Current opinion in gastroenterology · 2025
PBC-AIH variant syndrome: emerging new terminology and a new approach to diagnosis and management.
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Sebode Marcial, Gerussi Alessio
Аннотация
PURPOSE OF REVIEW: This review summarizes the recent developments of one of the most controversial entities in hepatology, variant syndromes of primary biliary cholangitis (PBC) with characteristics of autoimmune hepatitis (AIH).
RECENT FINDINGS: Recently a consensus process was initiated to find agreement on the terminology, diagnostic criteria and treatment recommendations for patients with PBC-AIH variant syndromes. The concept and terminology of a variant syndrome, with one component of either AIH or PBC dominating over the other, is currently preferred. No single test can establish the diagnosis of a variant syndrome, only a combination of biochemical, serological and/or histological tests can support the diagnosis. If classical PBC is dominating, histology is mandatory for the diagnosis of a PBC-AIH variant syndrome. Treatment of PBC-AIH variants is based on a combination of ursodeoxycholic acid and immunosuppression. Since the prognosis of a PBC-AIH variant syndrome seems to be worse than the prognosis of classical PBC, the diagnosis of PBC-AIH must not be missed.
SUMMARY: The recent consensus process on PBC-AIH variant syndromes does not provide answers to all questions regarding this entity. Rather, it serves as a starting point for future studies to confirm or even challenge the current consensus.
RECENT FINDINGS: Recently a consensus process was initiated to find agreement on the terminology, diagnostic criteria and treatment recommendations for patients with PBC-AIH variant syndromes. The concept and terminology of a variant syndrome, with one component of either AIH or PBC dominating over the other, is currently preferred. No single test can establish the diagnosis of a variant syndrome, only a combination of biochemical, serological and/or histological tests can support the diagnosis. If classical PBC is dominating, histology is mandatory for the diagnosis of a PBC-AIH variant syndrome. Treatment of PBC-AIH variants is based on a combination of ursodeoxycholic acid and immunosuppression. Since the prognosis of a PBC-AIH variant syndrome seems to be worse than the prognosis of classical PBC, the diagnosis of PBC-AIH must not be missed.
SUMMARY: The recent consensus process on PBC-AIH variant syndromes does not provide answers to all questions regarding this entity. Rather, it serves as a starting point for future studies to confirm or even challenge the current consensus.