Evidence for linkage of IgA deficiency with the major histocompatibility complex

A 57-year-old woman with IgA deficiency and Still's disease was the proband in a 20-member, three-generation kindred in which we studied the possible linkage of IgA deficiency with her HLA-A1-B8 haplotype. The presence of paternal A1-B8 haplotype complicated the analysis. Known maternal HLA-A1-B8 haplotype, present in three of the children of the proband, was associated with IgA deficiency, whereas all five family members with exclusively paternal A1-B8 had normal IgA. Of three third-generation family members whose A1-B8 haplotype was of indeterminate origin--that is, potentially either maternally or paternally derived--two had IgA deficiency and one did not.