The summaries are free for public
use. The Chronic Liver Disease
Foundation will continue to add and
archive summaries of articles deemed
relevant to CLDF by the Board of
Trustees and its Advisors.
Ethnicity, disease severity and survival in Canadian patients with Primary Biliary Cholangitis
Hepatology. 2022 Feb 26. doi: 10.1002/hep.32426. Online ahead of print.
Surain B Roberts12, Gideon M Hirschfield12, Lawrence J Worobetz3, Catherine Vincent4, Jennifer A Flemming5, Angela Cheung6, Karim Qumosani7, Mark Swain8, Dusanka Grbic9, Hin Hin Ko10, Kevork Peltekian11, Nazia Selzner12, Lusine Abrahamyan212, Bishoi Aziz13, Ellina Lytvyak13, Kattleya Tirona1, Aliya F Gulamhusein12, Harry LA Janssen1, Aldo J Montano-Loza13, Andrew L Mason13, Bettina E Hansen12, Canadian Network for Autoimmune Liver disease (CaNAL)
Toronto Centre for Liver Disease, Toronto General Hospital, University Health Network, Toronto, Canada.
Institute of Health Policy, Management and Evaluation, University of Toronto, Toronto, Canada.3
Department of Medicine, University of Saskatchewan, Saskatoon, Canada.
Département de Médecine, Université de Montréal, Montréal, Canada.
Department of Medicine, Queen's University, Kingston, Canada.
Department of Medicine, University of Ottawa, Ottawa, Canada.
Department of Medicine, Western University, London, Canada.
Department of Medicine, University of Calgary, Calgary, Canada.
Department de Médecine, Université de Sherbrooke, Sherbrooke, Canada.
Department of Medicine, University of British Columbia, Vancouver, Canada.
Department of Medicine, Dalhousie University, Halifax, Canada.
Toronto General Hospital Research Institute, University Health Network, Toronto, Canada.
Department of Medicine & Dentistry, University of Alberta, Edmonton, Canada.
Background & aims: We investigate associations between ethnicity, survival, and disease severity in a diverse Canadian cohort of patients with Primary Biliary Cholangitis (PBC).
Approach & results: Patients with PBC were included from the Canadian Network for Autoimmune Liver disease (CaNAL). Ethnicity was defined using a modified list adopted from Statistics Canada and ethnicities with small sample were grouped. Clinical events were defined as liver decompensation, hepatocellular carcinoma, liver transplantation (LTx), or death. Clinical event-free and LTx-free survival were analyzed using Cox regression. Trajectories of serum liver function tests were assessed over time using mixed-effects regression. HRQOL was assessed using the Short Form 36 (SF-36), PBC-40 questionnaire, and 5-D Itch scale and analyzed using mixed-effects regression. The cohort included 1538 patients with PBC from six sites and was comprised of 82% White patients, 4.7% Indigenous, 5.5% East Asian, 2.6% South Asian, and 5.1% Miscellaneous ethnicities. Indigenous patients were the only ethnic group with impaired liver transplant-free and event-free survival compared to White patients (HR 3.66, 95%CI 2.23-6.01; HR 3.09, 95%CI 1.94-4.92). Indigenous patients were more likely to have a clinical event before diagnosis (10%) than all other ethnicity groups despite similar age at diagnosis. Indigenous patients presented with higher alkaline phosphatase, total bilirubin, and GLOBE scores than White patients and these relative elevations persisted during follow-up.
Conclusions: Indigenous Canadians with PBC present with advanced disease and have worse long-term outcomes compared to White patients.