P2419 - Clinical Characteristics and Outcomes of Patients with Primary Biliary Cholangitis–Autoimmune Hepatitis Overlap Syndrome – A Multicentric Cohort Study
Mohamed Eissa, MD1, Lytvyak Ellina, MD, PhD, DABOM, FRCPC1, Hin Hin Ko, MD, FRCPC2, Mark Swain, MD3, Lawrence Worobetz, MD4, Jennifer Flemming, MD5, Cynthia Tsien, MD6, Angela Cheung, MD7, Aliya Gulamhusein, MD6, Karim Qumosani, MD8, Catherine Vincent, MD9, Julian Hercun, MD9, Tianyan Chen, MD10, Dusanka Grbic, MD11, Kevork M. Peltekian, MD12, Bettina E. Hansen, MD13, Aldo Montano-Loza, MD14, Gideon Hirschfield, PhD15, Andrew Mason, MD14 1University of Alberta, Edmonton, AB, Canada; 2University of British Columbia, Vancouver, BC, Canada; 3University of Calgary, Calgary, AB, Canada; 4University of Saskatchewan, Saskatoon, SK, Canada; 5Queen's University, Kingston, ON, Canada; 6University of Toronto, Toronto, ON, Canada; 7University of Ottawa, Ottawa, ON, Canada; 8London Health Sciences Centre - University Hospital, London, ON, Canada; 9Centre hospitalier de l'Université de Montréal, Montreal, PQ, Canada; 10Research Institute of McGill University Health Centre, Montreal, PQ, Canada; 11Université de Sherbrooke, Sherbrooke, PQ, Canada; 12Queen Elizabeth II Health Sciences Centre, Halifax, NS, Canada; 13Toronto General Hospital, Toronto, ON, Canada; 14University of Alberta Hospital, Edmonton, AB, Canada; 15Toronto Centre for Liver Disease, Toronto, ON, Canada
Introduction: Autoimmune hepatitis (AIH) and Primary Biliary Cholangitis (PBC) are the two main immune-mediated liver diseases. Occasionally, AIH can present with features of PBC. This overlap syndrome (OS) lacks standard diagnostic criteria and management strategies. “Paris criteria” (1998) is the most recognized diagnostic criteria for OS. The frequency of OS in literature has been reported between 7% and 13%. Our study will aim to evaluate the clinical characteristics and outcomes of OS patients in a multicentric Canadian cohort study.
Methods: This cohort study included 198 patients originally diagnosed as OS in the Canadian Network for Autoimmune Liver Disease (CaNAL) registry which collects data from multiple tertiary centres across Canada. Available biochemical results and biopsy reports were reviewed and the diagnosis of OS for patients was reviewed based on Paris criteria (1998).
Results: 57 patients (29%) did not have enough data to apply the criteria. 78 patients (55%) have met the Paris criteria for OS, of which 69 (88%) exhibited true OS on biopsy and 9 (12%) exhibited only AIH on biopsy. 63 patients (45%) did not meet criteria for OS, with 22 (35%) exhibiting PBC on biopsy and 19 (30%) exhibiting AIH on biopsy. 16 patients (25%) showed OS on biopsy despite not meeting Paris criteria. Of those that met the Paris criteria for OS, the majority were female (88%) and 9 patients (12%) received liver transplant.
Discussion: Our results show that OS tend to be over-diagnosed which raises concerns that patients with PBC could be unnecessarily exposed to steroids as a result. In our cohort, OS patients exhibited female predominance. Further analysis of OS patients will be performed to determine clinical characteristics, outcomes of treatment, biochemical response, prognosis at one year, progression to cirrhosis and need for liver transplantation or death from liver failure. We will also compare outcomes to those diagnosed as either AIH or PBC in our cohort.
Disclosures:
Mohamed Eissa indicated no relevant financial relationships.
Lytvyak Ellina indicated no relevant financial relationships.
