Pulmonary fibrosis
| Headline | |
|---|---|
| Etiology | infectious, autoimmune, drug-induced. |
| Epidemiology | Risk factors: smoking, age, ~FHx, environment |
| Clinical presentation | end-expiratory basal crackles, weight loss, fatigue, clubbing. Ddx: heart failure, asbestosis, sarcoidosis |
| Pathogenesis | |
| Diagnostic investigations | spirometry: restrictive CXR: reticular shadowing ± honeycombing High-res CT: ground glass opacity |
| Management | ➥ Acute exacerbation: admit + high-dose pred ➥ Antifibrotic therapy: pirfenidone/nintedanib ➥ Lung transplant (IPF pts have highest death rates on transplant list) ➥ Higher risk GORD – consider ~PPIs Complications: |
| Prevention |
| Upper zone fibrosis | Lower zone fibrosis |
|---|---|
| coal worker’s pneumoconiosis, histiocytosis, radiation, Ankylosing spondylitis, Tuberculosis, silicosis/sarcoid | drug-induced (Amiodarone, Bleomycin, Methotrexate, Radiotherapy); see pneumotox |