Spiteri M A, Clarke S W
Department of Thoracic Medicine, Royal Free Hospital, Hampstead, London.
Sarcoidosis. 1989 Sep;6(2):107-10.
Primary biliary cirrhosis is a disease of unknown aetiology, resulting in progressive granulomatous destruction of small intrahepatic bile ducts. The rate at which this occurs varies considerably producing a wide clinical spectrum of the disease itself, as well as expression in other organs. Pulmonary manifestations in PBC have only been intermittently reported. Nevertheless in practice, a vast array of pathophysiological mechanisms can be implicated for the pulmonary dysfunction seen in some of these patients. The observed clinical features can be attributed to pulmonary vascular abnormalities, to a secondary 'fibrosing alveolitis', or indeed to the emergence of a granulomatous disorder in the lungs similar to sarcoidosis. Physicians should thus be aware of such potential lung complications, which can occur not only during the course of the PBC but also following liver transplantation.
原发性胆汁性肝硬化是一种病因不明的疾病，可导致肝内小胆管进行性肉芽肿性破坏。其发生速度差异很大，导致该疾病本身以及在其他器官中的表现具有广泛的临床谱。原发性胆汁性肝硬化的肺部表现仅有间歇性报道。然而在实际中，多种病理生理机制可能与部分此类患者出现的肺功能障碍有关。观察到的临床特征可归因于肺血管异常、继发性“纤维化肺泡炎”，或者确实是肺部出现了类似于结节病的肉芽肿性疾病。因此，医生应意识到此类潜在的肺部并发症，其不仅可发生在原发性胆汁性肝硬化病程中，也可发生在肝移植后。
