PMID- 6437309 OWN - NLM STAT- MEDLINE DCOM- 19841218 LR - 20190619 IS - 0003-4819 (Print) IS - 0003-4819 (Linking) VI - 101 IP - 6 DP - 1984 Dec TI - Primary Sjogren's syndrome and other autoimmune diseases in families. Prevalence and immunogenetic studies in six kindreds. PG - 748-56 AB - The relationships of human leukocyte antigen (HLA) and heavy chain immunoglobulin (Gm) haplotypes to disease and autoantibody expression were examined in six large kindreds, each having one 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. These studies suggest that Sjogren's syndrome results from the interaction of several HLA-linked and non-HLA-linked genes. FAU - Reveille, J D AU - Reveille JD FAU - Wilson, R W AU - Wilson RW FAU - Provost, T T AU - Provost TT FAU - Bias, W B AU - Bias WB FAU - Arnett, F C AU - Arnett FC LA - eng GR - 5 M01RR00722/RR/NCRR NIH HHS/United States GR - IP60AM20558/AM/NIADDK NIH HHS/United States GR - IT35AM 07384/AM/NIADDK NIH HHS/United States PT - Case Reports PT - Journal Article PT - Research Support, Non-U.S. Gov't PT - Research Support, U.S. Gov't, P.H.S. PL - United States TA - Ann Intern Med JT - Annals of internal medicine JID - 0372351 RN - 0 (Autoantibodies) RN - 0 (HLA Antigens) RN - 0 (Histocompatibility Antigens Class II) RN - 0 (Immunoglobulin G) RN - 0 (Immunoglobulin Heavy Chains) SB - IM MH - Adult MH - Age Factors MH - Aged MH - Alleles MH - Autoantibodies/genetics MH - Autoimmune Diseases/epidemiology/*genetics/immunology MH - Female MH - Genes, Dominant MH - Genotype MH - HLA Antigens/genetics MH - Haploidy MH - Histocompatibility Antigens Class II/genetics MH - Humans MH - Immunoglobulin G/genetics MH - Immunoglobulin Heavy Chains/genetics MH - Male MH - Middle Aged MH - Pedigree MH - Sex Factors MH - Sjogren's Syndrome/epidemiology/*genetics/immunology MH - Syndrome MH - Xerophthalmia/genetics MH - Xerostomia/genetics EDAT- 1984/12/01 00:00 MHDA- 1984/12/01 00:01 CRDT- 1984/12/01 00:00 PHST- 1984/12/01 00:00 [pubmed] PHST- 1984/12/01 00:01 [medline] PHST- 1984/12/01 00:00 [entrez] AID - 10.7326/0003-4819-101-6-748 [doi] PST - ppublish SO - Ann Intern Med. 1984 Dec;101(6):748-56. doi: 10.7326/0003-4819-101-6-748.