A case af autoimmune hepatitis associated with mixed connective tissue disease and Sjögren syndrome

Abstract

A 49-year-old female case of autoimmune hepatitis associated with mixed connective tissue disease and Sjögren syndrome was reported. She was admitted to our hosgital because of abnormal liver test with camplaints of arthralgia, Raynaud phenomenon and general malaise. Histology of the biopsied-liver tissue revealed chronic inactive hepatitis. After one-year follow up, S-GOT and γ-globulin elevated up to 507IU and 3.2g/dl, respectively. Polyarthritis, puffiness of the dorsal aspect of bilateral hands, leucocytopenia and dryness af the nnouth were evident. Anti-RNP antibody, antinuclear antibody and anti-smooth muscle antibody were pasitive. The second biopsy of the liver revealed chronic active hepatitis with rossette formation of hepatocytes. Sialography showed the apple-tree lesian, and both Shirmer test and Rose-bengal test were positive. The level of serum transaminase and her symptom remarkably improved after steroid therapy. To our knowledge, this is the first report on association between autoimmune hepatitis, mixed connective tissue disease and Sjögren syndrome.