Idiopathic pulmonary fibrosis (IPF), also known as cryptogenic fibrosing alveolitis, is a chronic and progressive fibrosing interstitial pneumonia of unknown cause that typically manifests after the fifth to sixth decade of life. The fibrosis is limited to the lung, and clinical features include progressive dyspnea and a restrictive pulmonary physiology. IPF is characterized by a usual interstitial pneumonia (UIP) pattern on high-resolution computed tomography (HRCT) and histopathology. This review discusses the definition, epidemiology, etiology/genetics, pathogenesis, diagnosis, differential diagnosis, management, complications, and prognosis of IPF. Figures depict the pathogenesis of IPF, a chest x-ray in a patient with IPF, HRCT scans, and histopathologic features of UIP. Tables list HRCT criteria for UIP patterns, the elements required for IPF diagnosis, and the GAP model for IPF prognosis.
This review contains 8 highly rendered figures, 3 tables, and 108 references.