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From rheumatic fever to Libman-Sacks endocarditis: is there any possible pathogenetic link?

Department of Internal Medicine B and The Center for Autoimmune Diseases, Sheba Medical Center, Tel-Hashomer, Sackler Faculty of Medicine, Tel-Aviv University, Israel

A Aron-Maor

Department of Internal Medicine B and The Center for Autoimmune Diseases, Sheba Medical Center, Tel-Hashomer, Sackler Faculty of Medicine, Tel-Aviv University, Israel

Y Shoenfeld

Department of Internal Medicine B and The Center for Autoimmune Diseases, Sheba Medical Center, Tel-Hashomer, Sackler Faculty of Medicine, Tel-Aviv University, Israel, Incumbent of the Laura Schwarz-Kipp Chair for Research of Autoimmune Diseases, Sackler Faculty of Medicine, Tel-Aviv University, Israel, shoenfel{at}post.tau.ac.il

The heart lesions of rheumatic fever and the heart involvement in antiphospholipid syndrome (APS), have different clinical pictures. Yet, there are several common characteristics linking both diseases: 1) central nervous system (CNS) and heart involvement; 2) molecular mimicry between the a pathogen and the origin of the disease; 3) cross reacting antibodies between the pathogen and self molecules; 4) endothelial cell activation in the ‘crime-area’ i.e., the valves; 5) some of the patients with RF have circulating antiphospholipid antibodies, while APS may be associated with streptococcal infection; and 6) recently, a cross-reactivity between antibodies directed to the streptococcal M-protein and its synthetic derivative in rheumatic fever (RF) and antibodies derived from APS patients targeting the beta-2-glycoprotein-I (ß2GPI) and a ß2GPI related synthetic peptide. In the current paper, we summarize the possible links between the heart involvement in RF and APS.

Key Words: anti-b2GPI • antiphosphplipid syndrome • autoimmunity • Libman-Sacks endocarditis • M-protein • rheumatic fever

Lupus, Vol. 14, No. 9, 697-701 (2005)
DOI: 10.1191/0961203305lu2203oa


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