pulmonary disorders
Idiopathic Pulmonary Fibrosis
Key Points
Idiopathic pulmonary fibrosis accounts for most idiopathic interstitial pneumonia and tends to affect older people. Symptoms and signs (eg, subacute dyspnea, nonproductive cough, and Velcro crackles) are nonspecific and usually caused by other, more common disorders. High-resolution CT can help in diagnosis by showing findings such as diffuse, patchy, subpleural, reticular opacities with irregularly thickened interlobular septa and intralobular lines; subpleural honeycombing; and traction bronchiectasis. Treat supportively and, if available, usepirfenidoneornintedanib. Encourage participation in clinical trials and, if patients are < 65 years and otherwise healthy, consider lung transplantation at the time of diagnosis. 