Cirrhose biliaire primitive et sclérodermie systémique (syndrome de Reynolds): à propos de huit nouveaux cas. Apport de la biopsie des glandes salivaires accessoires

Abstract

We report eight new cases of the association primary biliary cirrhosis--systemic sclerosis (Reynolds' syndrome) and study the contribution of labial salivary gland biopsy to the disease diagnosis. We retrospectively collected clinical and biological data as well as results of labial salivary gland biopsies in eight patients with Reynolds' syndrome. The eight patients were female. Systemic sclerosis corresponded to a CREST syndrome in three patients, while in two other patients two features of the CREST syndrome were observed. Anticentromere antibodies were detected in these five cases. The last three patients had systemic sclerosis with bilateral pulmonary fibrosis in two cases. All patients presented with Sjögren's syndrome. Labial salivary gland biopsies performed in six patients showed in five cases an inflammatory infiltrate and focal sialadenitis typically associated with Sjögren's syndrome. In four cases, organic microangiopathy and fibrosis were suggestive of systemic sclerosis, and in four other cases, the presence of a pericanalicular infiltrate of lymphocytes and necrosis of the excretory ducts epithelial cells suggested the existence of primary biliary cirrhosis. In three patients, all histological lesions were found in the same labial salivary gland biopsy. Coexistence of histological lesions on the same labial salivary gland suggestive of primary biliary cirrhosis, systemic sclerosis and Sjögren's syndrome has never been described previously. The diagnostic value of labial salivary gland biopsy has to be assessed in this context.