PBC–AIH variant syndrome: emerging new terminology and a new approach to diagnosis and management

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.

Copyright © 2026 The American College of Gastroenterology. The American Journal of Gastroenterology.

Note: Obeticholic acid, marketed under the brand name Ocaliva^® for the treatment of primary biliary cholangitis (PBC), was voluntarily withdrawn from the US market by Intercept Pharmaceuticals following a request from the US Food and Drug Administration (FDA) on 11/14/2025