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
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