Diagnosis and Treatment of Primary Biliary Cirrhosis-Autoimmune Hepatitis Overlapping Syndrome: An Ana-lysis of 30 Cases

Background: Autoimmune liver diseases include autoimmune hepatitis (AIH), primary biliary cirrhosis (PBC) and primary sclerosing cholangitis (PSC), etc. The clinical manifestation, biochemical, immunological and histological changes of these diseases are varied and overlapped, the differential diagnosis is rather difficult. Aims: To study the clinical features, diagnosis and treatment of patients with PBC-AIH overlapping syndrome. Methods: Thirty cases each of PBC-AIH overlapping syndrome, AIH and PBC were selected from 164 patients with autoimmune liver diseases, the diagnosis and response to therapy of the patients with PBC-AIH overlapping syndrome were analyzed particularly. Results: PBC-AIH overlapping syndrome constituted 18% of the patients with autoimmune liver disease recruited in this study. No significant differences were found in sex, age and the symptoms of jaundice and pruritus among the three groups. In PBC-AIH group, the levels of aspartate aminotransferase (AST) and alanine aminotransferase (ALT) were significantly higher than those in AIH and PBC group; and the levels of alkaline phosphatase (ALP) and γ-glutamyl transpeptidase (GGT) were markedly higher than those in AIH group, but lower than those in PBC group (P0.05 or P0.01). After combination therapy with immunosuppressant, all the above parameters of liver function improved significantly in AIH group (P0.05 or P0.01); while in PBC-AIH group the levels of bilirubin, AST and ALT decreased significantly as well (P0.05 or P0.01), but the time required for improvement was longer than that in AIH group (P0.05 or P0.01). Conclusions: PBC-AIH overlapping syndrome is not rare as seen in this study. Its diagnosis should be based on the clinical presentation, biochemical, immunological indices and histological changes. Long-term combination therapy with corticosteroids, azathioprine and ursodeoxycholic acid (UDCA) is effective in patients with PBC-AIH overlapping syndrome.