Pulmonary involvement in primary biliary cirrhosis.

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.