USMLE/COMLEX - Step 2 - Idiopathic Pulmonary Fibrosis
Pathophysiology
Inflammation and/or fibrosis of the interstitum of the lung
Etiologies
Medications such as amiodarone
Asbestos
Polymyositis/dermatomyositis
Vasculitis
CREST syndrome
Sarcoidosis
Symptoms
Shallow breathing
Chronic nonproductive cough
Dyspnea
Diagnostics
Chest x-ray is the best initial test
High resolution CT scan will show honeycombing pattern indicating severe stage
Pulmonary function test
Biopsy is most accurate test
Treatment
Corticosteroids
Antifibrotic agents such as pirfenidone and nintedanib
Lung transplant