HY ILD

Question 1

ILD of known etiology

Answer 1

• occupational/envio lung diseases
• drug rxns
• collagen vascular disease

Question 2

ILD of unknown etiology

Answer 2

• sarcoidosis
• Pulmonary Langerhans cell histocytosis (eosinophilic granuloma)
• Granulomastosis

Question 3

idiopathic interstitial pneumonias (IIPS)

3 general types

Answer 3

chronic fibrosiing

acute/subacute fibrosing

smoking related

Question 4

idiopathic interstitial pneumonias (IIPS)

chronic fibrosing

Answer 4

1. idiopathic pulmonary fibrosis (IPF)

2. idiopathic nonspecific interstitial pneumonia

Question 5

idiopathic interstitial pneumonias (IIPS)

acute/subacute fibrosing

Answer 5

1. cryptogenic organizing pneumonia

2. acute interstitial pneumonia

Question 6

idiopathic interstitial pneumonias (IIPS)

smoking-related

Answer 6

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

Question 7

Idiopathic Pulmonary Fibrosis (IPF) mechanism

Answer 7

Injury to alveolar epithelium/bm and dysregulated repair

–> respiratory failure

Question 8

ILD PFTs

Answer 8

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

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

Question 9

ILD key clinical findings

Answer 9

• progressive dyspnea
• non-productive cough
• rapid shallow breathing

Question 10

Sarcoidosis mechanism

Answer 10

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

Question 11

Sarcoidosis key findings

Answer 11

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

Question 12

Granulomatosis with polyangiitis (wegeners)

Answer 12

• involves upper airways, kidney, lung
• granulomatous vasculitis
• multiple pulmonary nodules –> hemoptysis
• c-ANCA positive
• upper airway/kidney/lung
• tx: responds to steroids

Question 13

Cryptogenic organizing pneumonia (COP)

Answer 13

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

Question 14

acute interstitial pneumonia

Answer 14

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

Question 15

Desquamative Interstitial Pneumonia (DIP)

Answer 15

• smoker’s disease
• MILD fibrosis
• alveoli filled w/ alv macs (light brown)
• tx: good response to smoking cessation/steroids

Question 16

idiopathic pulmonary fibrosis (IPF)

Answer 16

• when UIP can’t be attributed to a known disease
• most common of IIPs
• usu seen in adults >50 yrs w/ insidious onset SOB/cough
• poor prognosis, median survival 3 years post-dx
• UIP histo pattern (honeycombing, lesions have “age/space” heterogeneity)

Question 17

usual interstitial pneumonia (UIP)

Answer 17

• usu seen in adults >50 yrs w/ insidious onset SOB/cough
• poor prognosis, median survival 3 years post-dx
• must exclude other lung causes of ILD (may change prognosis)
• honeycombing, lesions have “age/space” heterogeneity

Question 18

4 major categories of occupational/envio lung disease

Answer 18

1. pneumoconiosis
2. hypersensitivity pneumonitis
3. direct tissue injury
4. occupational asthma

Question 19

asbestosis

clinical findings

Answer 19

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

Question 20

asbestosis mechanism

Answer 20

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

Question 21

pneumoconosis

Answer 21

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

Question 22

berylliosis

Answer 22

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

Question 23

coal workers pneumoconiosis

Answer 23

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

Question 24

Hypersensitivity pneumonitis

predominant cell

Answer 24

lymphocyte is predominant cell (CD8+)

Question 25

Hypersensitivity pneumonitis | 3 clinical forms

Answer 25

acute, subacute, chronic (can progress to pulmonary fibrosis)

Question 26

direct tissue injury

Answer 26

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

Question 27

occupational asthma requirements

Answer 27

- 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 28

RADS

Answer 28

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 29

silicosis

Answer 29

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

Question 30

Pulmonary Langerhans Cell Histiocytosis

Answer 30

- when restricted to lung --> TOBACCO | - stellate lesions, birbeck granules (tennis rackets), cells have nuclear groove, eosinophils, S-100

Question 31

________ cells have prominent nuclear groove

Answer 31

LANGERHANS CELLS cells have prominent nuclear groove

Question 32

chronic Eos pneumonia

Answer 32

accumulation of Eos within lung wheeze, fever hx of asthma > 50%