Introduction and Context
Primary biliary cholangitis–autoimmune hepatitis (PBC-AIH) is one of the most debated entities in autoimmune liver disease. In practice, patients may show both cholestatic features typical of primary biliary cholangitis (PBC) and inflammatory features suggestive of autoimmune hepatitis (AIH). Yet terminology, diagnostic thresholds, and treatment pathways have varied widely across guidelines and specialty centers.
That variability matters. Some patients are treated too conservatively, leaving inflammatory hepatitis insufficiently suppressed; others are exposed to long-term immunosuppression without enough evidence that the mixed phenotype truly warrants it. To address this gap, an international expert Delphi process, endorsed by ERN RARE-LIVER, the Global PBC Study Group, the International Autoimmune Hepatitis Group (IAIHG), and the European Society of Pathology, sought to establish a practical consensus.
