Imaging of Idiopathic Pulmonary Fibrosis

Idiopathic interstitial pneumonias are a heterogeneous group of diffuse lung diseases characterized by distinct clinicopathologic entities with the usual interstitial pneumonia (UIP) being the most common. The pattern of UIP can be seen in idiopathic pulmonary fibrosis (IPF) as well as in secondary causes, most commonly in connective tissue diseases. IPF is usually progressive and associated with a very poor prognosis, and newer therapies pose a risk of serious complications; therefore, diagnostic certainty is crucial. This article reviews the radiologic findings in UIP with clinical correlation and histopathologic features along with its significance for prognosis and patients monitoring.