Overview
Definition
Chronic, progressive fibrosing interstitial pneumonia of unknown cause.
Epidemiology
Rare, typically affects adults > 60 years.
Etiology & Risk Factors
- Idiopathic
- Genetic predisposition
Clinical Symptoms
- Progressive dyspnea
- Non-productive cough
- Clubbing
Clinical Approach
Diagnosis
- High-resolution CT (Honeycombing)
Management
- Pirfenidone
- Nintedanib
- Lung transplant
Complications
- Respiratory failure
- Pulmonary hypertension