Primary Sjögren's Syndrome and Other Autoimmune Diseases in Families

Publisher Website
Google Scholar
Abstract
The relationships of human leukocyte antigen (HLA) and heavy chain Ig (Gm) haplotypes to disease and autoantibody expression were examined in 6 large kindreds, each having 1 or more members with primary Sjogren''s syndrome. Various other autoimmune diseases and autoantibodies occurred among the 117 relatives in these families. The HLA and Gm haplotypes did not necessarily segregate persons into those with Sjogren''s syndrome, other autoimmune disorders, or serologic abnormalities, but HLA alleles DR3 and DR2 occurred in significant excess in relatives with Sjogren''s syndrome, irrespective of HLA haplotype. Segregation analysis suggested a Mendelian dominant genetic defect common to the many autoimmune diseases and serologic reactions that was not linked to HLA or Gm. A significant effect of female sex was also documented. Sjogren''s syndrome evidently results from the interaction of several HLA-linked and non-HLA-linked genes.