Primary biliary cholangitis: Epidemiology, prognosis, and treatment

Primary biliary cholangitis (PBC) is a chronic cholestatic autoimmune liver disease characterized by a destructive, small duct, and lymphocytic cholangitis, and marked by the presence of antimitochondrial antibodies. The incidence and prevalence of PBC vary widely in different regions and time periods, and although disproportionally more common among White non-Hispanic females, contemporary data show a higher prevalence in males and racial minorities than previously described. Outcomes largely depend on early recognition of the disease and prompt institution of treatment, which, in turn, are directly influenced by provider bias and socioeconomic factors. Ursodeoxycholic acid remains the initial treatment of choice for PBC, with obeticholic acid and fibrates (off-label therapy) reserved as add-on therapy for the management of inadequate responders or those with ursodeoxycholic acid intolerance. Novel and repurposed drugs are currently at different stages of clinical development not only for the treatment of PBC but also for its symptomatic management. Here, we summarize the most up-to-date data regarding the epidemiology, prognosis, and treatment of PBC, providing clinically useful information for its holistic management.

Automated summary

Primary biliary cholangitis (PBC) is a chronic autoimmune liver disease characterized by destructive cholangitis and the presence of antimitochondrial antibodies. The prevalence of PBC varies globally and may be higher in males and racial minorities than previously thought. Early recognition and treatment are crucial for PBC outcomes, which can be influenced by provider bias and socioeconomic factors. Ursodeoxycholic acid is the initial treatment choice, with other drugs being considered as add-on therapy. This article provides a comprehensive summary of the epidemiology, prognosis, and treatment of PBC for holistic management.