Interstitial Lung Diseases Flashcards
What are interstitial lung diseases (ILDs)?
A group of disorders characterized by cellular infiltration, scarring and/or architectural disruption of the pulmonary parenchyma
What are the ssx of ILD?
Dry cough with progressive DOE
What are PFTs like with ILDs?
Restrictive pattern (decreased FEV, and normal FEV1/FVC)
What are the CT/ CXR findings with ILDs?
Interstitial infiltrates and Ground glass appearance
What are the major occupational exposures that lead to ILD? (4)
Silica
Asbestos
Farms
Fumes
What are the two classic drugs that lead to ILD?
Amiodarone
Nitrofurantoin (macrobid)
What are the connective tissue disorders that can lead to ILD?
SLE
RA
Scleroderma
What are the signs of ILD?
Clubbing
Erythema/rash
Arthritis
What are the lungs sounds with ILD?
Dry crackles
What heart findings are found with ILD? (4)
Increased right heart pressure:
- increased P2 intensity
- TR murmur
- edema
- JVD
What is usually the first change on a PFT with ILD? Why?
Decreased DLCO due to non homogeneous thickening of the alveoli
What is usually the first change on a PFT with COPD? Why?
FEV/FVC ratio
What are the two ILDs that have increased lung volumes?
LAM
Langerhans histiocytosis
What is the granulomatous ILD?
Sarcoidosis
What are the four known association ILDs?
CT diseases
Drugs
Occupational exposure
Hypersensitivity
What are the idiopathic Interstitial pnuemonias?
- Idiopathic pulmonary fibrosis
- non-specific interstitial pneumonia
- Cryptogenic organizing pneumonia
- respiratory bronchiolitis
- desquamative interstitial pneumonia
- acute interstitial pneumonia
What patients with idiopathic interstitial pneumonias respond to therapy?
No
What is the prognosis (generally) for idiopathic pulmonary fibrosis?
Bad-3-5 year survival rate
What is the history commonality for idiopathic pulmonary fibrosis?
Smoking
Who usually gets idiopathic pulmonary fibrosis?
50-70 year old smokers
What are the ssx of idiopathic pulmonary fibrosis?
Chronic and progressive DOE, and non-productive cough
What are the lung sounds with idiopathic pulmonary fibrosis?
Bibasilar inspiratory “velcro-like” crackles
What percent of patients with idiopathic pulmonary fibrosis have clubbing?
25%
What causes the crackles with idiopathic pulmonary fibrosis?
Expanding alveoli
What are the CXR findings with idiopathic pulmonary fibrosis? (which lobe? central or peripheral distribution? What type of CT changes)?
Lower lobe and peripheral distribution of reticular infiltrates and fibrotic changes
What are the CT changes with idiopathic pulmonary fibrosis?
Patchy honeycombing and traction bronchiectasis
What are the tissue changes that can be seen in with idiopathic pulmonary fibrosis?
Usual interstitial pneumonitis
What are the drugs used to treat idiopathic pulmonary fibrosis? MOA?
Pirfenidone
Nintedanib
Inhibit IL-1beta and TNF-alpha from human lung fibroblasts
What is the MOA of etanercept? Is this effective in idiopathic pulmonary fibrosis?
Anti-TNF antibody
Not effective
What is N-acetylcysteine used for usually? Is it effective for idiopathic pulmonary fibrosis?
Acetaminophen overdose
Not effective
What is the MOA of Pirfenidone? Use?
Inhibit IL-1beta and TNF-alpha from human lung fibroblasts
Treats idiopathic pulmonary fibrosis
What is the MOA of nintedanib? Use?
Inhibit IL-1beta and TNF-alpha from human lung fibroblasts
Treats idiopathic pulmonary fibrosis
What are nonspecific interstitial pneumonitis (NSIP)?
Homogenic CT disease of the lungs
What is the prognosis (generally) with interstitial pneumonitis?
better than IPF