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Canadian Journal of Gastroenterology
Volume 8, Issue 2, Pages 88-91
Clinical Hepatology

Primary Biliary Cirrhosis in HLA-Identical Twin Sisters

Hugh J Freeman1 and Robert J Bailey2

1Department of Medicine (Gastroenterology), University Hospital and University of British Columbia, Vancouver, British Columbia, Canada
2Department of Medicine (Gastroenterology), Royal Alexandra Hospital and University of Alberta, Edmonton, Alberta, Canada

Received 8 March 1993; Accepted 20 May 1993

Copyright © 1994 Hindawi Publishing Corporation. This is an open access article distributed under the Creative Commons Attribution License, which permits unrestricted use, distribution, and reproduction in any medium, provided the original work is properly cited.


Human leukocyte antigen (HLA)-identical twin sisters with chronic liver disease were evaluated. Both had a childhood history of transient jaundice suggestive of a possible infectious cause. Subsequent studies in both siblings at age 51 years revealed antimitochondrial antibody-positive primary biliary cirrhosis. This report documents HLA-identical twins with primary biliary cirrhosis, providing added evidence for a genetically determined abnormal immune response in this liver disorder, possibly to a specific viral or other environmental factor.