Primary biliary cirrhosis–autoimmune hepatitis overlap syndrome: Clinical features and response to therapy

摘要: The association of primary biliary cirrhosis (PBC) and autoimmune hepatitis (AIH) is thought to be rare, its optimal treatment unknown. Of 130 consecutive patients with a diagnosis PBC, we identified 12 cases (9.2%) overlap syndrome (10 females, 2 males; median age, 50 years) strictly defined by the presence at least two three recognized biochemical, serological, histological criteria each disease. One patient had initially pure PBC developed AIH characterized flare alanine transaminase (ALT) (1,330 IU/L; N < 35), elevated immunoglobulin G (IgG) (42 g/L; 14.0), anti-smooth muscle antibodies (ASMA) after 20 months ursodeoxycholic acid (UDCA) therapy. A complete clinical biochemical remission was achieved under combination corticosteroids UDCA. Eleven features both diseases presentation: high serum levels alkaline phosphatase (AP) (median: 280 100), ALT (140 IU/L), IgG (30.8 g/L), mitochondrial (n = 9) or ASMA 9), florid bile duct lesions 8), moderate severe periportal periseptal lymphocytic piecemeal necrosis 11). UDCA (13-15 mg/kg/d) given alone in 5 induced significant decrease cholestasis but not levels, liver fibrosis progressed 3 patients. Corticosteroids 6 ALT, IgG, AP none normalization. persistently abnormal tests either received corticosteroids. further marked improvement observed, all became asymptomatic. We conclude that, PBC: 1) rare; 2) flares may occur spontaneously UDCA; 3) required most obtain response. Overlap represent an important unrecognized cause resistance PBC.