Hepatology. 2022 Feb 26. doi: 10.1002/hep.32426. Online ahead of print.
ABSTRACT
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.
PMID:35220609 | DOI:10.1002/hep.32426