Abstracts of the 2023 Annual Meeting of the ALEH
More infoThe overlap between primary biliary cholangitis (PBC) and primary sclerosing cholangitis (PSC) is a very rare association infrequently described in the literature, with only a few cases reported as of 2023. There are no case reports published in Latin America. Its diagnosis is based on 2 of the following 3 criteria: biochemical cholestasis, presence of AMA (anti-antimitochondrial antibodies) or anti-gp210 or anti-sp100 antibodies, and a liver biopsy compatible with PBC, associated with imaging consistent with intrahepatic focal bile duct strictures or compatible biopsy with PSC. Diagnosing this association could be important for the follow-up of these patients since they may present relevant complications to screen or present an inadequate response to treatment. This study aimed to describe patients with PBC and PSC overlap syndrome in a Latin American hospital.
Materials and MethodsPerform an observational, retrospective and descriptive study. The clinical records of patients with PBC and PSC treated at the Hospital Clínico Universidad de Chile between 2021 and 2023 were reviewed. Laboratory information, imaging, and indicated treatment were analyzed.
ResultsDuring the study period, 8 patients with PBC-PEC overlap syndrome were identified; all patients were female, with cholestatic alterations in their liver profile, positive AMA/M2-3E type antibodies, and with focal stenosis on magnetic resonance cholangiography, compatible with PSC (except in one case, which was classified as small duct PSC on her liver biopsy). All received therapy with ursodeoxycholic acid in doses between 13 and 15 mg/kg, with good response (Table).
ConclusionThe overlap between PBC and PSC is rare; it is probably underdiagnosed and perhaps patients with PBC should be studied more often with magnetic resonance cholangiography. The overlap is not associated with worse response to ursodeoxycholic acid.
