Role of autoantibodies in the clinical management of primary biliary cholangitis

Primary biliary cholangitis (PBC) is a chronic cholestatic liver disease characterized by immune-driven destruction of small intrahepatic bile ducts leading a proportion of patients to hepatic failure over the years. Diagnosis at early stages in concert with ursodeoxycholic acid treatment has been linked with prevention of disease progression in the majority of cases. Diagnosis of PBC in a patient with cholestasis relies on the detection of disease-specific autoantibodies, including anti-mitochondrial antibodies, and disease-specific anti-nuclear antibodies targeting sp100 and gp210. These autoantibodies assist the diagnosis of the disease, and are amongst few autoantibodies the presence of which is included in the diagnostic criteria of the disease. They have also become important tools evaluating disease prognosis. Herein, we summarize existing data on detection of PBC-related autoantibodies and their clinical significance. Moreover, we provide insight on novel autoantibodies and their possible prognostic role in PBC patients.

Automated summary

Primary biliary cholangitis (PBC) is a chronic liver disease characterized by immune-mediated destruction of small bile ducts, which can progress to hepatic failure. Early diagnosis and ursodeoxycholic acid treatment have been shown to prevent disease progression in most cases. The presence of disease-specific autoantibodies, including anti-mitochondrial antibodies, anti-sp100 antibodies, and anti-gp210 antibodies, is used for the diagnosis of PBC. These autoantibodies not only aid in the diagnosis of the disease but also have prognostic value. This article summarizes the current knowledge on the detection and clinical significance of PBC-related autoantibodies and discusses the potential prognostic role of novel autoantibodies in PBC patients.