Selective IgA deficiency and the HLA-B8 antigen. Report of two cases with familial data

Abstract

Two patients had IgA deficiency, giardiasis, and the HLA-B8 antigen. The family of patient 1 included members with juvenile-onset diabetes mellitus, adrenal insufficiency, pernicious anemia, and hypothyroidism, a combination of unusual diseases that has been reported previously to occur as a syndrome with IgA deficiency and the HLA-B8 antigen. This coincidence makes it likely that these two patients and the one family previously described have a common pathogenic base, the inheritance of an abnormal immune-response gene that is acquired with the HLA-B8 antigen as a result of genetic dysequilibrium.

HLA‐A, B, C and DR antigens in immunoglobulin A deficiency

“…An association between IgA deficiency and genes within the HLA complex has been suggested by a number of investigators. Thus, in diabetes mellitus and concomitant IgA deficiency, the frequency of HLA B8 has been found to be significantly increased (Smith et al 1978), an association also confirmed in family studies (Van Thiel et al 1977, Perez-Jimenez et al 1981. However, juvenile onset diabetes mellitus is in itself associated with HLA B8, DR3 (for review see Ryder et al 1979), and therefore any linkage found be-tween HLA and IgA deficiency in these cases may be secondary to its well known disease association.…”