Recommendations for evaluating and managing idiopathic pulmonary fibrosis.

Idiopathic pulmonary fibrosis (IPF) is a chronic lung disease characterized by progressive and irreversible fibrosis of lung parenchyma that reduces lung function. This rare, incurable disease often is mistaken for an inflammatory condition. IPF typically manifests in older men and is associated with a history of smoking. Disease progression is rapid, with a 5-year survival rate of 20%. Treatment options include lung transplantation and medical therapies to reduce the steady decline in lung function. This article reviews the epidemiology, pathophysiology, presentation, diagnosis, and management of IPF.