From rheumatic fever to Libman-Sacks endocarditis: is there any possible pathogenetic link?
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Published on: April 7, 2015
M Blank1, A Aron-Maor, Y Shoenfeld
1Department of Internal Medicine B, The Center for Autoimmune Diseases, Sheba Medical Center, Tel-Hashomer, Sackler Faculty of Medicine, Tel-Aviv University, Israel.
Insights
Rheumatic fever and antiphospholipid syndrome share common mechanisms involving the heart and central nervous system. Both diseases exhibit molecular mimicry and cross-reacting antibodies, suggesting shared autoimmune pathways.
Area of Science:
- Immunology
- Cardiology
- Rheumatology
Background:
- Rheumatic fever (RF) and antiphospholipid syndrome (APS) present distinct cardiac manifestations.
- Shared features include central nervous system and heart involvement, molecular mimicry, and cross-reacting antibodies.
- Endothelial cell activation at the heart valves is a common pathological site.
Purpose of the Study:
- To explore the potential links between heart involvement in RF and APS.
- To highlight common pathogenic mechanisms underlying these two conditions.
Main Methods:
- Review of existing literature on RF and APS heart involvement.
- Analysis of shared immunological phenomena such as molecular mimicry and antibody cross-reactivity.
Main Results:
- Both RF and APS involve the CNS and heart, with endothelial activation in valves.
- Molecular mimicry and cross-reacting antibodies against pathogens and self-molecules are implicated in both.
- Patients with RF may have antiphospholipid antibodies, and APS can be linked to streptococcal infections.
- Cross-reactivity between antibodies to streptococcal M-protein and APS-related antibodies targeting beta-2-glycoprotein-I (beta2GPI) has been observed.
Conclusions:
- Common autoimmune pathways, particularly involving molecular mimicry and cross-reactive antibodies, may link heart involvement in RF and APS.
- Further research into these shared mechanisms could inform diagnostic and therapeutic strategies for both conditions.
Abstract:
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 (beta2GPI) and a beta2GPI related synthetic peptide. In the current paper, we summarize the possible links between the heart involvement in RF and APS.
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