smoking related ild

06/26/1306/26/13 amr badreldin hamdyamr badreldin hamdy 11

Smoking Related ILDSmoking Related ILD

Amr Badreldin HamdyAmr Badreldin HamdyMD, FCCPMD, FCCP


They represent a They represent a heterogeneous group of heterogeneous group of lung disorders, generally lung disorders, generally characterized by characterized by dyspnea, dry cough, dyspnea, dry cough, diffuse interstit ial diffuse interstit ial inf i l trates, restrict ive lung infi l trates, restrict ive lung function pattern, and function pattern, and impaired gas exchange.impaired gas exchange.


The majority of ILD The majority of ILD are of unknown are of unknown cause, and known cause, and known causes include causes include gases, fumes, drugs, gases, fumes, drugs, radiation, infections, radiation, infections, inorganic dusts…etc.inorganic dusts…etc.


ILD posit ively associated ILD posit ively associated with smoking:with smoking:

1. UIP/idiopathic pulmonary fibrosis.1. UIP/idiopathic pulmonary fibrosis. 2. Desquamative interstit ial 2. Desquamative interstit ial

pneumonia.pneumonia. 3. Respiratory bronchiolit is-3. Respiratory bronchiolit is-

associated interstit ial lung disease.associated interstit ial lung disease. 4. Pulmonary Langerhan’s cell 4. Pulmonary Langerhan’s cell

hist iocytosis.histiocytosis.


Respiratory bronchioli t is Respiratory bronchioli t is is extremely common in is extremely common in cigarette smokers cigarette smokers (smoker’s bronchioli t is). (smoker’s bronchioli t is). Bronchiol i t is is a generic Bronchiol i t is is a generic term used cl inically to term used cl inically to describe various describe various inflammatory diseases of inf lammatory diseases of small airways.small airways.


It usually occurs It usually occurs without symptoms or without symptoms or signif icant interstit ial signif icant interstit ial lung disease.lung disease.

It may account for It may account for sub-clinical radiological sub-clinical radiological changes in up to one changes in up to one fifth of smokers.fifth of smokers.


Although respiratory Although respiratory bronchiolit is occurs in bronchiolit is occurs in virtually all smokers it virtually all smokers it is of l i t t le cl inical is of l i t t le cl inical signif icance in the vast signif icance in the vast majority of cases.majority of cases.


ILD negatively ILD negatively associated with associated with smokingsmoking ::

1. Hypersensit ivity 1. Hypersensit ivity pneumonitis pneumonitis (exogenous allergic (exogenous allergic alveolit is).alveolit is).

2. Sarcoidosis.2. Sarcoidosis.


1. 1. Occupational/Environmental.Occupational/Environmental.

2. Granulomatous diseases.2. Granulomatous diseases.3. Iatrogenic/drug induced.3. Iatrogenic/drug induced.4. Collagen-vascular 4. Collagen-vascular

diseases.diseases.5. Inherited.5. Inherited.6. Unique entit ies.6. Unique entit ies.7. Idiopathic interstit ial 7. Idiopathic interstit ial

pneumonia.pneumonia.


Without a complete Without a complete thorough clinical thorough clinical evaluation, all ILD evaluation, all ILD are of unknown are of unknown causecause ..


Idiopathic Idiopathic Pulmonary FibrosisPulmonary Fibrosis


The role of smoking in The role of smoking in the pathogenesis of IPF is the pathogenesis of IPF is controversial. It appears controversial. It appears to increase the risk of to increase the risk of development of IPF, but development of IPF, but there is no evidence that there is no evidence that smoking per se directly smoking per se directly leads to the development leads to the development of IPF. of IPF.


Usually sporadic or famil ial.Usually sporadic or famil ial. Male to female ratio 2 to one.Male to female ratio 2 to one. Bilateral reticular or Bilateral reticular or

reticular-nodular opacit ies reticular-nodular opacit ies with small lung volumes.with small lung volumes.

Typically lower zone and Typically lower zone and peripheral predominance in peripheral predominance in the distribution.the distribution.


HRCT shows HRCT shows irregular l inear irregular l inear opacit ies and opacit ies and honeycombing, honeycombing, predominantly in the predominantly in the base and sub-pleural base and sub-pleural lung.lung.


PFT usually PFT usually demonstrates a demonstrates a restrictive defect with restrictive defect with reduced lung volumes reduced lung volumes and diffusing capacity.and diffusing capacity.

Exercise-induced de-Exercise-induced de-saturation is almost saturation is almost always seen.always seen.


In patients with pre-existing In patients with pre-existing emphysema, the lung emphysema, the lung volumes and f low rates may volumes and f low rates may be normal due to be normal due to counteracting physiological counteracting physiological effects of emphysema and effects of emphysema and f ibrosis.f ibrosis.

In such patients, PFT may In such patients, PFT may only reveal a severely only reveal a severely reduced diffusing capacityreduced diffusing capacity ..


Desquamative Desquamative Interstit ial PneumoniaInterstit ial Pneumonia


Ninety percent are Ninety percent are smokers or ex-smokers.smokers or ex-smokers.

May occasionally be May occasionally be seen in association with seen in association with systemic disorders or systemic disorders or infections, as well as infections, as well as exposure to exposure to occupational/environmentoccupational/environmental agents and drugs.al agents and drugs.


Average age of onset is Average age of onset is 40 years.40 years.

Male predominance (2:1).Male predominance (2:1). Inspiratory crackles are Inspiratory crackles are

heard in 60%.heard in 60%. Digital clubbing in nearly Digital clubbing in nearly

50%.50%.


On chest X-ray lung On chest X-ray lung volume appears volume appears reduced unless there reduced unless there is co-existent OAD is co-existent OAD such as smokers such as smokers with emphysema.with emphysema.


Predominant f inding by Predominant f inding by HRCT is the presence of HRCT is the presence of areas of ground-glass areas of ground-glass attenuations, typically attenuations, typically sub-pleural and lower sub-pleural and lower lung zone predominance.lung zone predominance.

Honeycombing is usually Honeycombing is usually not present.not present.


R-BILDR-BILD


It is a clinical It is a clinical pathological entity seen pathological entity seen almost exclusively in almost exclusively in current or former current or former smokers.smokers.


PFT may be normal, but PFT may be normal, but more commonly show a more commonly show a mixed obstructive-restrict ive mixed obstructive-restrict ive pattern of a mild-to-pattern of a mild-to-moderate degree.moderate degree.

Reduced diffusing capacity Reduced diffusing capacity is common.is common.

TLC may be normal, mildly TLC may be normal, mildly increased or mildly reduced.increased or mildly reduced.


Diffuse, f ine reticular or Diffuse, f ine reticular or reticular-nodular opacit ies reticular-nodular opacit ies are present in more than are present in more than 2/3.2/3.

Ground-glass pattern may Ground-glass pattern may be the predominant be the predominant abnormality.abnormality.

There is no honeycombing There is no honeycombing (DD IPF).(DD IPF).


Areas of ground-glass Areas of ground-glass attenuation are the attenuation are the most common finding most common finding (( smoker’s alveolit issmoker’s alveolit is ).).

Micro-nodules may be Micro-nodules may be present ( = respiratory present ( = respiratory bronchiolit is).bronchiolit is).


Pulmonary Langerhan’s Pulmonary Langerhan’s Cell HistiocytosisCell Histiocytosis


The isolated pulmonary The isolated pulmonary form in adults occurs form in adults occurs almost exclusively in almost exclusively in cigarette smokers. Adult cigarette smokers. Adult PLCH represents a PLCH represents a polyclonal, reactive polyclonal, reactive disorder tr iggered by disorder tr iggered by cigarette smoking.cigarette smoking.


Most patients are young adults (30-Most patients are young adults (30-40y).40y).

Sex distribution is equal.Sex distribution is equal. Ninety or more are current or Ninety or more are current or

previous cigarette smokers.previous cigarette smokers. The bronchiolar distribution of The bronchiolar distribution of

pathological lesions is consistent pathological lesions is consistent with the possibil ity that an inhaled with the possibil ity that an inhaled antigen is involved in the antigen is involved in the pathogenesis of this disorder.pathogenesis of this disorder.


Physical examination Physical examination frequently normal.frequently normal.

Cystic bone lesions Cystic bone lesions in 10% (skull, r ibs, in 10% (skull, r ibs, pelvis).pelvis).

Diabetes insipidus in Diabetes insipidus in 10%.10%.


PFT show both PFT show both obstructive and restrict ive obstructive and restrict ive changes (effects from changes (effects from cigarette smoking may be cigarette smoking may be superimposed and superimposed and diff icult to dist inguish diff icult to dist inguish from effects of PLCH from effects of PLCH itself).i tself).


Typical f inding on CXR Typical f inding on CXR include nodular or include nodular or reticular-nodular opacit ies reticular-nodular opacit ies most prominent in the most prominent in the middle and upper lung middle and upper lung zones, usually sparing of zones, usually sparing of the costo-phrenic anglesthe costo-phrenic angles

Lung volumes appear Lung volumes appear normal or increased.normal or increased.


HRCT show thin-HRCT show thin-walled cysts, nodules walled cysts, nodules (with or without (with or without cavitation) or a cavitation) or a combination of nodules combination of nodules and cysts.and cysts.


PrognosisPrognosis


IPFIPF Response to Response to steroids is poor.steroids is poor.

Prognosis is poor Prognosis is poor with no possibil i ty of with no possibil i ty of complete recovery.complete recovery.


DIPDIP

Most patients remain Most patients remain stable or improve with stable or improve with corticosteroid therapy corticosteroid therapy and complete recovery and complete recovery is possible.is possible.


R-BILDR-BILD

Good prognosis, Good prognosis, part icularly with smoking part icularly with smoking cessation.cessation.

Good response to Good response to corticosteroid therapy and corticosteroid therapy and complete recovery is complete recovery is possiblepossible ..


PLCHPLCH Smoking cessation may Smoking cessation may

prevent progression of the prevent progression of the disease.disease.

Response to steroids is Response to steroids is fair.fair.

Complete recovery is Complete recovery is possible.possible.

Prognosis is good.Prognosis is good.


ConclusionConclusion


• Smoking history is important in Smoking history is important in ILD.ILD.

• Quitt ing smoking is important Quitt ing smoking is important in ILD.in ILD.

• ILD may accompany COPD.ILD may accompany COPD.• HRCT may be of help in HRCT may be of help in

patients with COPD not patients with COPD not responding to usual broncho-responding to usual broncho-dilator therapy. They may need dilator therapy. They may need to add corticosteroids to their to add corticosteroids to their regimens.regimens.


• This may explain why so This may explain why so many patients with COPD many patients with COPD need cort icosteroids in need cort icosteroids in their treatment protocol.their treatment protocol.

• The incidence of The incidence of smoking in the smoking smoking in the smoking related ILD reaches 90%, related ILD reaches 90%, the same incidence as for the same incidence as for COPD.COPD.


THANK YOUTHANK YOU

smoking related ild

Health & Medicine

fccpamr badreldin hamdy

small lung volumes

reduced lung volumes

respiratory bronchiolitis

majority of ild

role of smoking

development of ipf

reduced diffusing