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Pulm > Lec 16 Interstitial Lung Disease > Flashcards

Lec 16 Interstitial Lung Disease Flashcards

1
Q

What is interstitial lung disease?

A

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

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2
Q

What are two types of idiopathic chronic fibrosing ILD?

A
  • idiopathic pulmonary fibrosis

- idiopathic nonspecific interstitial pneumonitis

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3
Q

What is morphologic pattern of idiopathic pulmonary fibrosis?

A

usual interstitial pneumonia

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4
Q

What is morphologic pattern of idiopathic nonspecific interstitial pneumonia?

A

nonspecific interstitial pneumonia

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5
Q

What are two types of idiopathic smoking related ILD?

A
  • respiratory bronchiolitis interstitial lung disease

- desquamative interstitial pneumonia

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6
Q

What is morphologic pattern of respiratory bronchiolitis interstitial lung disease?

A

respiratory bronchiolitis

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7
Q

What is morphologic pattern of desquamative interstitial pneumonia?

A

desquamative interstitial pneumonia

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8
Q

What are two types of idiopathic acute/subacute ILD?

A
  • cryptogenic organizing pneumonia

- acute interstitial pneumonia

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9
Q

What is morphologic pattern of cryptogenic organizing pneumonia?

A

organizing pneumonia

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10
Q

What is morphologic pattern of acute interstitial pneumonia?

A

diffuse alveolar damage

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11
Q

What happens to PFT/lung volumes in ILD?

A
  • restrictive; impaired gas exchange
  • decrease FVC, TLC
  • preserved FEV1/FVC
  • decrease DLCO
  • increase A-a gradient
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12
Q

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

A

decreased DLCO

increased Aa gradient

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13
Q

What symptoms in ILD?

A

nonspecific respiratory symptoms

dyspnea, cough

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14
Q

What are radiographic findings of ILD?

A

fibrosis: appear as traction bronchiectasis [dilated airways]; reticular densities [linear markings]; honeycombing; cysts
inflammation: ground glass, consolidation

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15
Q

What is most common type of idiopathic interstitial pneumonia?

A

idiopathic pulmonary fibrosis

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16
Q

What is most deadly type of idiopathic interstitial pneumonia?

A

idiopathic pulmonary fibrosis

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17
Q

What is mortality of idiopathic pulmonary fibrosis?

A
  • median survival 2.8 years

- 90% mortality

18
Q

What are clinical feat of idiopathic pulmonary fibrosis?

A
  • subacute or chronic; gradual onset
  • dyspnea
  • cough
  • rales
  • clubbing
  • restrictive PFTs
  • low DLCo, high A-a gradient
19
Q

What is typical IPF radiology?

A
  • bases of lung more involved than apex

- IPF spreads inward from out

20
Q

What is hypothesis of idiopathic pulmonary fibrosis pathogenesis?

A
  • 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
Q

What are genetics of IPF?

A

up to 25% have family members with interstitial lung disease

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

22
Q

What is prognosis of IPF?

A

median survival 2-3 years

23
Q

What factors associated with decreased survival in IPF?

A
  • pulmonary HTN
  • low DLCO
  • decrease in PFTs
  • desaturation to SPO2 < 88%
  • fibroblast foci
24
Q

What are the 2 approved treatments for IPF?

A
  • pirfenidone

- nintedanib

25
Q

What is mech of action pirfenidone?

A

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

26
Q

What is mech of action nintedanib?

A

triple tyrosine kinase inhibitor

–> inhibits VEGF, FGF, PDGF

27
Q

What treatment of smoking related ILDs?

A

treatment = stop smoking to slow down progression of disease

28
Q

What is AIP?

A

acute respiratory failure associated with DAD on pathology

mimics ARDS but without a known cause
high mortality

29
Q

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

A

connective tissue and autoimmune disease; drugs

scleroderma, polymyocytis

30
Q

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

A

better prognosis than IPF

31
Q

What is prognosis of COP?

A

best prognosis of all; shorter onset

32
Q

WHat is COP associated with?

A

connective tissue disorders and drugs

33
Q

What are pulmonary manifestations of connective tissue disorders?

A
  • inflammation/fibrosis/bleeding of parenchyma
  • bronchitis/ bronchiolitis/ bronchiectasis
  • vasculitis, pulm HTN
  • pleural effusion, inflammation
34
Q

What is example of drug that induces ILD?

A

nitrofurantoin

35
Q

What do you see in sarcoidosis?

A

noncaseating granulomas

multi-systemic disease

36
Q

What organs involved in sarcoidosis

A
  • resp tract
  • lymph nodes
  • larynx
  • sinonasal
  • ocular
  • salivary gland
  • skin
  • cardiac
  • bone and joint
  • nervous system
  • liver, spleen, kidney
37
Q

What is stage 1 sarcoidosis?

A

bilar hilar adenopathy +/- paratracheal adenopathy

38
Q

What is stage 2 sarcoidosis?

A

bilateral hilar adenopathy + pulmonary infiltrates

39
Q

What is stage 3 sarcoidosis?

A

pulmonary infiltrates

40
Q

What is stage 4 sarcoidosis?

A

pulmonary fibrosis

Pulm (24 decks)

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