Clinical Features and Differential Diagnosis of Idiopathic Pulmonary Fibrosis

Idiopathic pulmonary fibrosis (IPF) (also called cryptogenic fibrosing alveolitis) is a common and well-defined diffuse parenchymal lung disease of unknown etiology (1). It has characteristic clinical, radiographic, physiological, and pathological manifestations (2). To emphasize the distinct pathophysiology and clinical course of IPF, the American Thoracic Society/European Respiratory Society has recommended reserving the term idiopathic pulmonary fibrosis for the clinical condition characterized by progressive dyspnea, cough, restrictive lung disease, and the histopathological pattern of usual interstitial pneumonia (UIP) (Table 1) (3).