Abstract
Inherited susceptibility to Addison's disease has been linked to the leukocyte antigen (HLA)-B8 allele, and by inference because of linkage disequilibrium between B8 and DR3, this susceptibility should be primarily to that of the DR3 antigen. Twenty four patients who had presented with overt Addison's disease, and another 28 patients who had been found to have adrenocortical autoantibodies, were HLA typed. Six of the Addisonian patients and 22 of the adrenal autoantibody positive patients had co-existing insulin dependent diabetes (IDD). In relation to HLA typing data from 253 Caucasian controls, Addison's disease was significantly associated with DR3 (X2 6.0 ; p = 0.014), DR4 (X2 7.4; p = 0.007) and DR3/DR4 heterozygosity (X2 25.4; p 0.0001). Of the 6 Addison's patients who lacked DR3 and DR4 alleles in their HLA phenotype, 3 had type I autoimmune polyglandular syndrome.
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