Treatment response in primary biliary cholangitis: The role of autoimmune hepatitis features

Abstract

Introduction and aim: Primary biliary cholangitis is a rare disease with scarce epidemiological data in Southern Europe. The authors aimed to evaluate treatment response in a cohort of patients. Materials and methods: This retrospective observational single-centre study included patients with diagnostic criteria of primary biliary cholangitis. Data on disease presentation, laboratory results, treatment and clinical endpoints were collected and analyzed. Results: Fifty-three patients were included, 89% women, with mean age of 62 ± 15 years at diagnosis. The majority was asymptomatic (49%), tested positive for antimitochondrial antibodies (96%) and had increased alkaline phosphatase (median = 214 U/L). 75% of the patients had liver histology and the majority were in Ludwig's stage I (42%). Autoimmune hepatitis (AIH) features were found in seven patients (13%). All were treated with ursodeoxycholic acid (UDCA) and 56% achieved biochemical response at one year; patients with AIH features exhibited steeper decreases in alkaline phosphatase (p = 0.007) and reached the endpoint of 40% decrease in alkaline phosphatase more frequently (p = 0.017). Conclusion: In conclusion a significant proportion of patients failed to achieve an adequate response to UDCA treatment. The response rate of patients with AIH features was better, which could be related to a different phenotype or to the potential impact of immunosuppressive agents.

Keywords

• Overlap syndrome
• Alkaline phosphatase
• Ursodeoxycholic acid