Clinical and histological characteristics of the primary biliary cirrhosis and autoimmune hepatitis overlap syndrome:a retrospective study

Objective To compare clinical,biological,immunologic and histological features in patients with primary biliary cirrhosis (PBC) and autoimmune hepatitis (AIH) overlap syndrome and uncomplicated PBC.Methods Forty-eight patients were re-evaluated using current diagnostic criteria of PBC.At the same time,we reviewed all the patients for evaluation of PBC-AIH overlap syndrome based on the IAIH-G (the International Autoimmune Hepatitis Group) Scoring System.A scores of 10 or greater and seropositivity for AMA before treatment denoted the presence of AIH overlap.The clinical findings,laboratory tests and histopathology of liver biopsies were analyzed and compared.Results Seventeen patients (16 females) had features of PBC-AIH overlap and 31 PBC did not (30 females).Fatigue (58.8%),decreased appetite (23.5%) and Jaundice (23.5%) were the main initial symptoms in overlap syndrome.There was no significant statistical difference between patients with overlap syndrome and PBC patients without overlap in average age,serum alkaline phosphatase levels,gamma-glutamyl-transpeptidase levels,and IgM levels.The serum transaminase,globulin and IgG levels were significantly higher in patient with overlap syndrome than those patients with PBC.Histological analysis showed all patients (17 cases) in overlap syndrome group had moderate or severe piecemeal necrosis,14(82.4%) had hepatocyte rosette formation,and 11(64.7%) had biliary changes.Conclusion Patients with the PBC-AIH overlap syndrome have high serum aspartate aminotransferase levels and IgG,moderate to severe interface hepatitis and biliary changes on histological examination.