Alessio Gerussi, Marcial Sebode, Eugenia Nofit, Anna Stoelinga, D. Bernasconi, Angela Leburgue, P Invernizzi, Christoph Schramm, Bart Van Hoek, Dina Tiniakos, Luigi Terracciano, Marco Carbone, Ansgar W. Lohse, on behalf of the ERN RARE-LIVER, the Global PBC Study Group, and the International Autoimmune Hepatitis Group (IAIHG)
BACKGROUND AND AIMS: Heterogeneity in the definition, diagnosis, and treatment of primary biliary cholangitis-autoimmune hepatitis (PBC-AIH) is present in guidelines and daily practice. This initiative aimed to establish expert consensus on terminology, diagnosis, and management. APPROACH AND RESULTS: The initiative was endorsed by the ERN RARE-LIVER, the Global PBC Study Group, and the IAIHG, in partnership with the European Society of Pathology. Using a modified RAND/UCLA Appropriateness Method, the Delphi process involved 2 voting rounds. The final panel comprised 74 hepatologists and 14 liver pathologists from diverse geographic regions. Consensus was reached that PBC-AIH should be defined as a variant rather than an overlap syndrome. An additional agreement was achieved on diagnostic criteria and the requirement of a liver biopsy to make the diagnosis. The panel agreed that the diagnosis should be periodically re-evaluated, since features can occur sequentially, and that PBC-AIH is associated with a worse prognosis than PBC alone unless treated by immunosuppression. The indication and choice of immunosuppressive therapy should be based on the severity of interface hepatitis (ideally confirmed by review from an experienced hepatopathologist), disease stage, age, comorbidities, and patient preference. CONCLUSIONS: This Delphi initiative established consensus in a complex, understudied area lacking evidence-based guidelines. The resulting statements offer a basis for prospective studies and standardized clinical protocols, aiming to enhance consistent management of PBC-AIH.