PRIMARY BILIARY-CIRRHOSIS AND SYSTEMIC-SCLEROSIS (REYNOLDS-SYNDROME) - 8 NEW CASES - INTEREST OF THE LABIAL SALIVARY-GLAND BIOPSY

Purpose. - We report eight new cases of the association primary biliar y cirrhosis - systemic sclerosis (Reynolds' syndrome) and study the co ntribution of labial salivary gland biopsy to the disease diagnosis. M ethods. - We retrospectively collected clinical and biological data as well as results of labial salivary gland biopsies in eight patients w ith Reynolds' syndrome. Results. - The eight patients were female. Sys temic sclerosis corresponded to a CREST syndrome in three patients, wh ile in two other patients two features of the CREST syndrome were obse rved. Anticentromere antibodies were detected in these five cases. The last three patients had systemic sclerosis with bilateral pulmonary f ibrosis in two cases. All patients presented with Sjogren's syndrome. Labial salivary gland biopsies performed in six patients showed in fiv e cases an inflammatory infiltrate and focal sialadenitis typically as sociated with Sjogren's syndrome. In four cases, organic microangiopat hy and fibrosis were suggestive of systemic sclerosis, and in four oth er cases, the presence of a pericanalicular infiltrate of lymphocytes and necrosis of the excretory ducts epithelial cells suggested the exi stence of primary biliary cirrhosis. In three patients, all histologic al lesions were found in the same labial salivary gland biopsy. Conclu sion. - Coexistence of histological lesions on the same labial salivar y gland suggestive of primary biliary cirrhosis, systemic sclerosis an d Sjogren's syndrome has never been described previously. The diagnost ic value of labial salivary gland biopsy has to be assessed in this co ntext (C) 1998, Elsevier, Paris.