The association of HLA-B8 with visceral disease in systemic sclerosis
Hughes, P.; Gelsthorpe, K.; Doughty, R.W.; Rowell, N.R.; Rosenthal, F.D.; Sneddon, I.B.
Clinical and Experimental Immunology 31(3): 351-356
1978
ISSN/ISBN: 0009-9104 PMID: 306903 Document Number: 130573
Seventy-one patients with systemic sclerosis (SS) were typed for twenty-seven HLA alleles of the A and B loci, and the findings were related to both the extent of visceral disease and tests of cellular immune competence in a subgroup of fifty-two of these patients. Nineteen pa;ients with widespread visceral involvement and more rapidly progressive disease had an increased frequency of HLA-B8 (relative risk = 4.14; P less than 0.05) when compared to thirty-three less severely affected patients and 3000 controls. Patients with severe and progressive disease also had defective cell-mediated immunity with reductions in both the numbers of circulating thymus-dependent (T) lymphocytes and in the lymphocyte transformation response to phytohaemagglutinin. These findings suggest that a genetic factor, such as an abnormal immune response gene, may be involved in the progression of the disease.