Increased frequency of B8/DR3 in scleroderma and association of the haplotype with impaired cellular immune response.

Twenty-eight patients with scleroderma were typed for 40 HLA antigens. A highly significant increase in the frequency of HLA-B8 and HLA-DR3 was observed, which was not related to the severity of the disease. In vitro lymphocyte stimulation tests were performed in 26 patients. In addition, humoral and in vitro cellular immune responses to a primary test antigen were measured in 12 of them. Although the group of scleroderma patients as a whole had an impaired cellular response, only the B8/DR3+ patients had a strongly depressed response, whereas the immune response of the others was normal. These findings suggest an association of the haplotype B8/DR3 with impaired cellular immunity.