HY ILD

Question 1

idiopathic interstitial pneumonias (IIPS)

smoking-related

Answer 1

1. respiratory bronchiolitis interstitial lung disease
2. desquamative interstitial pneumonia
3. Pulmonary Langerhans Cell Histiocytosis

Question 2

ILD PFTs

Answer 2

FEV1/FVC may be incr (improved tethering of airways)

Decreased FEV1, FVC, TLC and DLC (dec compliance, inc WOB)

Question 3

Sarcoidosis mechanism

Answer 3

-immunologic response to unknown antigen –> CD4 alveolitis –> granulomas

Question 4

Sarcoidosis key findings

Answer 4

• hilar/mediastinal adenopathy
• Non-necrotizing, well circumscribed granulomas (bronchovascular bundles and alveolar septae
• elevated ACE
• responds to steroids, immunosuppressants

Question 5

Granulomatosis with polyangiitis (wegeners)

Answer 5

• involves upper airways, kidney, lung
• granulomatous vasculitis
• multiple pulmonary nodules –> hemoptysis
• c-ANCA positive
• necrotizing granulomas
• elastin stain for remnants of vessels
• tx: responds to steroids, immunosuppressants

Question 6

Cryptogenic organizing pneumonia (COP)

Answer 6

• can be idiopathic or secondary to pneumonia etc.
• plugs of fibroblastic tissue within bronchioles to alveoli (plug individual airways)
• fever, weight loss
• tx: good prognosis w/ steroids

Question 7

acute interstitial pneumonia

Answer 7

• diffuse alveolar damage w/ hyaline membranes
• clinical syndrome/histologic changes of ARDS, but no known cause of ARDS found
• ground glass opacities
• high mortality

Question 8

Desquamative Interstitial Pneumonia (DIP)

Answer 8

• smoker’s disease
• MILD fibrosis
• alveoli filled w/ all MACROPHAGES (light brown)
• tx: smoking cessation/steroids

Question 9

idiopathic pulmonary fibrosis (IPF)

Answer 9

• when UIP can’t be attributed to a known disease
• SMOKING, GERD, age, men
• poor prognosis, median survival 3 years post-dx
• subpleural, lower lobes
• UIP histo pattern (honeycombing, lesions have “temporal/spacial” heterogeneity, reticular infiltrates)

Question 10

asbestosis

clinical findings

Answer 10

• ship builders
• decades after heavy exposure
• lower lobes
• other manifestations: pleural plaques, effusions, mesothelioma
• honeycombing
• ferruginous bodies
• PFTs: restrictive pattern w/ dec DLCO

Question 11

asbestosis mechanism

Answer 11

(ineffective phagocytosis by alveolar macrophages. Alv macs release cytokines that lead to fibroblast proliferation)

Question 12

pneumoconosis

Answer 12

Asbestos 
Berylliosis
Coal Workers’ pneumoconiosis (CWP)
Silicosis
Talcosis
Hard metal

Question 13

berylliosis

Answer 13

• aerospace industry
• like pulmonary sarcoid (non-caveating granulomas)
• responsive to steroids
• upper lobes
• inc risk of CA and cor pulmonale

Question 14

coal workers pneumoconiosis

Answer 14

• clinically similar to silicosis, coal dust causes alv macs to release cytokines and oxidants → inflammation
• affects upper lobes

Question 15

Hypersensitivity pneumonitis

predominant cell

Answer 15

lymphocyte is predominant cell (CD8+)

Question 16

direct tissue injury

Answer 16

• Silo fillers lung (NO2)
• metal fume fever
• byssinosis

Question 17

occupational asthma requirements

Answer 17

• no previous hx of asthma
• worsening peak flow during work week, initially better on weekends and vacations (though this peak flow pattern can also be seen in work-exacerbated asthma

Question 18

RADS

Answer 18

reactive airways dysfunction syndrome – asthma-like with airways hyper-reactivity that can occur in response to fumes (household cleaning agents)
-often responds to steroids

Question 19

silicosis

Answer 19

• asymptomatic, dyspnea, non-productive cough
• upper lobes
• inc risk for TB (ineffective phagocytosis)

Question 20

Pulmonary Langerhans Cell Histiocytosis

Answer 20

• when restricted to lung –> TOBACCO
• stellate lesions, birbeck granules (tennis rackets), eosinophils
• positive for S100