Background: There is limited evidence on the treatment response of primary biliary cholangitis (PBC) with autoimmune hepatitis (AIH) features but not meet the criteria of PBC-AIH syndromes. This study was to elucidate the clinical characteristics of PBC patients with features of AIH.
Methods: We included patients with diagnostic criteria of PBC. All patients were treated with ursodeoxycholic acid (UDCA) and without immunosuppressive agents for more than one year. The biochemical response was evaluated at one year after treatment of UCDA.
Results: Among 432 patients with PBC, 166 (38.4%) patients did not achieve biochemical response within one year of UDCA treatment. Non-responders had lower albumin (ALB) level and higher immunoglobulin G (IgG), alanine transaminase (ALT), alanine aminotransferase (AST), alkaline phosphatase (ALP), glutamyl transpeptidase (GGT) and total bilirubin (TB) levels (P < 0.05). The response rates were significantly lower in patients with elevated level of IgG or ALT or AST. Moreover, the higher the IgG or AST level was, the lower the response rate was in patients with PBC regardless of cirrhosis. For patients with cirrhosis, there was no differences among patients with different level of ALT. Patients in the PBC with AIH features group had a significant lower response rate than patients in the PBC-only group. Among the 139 patients who underwent liver biopsy, 54 were non-responsive to UDCA and 48 (88.9%) shown mild interface hepatitis.
Conclusion: In conclusion, PBC patients with AIH features had a worse response to UDCA therapy.
Combination Therapy of Ursodeoxycholic Acid and Corticosteroids for Primary Biliary Cirrhosis with Features of Autoimmune Hepatitis: A Meta-Analysis
