Lec 16 Interstitial Lung Disease Flashcards
What is interstitial lung disease?
heterogenous group of disorders w/ infiltration of interstitium of lungs by fluid, cells, and connective tissue
What are two types of idiopathic chronic fibrosing ILD?
- idiopathic pulmonary fibrosis
- idiopathic nonspecific interstitial pneumonitis
What is morphologic pattern of idiopathic pulmonary fibrosis?
usual interstitial pneumonia
What is morphologic pattern of idiopathic nonspecific interstitial pneumonia?
nonspecific interstitial pneumonia
What are two types of idiopathic smoking related ILD?
- respiratory bronchiolitis interstitial lung disease
- desquamative interstitial pneumonia
What is morphologic pattern of respiratory bronchiolitis interstitial lung disease?
respiratory bronchiolitis
What is morphologic pattern of desquamative interstitial pneumonia?
desquamative interstitial pneumonia
What are two types of idiopathic acute/subacute ILD?
- cryptogenic organizing pneumonia
- acute interstitial pneumonia
What is morphologic pattern of cryptogenic organizing pneumonia?
organizing pneumonia
What is morphologic pattern of acute interstitial pneumonia?
diffuse alveolar damage
What happens to PFT/lung volumes in ILD?
- restrictive; impaired gas exchange
- decrease FVC, TLC
- preserved FEV1/FVC
- decrease DLCO
- increase A-a gradient
What happens to DLCO in ILD? A-a gradient?
decreased DLCO
increased Aa gradient
What symptoms in ILD?
nonspecific respiratory symptoms
dyspnea, cough
What are radiographic findings of ILD?
fibrosis: appear as traction bronchiectasis [dilated airways]; reticular densities [linear markings]; honeycombing; cysts
inflammation: ground glass, consolidation
What is most common type of idiopathic interstitial pneumonia?
idiopathic pulmonary fibrosis
What is most deadly type of idiopathic interstitial pneumonia?
idiopathic pulmonary fibrosis
What is mortality of idiopathic pulmonary fibrosis?
- median survival 2.8 years
- 90% mortality
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
What is typical IPF radiology?
- bases of lung more involved than apex
- IPF spreads inward from out
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
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
What is prognosis of IPF?
median survival 2-3 years
What factors associated with decreased survival in IPF?
- pulmonary HTN
- low DLCO
- decrease in PFTs
- desaturation to SPO2 < 88%
- fibroblast foci
What are the 2 approved treatments for IPF?
- pirfenidone
- nintedanib
What is mech of action pirfenidone?
blocks pro-fibrotic cytokines [TFG-B and PDGF]
What is mech of action nintedanib?
triple tyrosine kinase inhibitor
–> inhibits VEGF, FGF, PDGF
What treatment of smoking related ILDs?
treatment = stop smoking to slow down progression of disease
What is AIP?
acute respiratory failure associated with DAD on pathology
mimics ARDS but without a known cause
high mortality
What is non-specific interstitial pneumonia (NSIP) associated with?
connective tissue and autoimmune disease; drugs
scleroderma, polymyocytis
What is prognosis of non-specific interstitial pneumonia (NSIP)?
better prognosis than IPF
What is prognosis of COP?
best prognosis of all; shorter onset
WHat is COP associated with?
connective tissue disorders and drugs
What are pulmonary manifestations of connective tissue disorders?
- inflammation/fibrosis/bleeding of parenchyma
- bronchitis/ bronchiolitis/ bronchiectasis
- vasculitis, pulm HTN
- pleural effusion, inflammation
What is example of drug that induces ILD?
nitrofurantoin
What do you see in sarcoidosis?
noncaseating granulomas
multi-systemic disease
What organs involved in sarcoidosis
- resp tract
- lymph nodes
- larynx
- sinonasal
- ocular
- salivary gland
- skin
- cardiac
- bone and joint
- nervous system
- liver, spleen, kidney
What is stage 1 sarcoidosis?
bilar hilar adenopathy +/- paratracheal adenopathy
What is stage 2 sarcoidosis?
bilateral hilar adenopathy + pulmonary infiltrates
What is stage 3 sarcoidosis?
pulmonary infiltrates
What is stage 4 sarcoidosis?
pulmonary fibrosis
