and environmental exposures to organicand:inorganicdustsycollagenwascular and medicationitoxicity: There are also diffuse parenchymal lung diseases of unknown etiology, such as idiopathic pulmonary fibrosis (IPF). The physiologic consequences seen in IPF are typical of other causes of diffuse parenchymal lung disease, particularly in their advanced stages. For that reason, the remainder of this section focuses on IPF as a prototypical interstitial lung disease. Clinical Presentation IPF, previously known as interstitial pulmonary fibrosis or cryptogenic fibrosing alveolitis, is caused by progressive fibrosis leading to destruction of normal lung architecture. This process produces not only a restrictiverdefectywithraltered , but also vascular alterations that can severely impair normal pulmonary perfusion and gas exchange. The usual presentation of IPF is one of insidious onset. Patientspresent:with productive:cough. Reverandichestpainaregenerallyabsent. As the disease worsens, dyspnea may occur even at rest. Digitalicyanosisyiclubbingyand The diagnosis of IPF may be made by high-resolution chest CT in the setting of an appropriate clinical history and pulmonary function tests or by surgical lung biopsy. Typical CT findings are described below. The histopathologic correlate to IPF is usual interstitial pneumonia (UIP), a temporally and spatially heterogeneous patter ofiscatterediclusters‘ofimature'collagen deposition and alveolar wall destruction interspersed with areas of normal alveolararchitecture. These clusters of ongoing fibrosis are called fibroblastic foci and contain both fibroblasts and myofibroblasts; inflammatory cells may be present but are usually sparse. Etiology & Epidemiology Compared with COPD, IPF is an uncommon disorder with an estimated prevalence of 18jper00,000 peopleiinithe United:States: This disease has a male predominance and typically presents in the i itis rare in indivi ). By definition, the term “idiopathic” indicates that none of the known causes of diffuse parenchymal lung disease are present in an individual patient. Major risk factors include