAntioxidant
bull600 mg PO tid added to prednisone and azathioprine preserves vital capacity and FVC and DLCO
PIRFENIDONE
bullAntifibroticbullReduces acute exacerbations and reduction in FVC
OXYGEN THERAPY
bull F or pa tients w i th d o c u m e nted h y p ox i a ndash S p O 2 lt89 P a O 2 lt55m m H g
bull Improves exerc i se to le rance
CONCLUSION
bull DPLD is the better term not a single disease bull IPF is the most common DPLDbull Restrictive Airway Diseasebull Main treatment aim is to suppress inflammation to prevent
further destruction of the pulmonary parenchyma
Thank you
- Outline
- introduction
- Introduction
- Introduction (2)
- Classification of dpld
- Idiopathic ILD
- Those with identifiable cause
- classification
- Epidemiology
- Ipf - PATHOgenesis VIDEO
- Slide 12
- Respiratory SYMPTOMS
- Non Respiratory symptoms associated with different dplds
- ONSET OF SYMPTOMS
- ILD-HISTORY
- Occupational history
- Environmental exposure history
- ILD-SIGNS
- Extra Pulmonary -SIGNS
- INVESTIGATIONS
- Chest X Ray
- High-resolution Computed topography (HRCT)
- PULMONARY FUNCTION TESTING
- PULMONARY FUNCTION TESTING (2)
- Laboratory investigations
- OTHER INVESTIGATIONS
- Slide 28
- MANEGEMENT
- CORTICOSTERIODS
- IMMUNO SUPRISSIVE AGENTS
- N-acetyl cystein
- Pirfenidone
- Oxygen therapy
- Conclusion
- Slide 36
-
IMMUNO SUPRISSIVE AGENTS
Cytotoxic agents (Cyclophosphamide)or immunosuppressive agents (Azathioprine) may be used in patients who do not improve on steroid therapy or who cannot tolerate corticosteroids
N-ACETYL CYSTEIN
bullAntioxidant
bull600 mg PO tid added to prednisone and azathioprine preserves vital capacity and FVC and DLCO
PIRFENIDONE
bullAntifibroticbullReduces acute exacerbations and reduction in FVC
OXYGEN THERAPY
bull F or pa tients w i th d o c u m e nted h y p ox i a ndash S p O 2 lt89 P a O 2 lt55m m H g
bull Improves exerc i se to le rance
CONCLUSION
bull DPLD is the better term not a single disease bull IPF is the most common DPLDbull Restrictive Airway Diseasebull Main treatment aim is to suppress inflammation to prevent
further destruction of the pulmonary parenchyma
Thank you
- Outline
- introduction
- Introduction
- Introduction (2)
- Classification of dpld
- Idiopathic ILD
- Those with identifiable cause
- classification
- Epidemiology
- Ipf - PATHOgenesis VIDEO
- Slide 12
- Respiratory SYMPTOMS
- Non Respiratory symptoms associated with different dplds
- ONSET OF SYMPTOMS
- ILD-HISTORY
- Occupational history
- Environmental exposure history
- ILD-SIGNS
- Extra Pulmonary -SIGNS
- INVESTIGATIONS
- Chest X Ray
- High-resolution Computed topography (HRCT)
- PULMONARY FUNCTION TESTING
- PULMONARY FUNCTION TESTING (2)
- Laboratory investigations
- OTHER INVESTIGATIONS
- Slide 28
- MANEGEMENT
- CORTICOSTERIODS
- IMMUNO SUPRISSIVE AGENTS
- N-acetyl cystein
- Pirfenidone
- Oxygen therapy
- Conclusion
- Slide 36
-
N-ACETYL CYSTEIN
bullAntioxidant
bull600 mg PO tid added to prednisone and azathioprine preserves vital capacity and FVC and DLCO
PIRFENIDONE
bullAntifibroticbullReduces acute exacerbations and reduction in FVC
OXYGEN THERAPY
bull F or pa tients w i th d o c u m e nted h y p ox i a ndash S p O 2 lt89 P a O 2 lt55m m H g
bull Improves exerc i se to le rance
CONCLUSION
bull DPLD is the better term not a single disease bull IPF is the most common DPLDbull Restrictive Airway Diseasebull Main treatment aim is to suppress inflammation to prevent
further destruction of the pulmonary parenchyma
Thank you
- Outline
- introduction
- Introduction
- Introduction (2)
- Classification of dpld
- Idiopathic ILD
- Those with identifiable cause
- classification
- Epidemiology
- Ipf - PATHOgenesis VIDEO
- Slide 12
- Respiratory SYMPTOMS
- Non Respiratory symptoms associated with different dplds
- ONSET OF SYMPTOMS
- ILD-HISTORY
- Occupational history
- Environmental exposure history
- ILD-SIGNS
- Extra Pulmonary -SIGNS
- INVESTIGATIONS
- Chest X Ray
- High-resolution Computed topography (HRCT)
- PULMONARY FUNCTION TESTING
- PULMONARY FUNCTION TESTING (2)
- Laboratory investigations
- OTHER INVESTIGATIONS
- Slide 28
- MANEGEMENT
- CORTICOSTERIODS
- IMMUNO SUPRISSIVE AGENTS
- N-acetyl cystein
- Pirfenidone
- Oxygen therapy
- Conclusion
- Slide 36
-
PIRFENIDONE
bullAntifibroticbullReduces acute exacerbations and reduction in FVC
OXYGEN THERAPY
bull F or pa tients w i th d o c u m e nted h y p ox i a ndash S p O 2 lt89 P a O 2 lt55m m H g
bull Improves exerc i se to le rance
CONCLUSION
bull DPLD is the better term not a single disease bull IPF is the most common DPLDbull Restrictive Airway Diseasebull Main treatment aim is to suppress inflammation to prevent
further destruction of the pulmonary parenchyma
Thank you
- Outline
- introduction
- Introduction
- Introduction (2)
- Classification of dpld
- Idiopathic ILD
- Those with identifiable cause
- classification
- Epidemiology
- Ipf - PATHOgenesis VIDEO
- Slide 12
- Respiratory SYMPTOMS
- Non Respiratory symptoms associated with different dplds
- ONSET OF SYMPTOMS
- ILD-HISTORY
- Occupational history
- Environmental exposure history
- ILD-SIGNS
- Extra Pulmonary -SIGNS
- INVESTIGATIONS
- Chest X Ray
- High-resolution Computed topography (HRCT)
- PULMONARY FUNCTION TESTING
- PULMONARY FUNCTION TESTING (2)
- Laboratory investigations
- OTHER INVESTIGATIONS
- Slide 28
- MANEGEMENT
- CORTICOSTERIODS
- IMMUNO SUPRISSIVE AGENTS
- N-acetyl cystein
- Pirfenidone
- Oxygen therapy
- Conclusion
- Slide 36
-
OXYGEN THERAPY
bull F or pa tients w i th d o c u m e nted h y p ox i a ndash S p O 2 lt89 P a O 2 lt55m m H g
bull Improves exerc i se to le rance
CONCLUSION
bull DPLD is the better term not a single disease bull IPF is the most common DPLDbull Restrictive Airway Diseasebull Main treatment aim is to suppress inflammation to prevent
further destruction of the pulmonary parenchyma
Thank you
- Outline
- introduction
- Introduction
- Introduction (2)
- Classification of dpld
- Idiopathic ILD
- Those with identifiable cause
- classification
- Epidemiology
- Ipf - PATHOgenesis VIDEO
- Slide 12
- Respiratory SYMPTOMS
- Non Respiratory symptoms associated with different dplds
- ONSET OF SYMPTOMS
- ILD-HISTORY
- Occupational history
- Environmental exposure history
- ILD-SIGNS
- Extra Pulmonary -SIGNS
- INVESTIGATIONS
- Chest X Ray
- High-resolution Computed topography (HRCT)
- PULMONARY FUNCTION TESTING
- PULMONARY FUNCTION TESTING (2)
- Laboratory investigations
- OTHER INVESTIGATIONS
- Slide 28
- MANEGEMENT
- CORTICOSTERIODS
- IMMUNO SUPRISSIVE AGENTS
- N-acetyl cystein
- Pirfenidone
- Oxygen therapy
- Conclusion
- Slide 36
-
CONCLUSION
bull DPLD is the better term not a single disease bull IPF is the most common DPLDbull Restrictive Airway Diseasebull Main treatment aim is to suppress inflammation to prevent
further destruction of the pulmonary parenchyma
Thank you
- Outline
- introduction
- Introduction
- Introduction (2)
- Classification of dpld
- Idiopathic ILD
- Those with identifiable cause
- classification
- Epidemiology
- Ipf - PATHOgenesis VIDEO
- Slide 12
- Respiratory SYMPTOMS
- Non Respiratory symptoms associated with different dplds
- ONSET OF SYMPTOMS
- ILD-HISTORY
- Occupational history
- Environmental exposure history
- ILD-SIGNS
- Extra Pulmonary -SIGNS
- INVESTIGATIONS
- Chest X Ray
- High-resolution Computed topography (HRCT)
- PULMONARY FUNCTION TESTING
- PULMONARY FUNCTION TESTING (2)
- Laboratory investigations
- OTHER INVESTIGATIONS
- Slide 28
- MANEGEMENT
- CORTICOSTERIODS
- IMMUNO SUPRISSIVE AGENTS
- N-acetyl cystein
- Pirfenidone
- Oxygen therapy
- Conclusion
- Slide 36
-
Thank you
- Outline
- introduction
- Introduction
- Introduction (2)
- Classification of dpld
- Idiopathic ILD
- Those with identifiable cause
- classification
- Epidemiology
- Ipf - PATHOgenesis VIDEO
- Slide 12
- Respiratory SYMPTOMS
- Non Respiratory symptoms associated with different dplds
- ONSET OF SYMPTOMS
- ILD-HISTORY
- Occupational history
- Environmental exposure history
- ILD-SIGNS
- Extra Pulmonary -SIGNS
- INVESTIGATIONS
- Chest X Ray
- High-resolution Computed topography (HRCT)
- PULMONARY FUNCTION TESTING
- PULMONARY FUNCTION TESTING (2)
- Laboratory investigations
- OTHER INVESTIGATIONS
- Slide 28
- MANEGEMENT
- CORTICOSTERIODS
- IMMUNO SUPRISSIVE AGENTS
- N-acetyl cystein
- Pirfenidone
- Oxygen therapy
- Conclusion
- Slide 36
-