Wallace J G, Tong M J, Ueki B H, Quismorio F P
Huntington Memorial Hospital Liver Center, Pasadena, CA 91105.
J Clin Gastroenterol. 1987 Aug;9(4):431-5. doi: 10.1097/00004836-198708000-00015.
Four patients with primary biliary cirrhosis (PBC) and Sjogren's syndrome presented with pulmonary disease manifested by fever, cough, and dyspnea. One patient had obstructive lung disease secondary to an autoimmune process documented by immunofluorescent staining of lung parenchyma. The other three patients had interstitial lung disease, pulmonary nodules, and multiple granulomas of the lung, respectively. Primary biliary cirrhosis is a complex autoimmune syndrome involving a disorder of the secretory immune system and its epithelial end organs. These four cases suggest that, in addition to biliary, lacrimal, intestinal, renal tubular, and salivary epithelium, lung tissue also is a possible site for autoimmune involvement in PBC.
四名原发性胆汁性肝硬化（PBC）合并干燥综合征的患者出现了以发热、咳嗽和呼吸困难为表现的肺部疾病。一名患者患有继发于自身免疫过程的阻塞性肺病，肺实质免疫荧光染色证实了这一点。另外三名患者分别患有间质性肺病、肺结节和肺部多发肉芽肿。原发性胆汁性肝硬化是一种复杂的自身免疫综合征，涉及分泌免疫系统及其上皮终末器官的紊乱。这四例病例表明，除了胆管、泪腺、肠道、肾小管和唾液腺上皮外，肺组织也可能是PBC自身免疫累及的部位。
