ASSOCIATION OF HLA-B8 WITH VISCERAL DISEASE IN SYSTEMIC-SCLEROSIS

  • 1 January 1978
    • journal article
    • research article
    • Vol. 31  (3) , 351-356
Abstract
Seventy-one patients with systemic sclerosis (SS) were typed for 27 HLA alleles of the A and B loci, and the findings were related to the extent of visceral disease and tests of cellular immune competence in a subgroup of 52 of these patients. Nineteen patients with widespread visceral involvement and more rapidly progressive disease had an increased frequency of HLA-B8 (relative risk = 4.14; P < 0.05) when compared 33 less severely affected patients and 3000 controls. Patients with severe and progressive disease also had defective cell-mediated immunity with reductions in the numbers of circulating thymus-dependent (T) lymphocytes and in the lymphocyte transformation response to phytohemagglutinin. A genetic factor, such as an abnormal immune response gene, may be involved in the progression of the disease.