interstitial lung fibrosis Flashcards
1
Q
What will a patient present with in case of interstitial lung disease?
A
- progressive worsening of cough for a long period
- continuous
- dry cough (no hemoptysis)
- aggravated by smoking
- alleviated by nothing
- associated with worsening dyspnea
2
Q
What will be found on physical examination & labs of interstitial lung fibrosis patient?
A
- hypertension
- tachycardia
- hypoxia
- tachypnea
- bilateral crackles
- increased CO2 (normal = 22-32)
3
Q
What is seen on x-ray of patient suffering from interstitial lung fibrosis?
A
- increased reticular opacities bilaterally
4
Q
What is the pattern identified by pulmonary function test?
A
- restrictive -> FEV1/FVC = 75
5
Q
What is the pathophysiology of interstitial lung fibrosis?
A
- repeated cycles of tissue injury with aberrant wound healing -> collagenous fibrosis -> remodeling of pulmonary interstitium
6
Q
What are the causes of interstitial lung fibrosis?
A
- idiopathic pulmonary fibrosis
- acute interstitial pneumonia (AIP)
- cryptogenic organizing pneumonia
- respiratory bronchiolitis-associated interstitial lung disease
- desquamative interstitial pneumonia
7
Q
What are the secondary causes of interstitial lung pneumonia?
A
- hypersensitivity pneumonitis -> Farmer lung
- pneumoconiosis -> asbestosis, Coal worker’s lung
- radiation pneumonitis
- substance induced ILD -> bleomycin, methotrexate, amiodarone, nitrofurantoin
- autoimmune -> scleroderma, rheumatoid arthritis, vasculitis, sarcoidosis
8
Q
What are the clinical features of ILD?
A
- progressive dyspnea -> exertional & at rest
- chronic dry cough
- fever with hypersensitivity pneumonitis & sarcoidosis
- clubbing
- cyanosis
- loud inspiratory wheeze
9
Q
What is the gold standard for diagnosis of ILD?
A
high resolution CT chest
10
Q
What investigations should be done in rapidly progressive disease?
A
- bronchoscopy
- biopsy (unknown cause)
11
Q
What is the treatment of ILD?
A
- stop smoking
- stop exposure
- oxygen
- bronchodilator
- corticosteroid -> in hypersensitivity pneumonitis
- anti-fibrotic agents (Pirfenidone or Nintedanib) -> for idiopathic pulmonary fibrosis
- lung transplant if advanced
12
Q
What are the complications of ILD?
A
- pulmonary hypertension
- Cor pulmonale
- respiratory failure
