Worse Response to Ursodeoxycholic Acid in Primary Biliary Cholangitis Patients with Autoimmune Hepatitis Features.

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. The aim of 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 >1 year. The biochemical response was evaluated at 1 year after the treatment of UDCA. RESULTS: Among 432 patients with PBC, 166 (38.4%) patients did not achieve biochemical response within 1 year of UDCA treatment. Nonresponders had a lower albumin level and higher immunoglobulin G, alanine transaminase (ALT), alanine aminotransferase (AST), alkaline phosphatase, glutamyl transpeptidase and total bilirubin 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 levels 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 nonresponsive 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.

Ursodeoxycholic acid therapy in hepatobiliary disease.

Ursodeoxycholic acid is a hydrophilic bile acid that under normal circumstances represents a small fraction of the bile acid pool in humans. It is effective in dissolving cholesterol gallstones in appropriately selected patients. Ursodeoxycholic acid improves serum alkaline phosphatase and aminotransferase levels in primary biliary cirrhosis, but its effects on rates of liver transplantation and death are less certain. Ursodeoxycholic acid has had promising [corrected] effects in several other cholestatic liver diseases, such as cystic fibrosis and intrahepatic cholestasis of pregnancy, but data are too preliminary to make recommendations about its routine use in these conditions. Its effects are mediated by amelioration of damage to cell membranes caused by retained toxic bile acids. Ursodeoxycholic acid improves biliary secretion of bile acids, may improve bile flow, and it has immunomodulatory properties that may reduce immune-mediated liver damage. However, its use in the treatment of cholestatic liver disease remains uncertain pending additional randomized trials.