EXTENDED REPORT

Familial systemic lupus erythematosus: the role of genetic and environmental factors

G E Eroglu, P F Kohler

Department of Medicine, Division of Allergy and Clinical Immunology, Tulane University School of Medicine, New Orleans, Louisiana, USA

Correspondence to:
Correspondence to:
Dr G E Eroglu, 1500 W Esplanade 35G, Kenner, LA 70065, USA;
geroglu{at}cs.com

Objective: To examine the contribution of genetic and environmental factors to disease occurrence in 26 families with two or more members affected with systemic lupus erythematosus (SLE).

Methods: Genetic and environmental factors were examined by HLA-A, B, C/DR typing and by determining the presence of lymphocytotoxic antibodies (LCA) in patients and their consanguineous and non-consanguineous relatives.

Results: No association between SLE and HLA-A, B, C antigens was found. There was, however, a significant association with HLA-DR2 in white subjects with SLE. The most striking finding was that HLA sharing was increased among the affected members, suggesting genetic similarities. Seven of 14 sib pairs (50%) who had concordant SLE were HLA identical as opposed to an expected 25%. Another interesting finding was that 15/18 (83%) patients with SLE and 11/22 (50%) consanguineous relatives had LCA, while 1/9 (11%) spouses, and 2/42 (5%) healthy controls had these antibodies.

Conclusion: Genetic factors have a role in the development and expression of SLE. Environmental factors may trigger the disease in genetically susceptible hosts.

Keywords: systemic lupus erythematosus; HLA; genetics

Abbreviations: LCA, lymphocytotoxic antibodies; SLE, systemic lupus erythematosus

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