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Jitesh Ahuja, MD

DIFFUSE LUNG DISEASERESIDENT REVIEW COURSE 2015

UNIVERSITY OF WASHINGTON, SEATTLE

INTRODUCTION

• Interstitial lung disease• Cystic lung disease• Vasculitis• Infection • Neoplastic • Miscellaneous

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APPROACH

Distribution

Pattern

Associated abnormalities

Clinical information

PATTERN

Reticular

Nodular

Cystic

GGO/consolidation

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SECONDARY PULMONARY LOBULE

RETICULATION

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TRACTION BRONCHIECTASIS AND HONEYCOMBING

NODULAR

Perilymphatic Centrilobular

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40-YEAR-OLD MAN WITH COUGH AND SOBWHAT IS THE DIAGNOSIS?

a. Lymphangioleiomyomatosis (LAM)

b. Langerhans cell histiocytosis (LCH)

c. Centrilobular emphysema

d. Cystic fibrosis

LANGERHANS CELL HISTIOCYTOSIS (LCH)

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• Almost exclusively in adult smokers• Diagnosis with history and CT

LANGERHANS CELL HISTIOCYTOSIS (LCH)

CENTRILOBULAR EMPHYSEMA

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Exclusively women, 20-40 years

LYMPHANGIOLEIOMYOMATOSIS (LAM)

Sporadic or tuberous sclerosis complex (TSC)

• Recurrent pneumothorax, chylothorax, centrilobular nodules (pneumocyte or muscle cell hyperplasia), GGO (pulmonary hemorrhage), septal thickening

• Vascular endothelial growth factor (VEGF-D) elevated (>800pg/ml)

LYMPHANGIOLEIOMYOMATOSIS (LAM)

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LYMPHOCYTIC INTERSTITIAL PNEUMONIA (LIP)

Sjögren's syndrome

LYMPHOCYTIC INTERSTITIAL PNEUMONIA (LIP)

• Interstitial lung disease

• Within the spectrum of lymphoproliferative disorders

• Polyclonal or monoclonal gammopathy

• F>M; 4thto5th decade

• Autoimmune disease: Sjögren's syndrome, SLE, RA

• 1/4th reported cases have Sjögren's syndrome

• HIV (pediatric)

• Common variable immunodeficiency (CVID)

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BIRT-HOGG-DUBE SYNDROME

Skin lesions

• AD, mutation of gene encoding tumor suppressor protein “folliculin”

• Clinical manifestations• Skin lesions such as fibrofolliculomas

face, neck and upper back

• Renal tumors (oncocytoma or RCC)

• Pneumothorax 33-38% patients

BIRT-HOGG-DUBE SYNDROME

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LCH LAM

LIP Birt-Hogg-Dube

50-YEAR-OLD MALE WITH COUGH AND SOB FOR 2 YEARSWHICH OF THE FOLLOWING IS TRUE?

a. Predominant abnormality is ground glass opacity

b. Predominant abnormality is reticulation

c. There is no honeycombingd. There is no apicobasilar

disease gradient

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• UIP pattern• Subpleural and basilar

predominant

• Reticular abnormality

• Honeycombing with or without traction bronchiectasis

• Absence of features which are inconsistent with UIP

• Possible UIP• Subpleural and basilar

predominant

• Reticular abnormality

• Honeycombing with or without traction bronchiectasis

• Absence of features which are inconsistent with UIP

USUAL INTERSTITIAL PNEUMONIA (UIP)

INCONSISTENT WITH UIP

• Upper and mid lung predominance

• Peribronchovascular pattern

• Extensive ground glass opacity (GGO>reticulation)

• Profuse micronodules

• Discrete cysts

• Diffuse mosaic attenuation/air-trapping

• Consolidation

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USUAL INTERSTITIAL PNEUMONIA (UIP)

USUAL INTERSTITIAL PNEUMONIA (UIP)

• Idiopathic pulmonary fibrosis (IPF)- when idiopathic

• Collagen vascular disease (RA, SS), drugs (bleomycin), occupational or environmental exposure (asbestosis)

• Poor prognosis: median survival 2 to 4 years• Definite UIP pattern on HRCT does not need

confirmation by histology

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POSSIBLE UIP

NON SPECIFIC INTERSTITIAL PNEUMONIA (NSIP)

Dermatopolymyositis

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NON SPECIFIC INTERSTITIAL PNEUMONIA (NSIP)

NON SPECIFIC INTERSTITIAL PNEUMONIA (NSIP)

• Idiopathic or associated with collagen vascular disease (polymyositis, scleroderma, mixed connective tissue disorder), drug toxicity, occupational exposure

• Cellular or fibrotic• Prognosis much better than UIP

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SUBACUTE HYPERSENSITIVITY PNEUMONITIS

Exposure to birds

CHRONIC HYPERSENSITIVITY PNEUMONITIS

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HYPERSENSITIVITY PNEUMONITIS

• Farmer’s lung and bird fancier’s lung • Immunologically-mediated diffuse

granulomatous lung disease

• Aspergillus and thermophilic actinomycesare common inciting agents

• Removal of offending agent can prevent development of fibrosis

DESQUAMATIVE INTERSTITIAL PNEUMONIA

Chronic smoker

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CRYPTOGENIC ORGANIZING PNEUMONIA

No white countNo symptoms of infection

ORGANIZING PNEUMONIA

• Inflammatory response to pulmonary insult.

• Idiopathic: cryptogenic organizing pneumonia (COP)

• Secondary: connective tissue diseases, infections, malignancies, drugs, radiation, organ/stem cell transplantation and aspiration

• Reverse halo or Atoll sign

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a. Perilymphatic distribution of nodules

b. Centrilobular distribution of nodules

c. Lymphadenopathy is uncommon

d. Peripheral and basal predominant pattern

40-YEAR-OLD FEMALE WITH COUGH AND SOB FOR >6 MONTHS. WHICH OF THE FOLLOWING IS CORRECT ABOUT THIS DISEASE?

SARCOIDOSIS

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SARCOIDOSIS

Lymphadenopathy Galaxy sign

SARCOIDOSIS

Progressive massive fibrosis (PMF) Air trapping

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• Multisystem chronic granulomatous disease of unknown etiology

• African-American females• Thoracic involvement

90% cases• ~50% asymptomatic• 20% progress to

pulmonary fibrosis

SARCOIDOSIS

40-YEAR-OLD MALE WITH COUGH AND DYSPNEA WHICH OF THE FOLLOWING IS FALSE ABOUT THIS DISEASE?

a. Intraalveolar proteinaceousmaterial

b. Associated with hematologic malignancies

c. No association with smoking

d. Treatment is whole lung lavage

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PULMONARY ALVEOLAR PROTIENOSIS

Crazy paving

PULMONARY ALVEOLAR PROTIENOSIS

• Intraalveolar accumulation of proteinaceous material• Impaired surfactant homeostasis• Idiopathic (>90%) or secondary• Antibodies to GM-CSF• Secondary: hematologic malignancies, infections,

immunodeficiency including HIV or immunosuppressive drugs

• Strong association with smoking• Treatment: whole lung lavage and curing primary cause

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DIFFUSE ALVEOLAR HEMORRHAGE

Hemoptysis, SOB, no feverNo fluid overloadMicroscopic polyangitis

• Blood filling up the alveolar spaces

• Hemoptysis, anemia, radiographic pulmonary opacities and hypoxic respiratory failure

• Hemoptysis is absent in 1/3rd of cases

• Vasculitis, infections, coagulation disorders, leukemia, stem cell transplantation, crack lung, drug toxicity

• BAL helps in making the diagnosis but not underlying cause

DIFFUSE ALVEOLAR HEMORRHAGE

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PNEUMOCYSTIS PNEUMONIA

Fever and SOB with hypoxiaHIVCD4 count

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54-YEAR-OLD MALE WITH SOBWHAT IS THE DIAGNOSIS?

a. Pulmonary edemab. Ca lung with lymphangitic

carcinomatosisc. Hypersensitivity pneumonitisd. Sarcoidosis

LYMPHANGITIC CARCINOMATOSIS

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LYMPHANGITIC CARCINOMATOSIS

• Usually with carcinoma of lung, breast, stomach, colon, pancreas

• Hematogenous dissemination or direct invasions of lymphatics from mediastinal or hilar LN

• Unilateral/bilateral; focal or diffuse• Poor prognosis

PULMONARY EDEMA

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SUMMARY

• Cystic lung disease• LCH: upper lobes bizarre shaped cysts, CLN in

young smoker• LAM: uniform randomly distributed cysts in

women ± TSC• LIP: perivascular cysts• Birt-Hogg-Dube: Lenticular cysts with renal

tumors, skin lesions

SUMMARY

• ILD• UIP: honeycombing, heterogeneity• NSIP: GGO, subpleural sparing, homogeneity• COP: Peribronchovascular and subpleural

distribution

• Sarcoidosis: perilymphatic nodules • Crazy-paving: PAP, DAH, pulmonary edema,

infections, adenocarcinoma