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Flashcards in Lec 16 Interstitial Lung Disease Deck (40):
1

What is interstitial lung disease?

heterogenous group of disorders w/ infiltration of interstitium of lungs by fluid, cells, and connective tissue

2

What are two types of idiopathic chronic fibrosing ILD?

- idiopathic pulmonary fibrosis
- idiopathic nonspecific interstitial pneumonitis

3

What is morphologic pattern of idiopathic pulmonary fibrosis?

usual interstitial pneumonia

4

What is morphologic pattern of idiopathic nonspecific interstitial pneumonia?

nonspecific interstitial pneumonia

5

What are two types of idiopathic smoking related ILD?

- respiratory bronchiolitis interstitial lung disease
- desquamative interstitial pneumonia

6

What is morphologic pattern of respiratory bronchiolitis interstitial lung disease?

respiratory bronchiolitis

7

What is morphologic pattern of desquamative interstitial pneumonia?

desquamative interstitial pneumonia

8

What are two types of idiopathic acute/subacute ILD?

- cryptogenic organizing pneumonia
- acute interstitial pneumonia

9

What is morphologic pattern of cryptogenic organizing pneumonia?

organizing pneumonia

10

What is morphologic pattern of acute interstitial pneumonia?

diffuse alveolar damage

11

What happens to PFT/lung volumes in ILD?

- restrictive; impaired gas exchange
- decrease FVC, TLC
- preserved FEV1/FVC
- decrease DLCO
- increase A-a gradient

12

What happens to DLCO in ILD? A-a gradient?

decreased DLCO
increased Aa gradient

13

What symptoms in ILD?

nonspecific respiratory symptoms
dyspnea, cough

14

What are radiographic findings of ILD?

fibrosis: appear as traction bronchiectasis [dilated airways]; reticular densities [linear markings]; honeycombing; cysts

inflammation: ground glass, consolidation

15

What is most common type of idiopathic interstitial pneumonia?

idiopathic pulmonary fibrosis

16

What is most deadly type of idiopathic interstitial pneumonia?

idiopathic pulmonary fibrosis

17

What is mortality of idiopathic pulmonary fibrosis?

- median survival 2.8 years
- 90% mortality

18

What are clinical feat of idiopathic pulmonary fibrosis?

- subacute or chronic; gradual onset
- dyspnea
- cough
- rales
- clubbing
- restrictive PFTs
- low DLCo, high A-a gradient

19

What is typical IPF radiology?

- bases of lung more involved than apex
- IPF spreads inward from out

20

What is hypothesis of idiopathic pulmonary fibrosis pathogenesis?

- alveolar epithelium undergoes cell death and have impaired reepithelialization
- when cells do re-epithelialize they are not normal
- pro-fibrotic growth factors get lots of myofibroblast deposition = hybrid myo + fibro

weird phenotype = resistatnt to cell death; produce lots of collagen cause honeycomb changes; lung remodeling + vascular remodeling

21

What are genetics of IPF?

up to 25% have family members with interstitial lung disease

autosomal dominant
genetic form = more aggressive, occurs at younger age

22

What is prognosis of IPF?

median survival 2-3 years

23

What factors associated with decreased survival in IPF?

- pulmonary HTN
- low DLCO
- decrease in PFTs
- desaturation to SPO2 < 88%
- fibroblast foci

24

What are the 2 approved treatments for IPF?

- pirfenidone
- nintedanib

25

What is mech of action pirfenidone?

blocks pro-fibrotic cytokines [TFG-B and PDGF]

26

What is mech of action nintedanib?

triple tyrosine kinase inhibitor

--> inhibits VEGF, FGF, PDGF

27

What treatment of smoking related ILDs?

treatment = stop smoking to slow down progression of disease

28

What is AIP?

acute respiratory failure associated with DAD on pathology

mimics ARDS but without a known cause
high mortality

29

What is non-specific interstitial pneumonia (NSIP) associated with?

connective tissue and autoimmune disease; drugs
scleroderma, polymyocytis

30

What is prognosis of non-specific interstitial pneumonia (NSIP)?

better prognosis than IPF

31

What is prognosis of COP?

best prognosis of all; shorter onset

32

WHat is COP associated with?

connective tissue disorders and drugs

33

What are pulmonary manifestations of connective tissue disorders?

- inflammation/fibrosis/bleeding of parenchyma
- bronchitis/ bronchiolitis/ bronchiectasis
- vasculitis, pulm HTN
- pleural effusion, inflammation

34

What is example of drug that induces ILD?

nitrofurantoin

35

What do you see in sarcoidosis?

noncaseating granulomas
multi-systemic disease

36

What organs involved in sarcoidosis

- resp tract
- lymph nodes
- larynx
- sinonasal
- ocular
- salivary gland
- skin
- cardiac
-bone and joint
- nervous system
- liver, spleen, kidney

37

What is stage 1 sarcoidosis?

bilar hilar adenopathy +/- paratracheal adenopathy

38

What is stage 2 sarcoidosis?

bilateral hilar adenopathy + pulmonary infiltrates

39

What is stage 3 sarcoidosis?

pulmonary infiltrates

40

What is stage 4 sarcoidosis?

pulmonary fibrosis