Primary biliary cirrhosis and sarcoidosis.

1 Pratter MR, Irwin RS. Usefulness and safety of pharmacological bronchoprovocation challenge in evaluating patients with normal spirometric tests who are suspected of having asthnaa. Chest 1988; 93:898-900 2 Pratter MR. Irwin RS. The clinical value of pharmacological bronchoprovocation challenge. In a recent review ofprimary biliary cirrhosis (PBC), the possible association of this disease with sarcoidosis is not' Although most patients with sarcoidosis have no clinical evidence of liver disease, hepatic granulomas occur in 60 percent of patients with this disease.2 Rarely, patients with sarcoidosis may develop chronic intrahepatic cholestasis, which may progress to biliary cirrhosis and liver failure. Since many patients with PBC show hepatic granulomas, the question arises of the relation between these two disorders. Similarities between PBC and sarcoidosis with chronic intrahepatic cholestasis were described more than 25 years ago. In 1969, Karlish et all reported the case ofa 47-year-old woman with enlarged hilar and paratracheal lymph nodes, a positive Kveim test, circulating antimitochondrial antibodies and progressive cho-lestatic liver disease. Maddrey et al,, in a study of 20 patients with sarcoidosis and chronic hepatic disease, described two patients who had features ofPBC: they subsequently described two other patients with pulmonary granulomas and progressive cholestatic liver disease .5 Antimitochondrial antibodies were positive in one of two patients tested in this group of four. Staisley et all described two middle-aged women with pruritus, liver findings compatible with PBC, and positive antisnitochondrial antil)()dies. Both patielits had pulmonary infiltrates; after they died from liver disease, autopsy showed pulmonary granuloma.s. Rudzki et all described a series of five young men who had evidence ofsystemic grassulomatous disease with clinical and biochemical data similar to those of PBC. Antimitochondrial asitibalies were not found; survival exceeded the usual survival ofpatients with PBC and the authors considered sarcoide)sis as the most likely diagnosis. Fagan et all descril)ed four women with high titers ofantimitoehon(lrial antibodies and hepatic granulomas, in addition to prominent pultnonary signs and svtnp-toms. Kveim test was positive in one of the three patients tested; all patients had al)norsnal chest radiographs and three of them had lung granulomas Ofl biopsy. The authors stressed that their patients' disorders were not discrete entities, hut had clinical, serologic and histologic findings that overlapped those of Sj#{246}gren's syndrome, celiac disease and mixed connective disease as well as sarcoidosis and PBC. About 95 percent of patients with PBC have positive antimitochondrial antilxxly tests, as opposed to 1 percent of patients …