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Definition, diagnosis, and treatment of primary biliary cholangitis-autoimmune hepatitis (PBC-AIH) variant: An
Alessio Gerussi1,2, Marcial Sebode3,4, Eugenia Nofit1,2
1Division of Gastroenterology, Center for Autoimmune Liver Diseases, European Reference Network on Hepatological Diseases (ERN RARE-LIVER), Fondazione IRCCS San Gerardo dei Tintori, Monza, Italy.
Insights
Experts reached consensus defining primary biliary cholangitis-autoimmune hepatitis (PBC-AIH) as a variant, not overlap syndrome. This establishes standardized diagnostic criteria and management for PBC-AIH, improving patient care.
Area of Science:
- Hepatology
- Autoimmune Liver Diseases
- Clinical Consensus
Background:
- Significant heterogeneity exists in the definition, diagnosis, and management of primary biliary cholangitis-autoimmune hepatitis (PBC-AIH).
- Current guidelines and clinical practice lack standardization for this complex condition.
- This variability impacts patient outcomes and research efforts.
Purpose of the Study:
- To establish expert consensus on the terminology, diagnosis, and management of PBC-AIH.
- To address the lack of evidence-based guidelines in this understudied area.
- To provide a foundation for standardized clinical protocols and future research.
Main Methods:
- A modified RAND/UCLA Appropriateness Method was employed, utilizing a Delphi process with two voting rounds.
- The consensus panel consisted of 74 hepatologists and 14 liver pathologists from diverse geographical regions.
- The initiative was endorsed by major liver associations, including ERN RARE-LIVER, Global PBC Study Group, and IAIHG.
Main Results:
- Consensus was achieved defining PBC-AIH as a variant, distinct from an overlap syndrome.
- Agreement was reached on specific diagnostic criteria, including the necessity of liver biopsy.
- PBC-AIH was associated with a worse prognosis than PBC alone, emphasizing the need for immunosuppression.
- Indications and choices for immunosuppressive therapy were outlined, considering disease severity, stage, and patient factors.
Conclusions:
- This Delphi initiative successfully established expert consensus in the complex and understudied field of PBC-AIH.
- The developed statements provide a basis for prospective studies and standardized clinical protocols.
- The consensus aims to enhance consistent and improved management of PBC-AIH globally.
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.
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