Systemic lupus erythematosus: immunopathogenesis and the card game analogy
Isenberg, D.A.
Journal of Rheumatology. Supplement 48: 62-66
1997
ISSN/ISBN: 0380-0903 PMID: 9150121 Document Number: 480189
Systemic lupus erythematosus (SLE) is a multifactorial disease with both genetic and environmental etiology. The complexity of factors contributing to SLE are considered in an analogy with a card game. The hearts suit represents sex hormones. SLE is a disease of marked female prevalence and abnormal estrogen metabolism has been described in women with SLE. The clubs suit considers complement and other genetic factors. Increased risk of SLE has been described in association with some HLA markers and the complement C4A0 null allele. Although convincing evidence has not yet emerged, other candidate genes of importance are T cell receptor genes and genes encoding B cell immunoglobulin receptors and antibodies. Recently, abnormalities of apoptosis and of expression of the protooncogene Bcl-2 have been investigated. Overall different genes have been shown to increase the risk of SLE, and/or to influence the development of particular antibodies, and particular subsets of disease. The diamonds suit considers antigens and antibodies in the etiopathogenesis of SLE. Numerous autoantibodies have been described that bind a variety of targets on the cell surface, within the cytoplasm, or in the nucleus. It is generally agreed that autoantibodies develop as a consequence of both generalized polyclonal activation and antigen drive. The final suit of spades considers infectious, environmental, and other agents such as drugs, as triggers in the development of SLE.