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