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      Editorial: Frontiers in Autoimmune Disease: Rheumatic Fever and Rheumatic Heart Disease


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          Rheumatic fever (RF) and rheumatic heart disease (RHD), its most clinically consequence, resulting from untreated throat Streptococcus pyogenes infection in susceptible children, are considered as models of autoimmune post-infectious disease. This Research Topic compiled clinical and scientific data and brings interesting viewpoints of clinicians and basic researchers. The ensemble of data and ideas certainly makes a new portrait of the mechanisms leading the autoimmune reactions. On the other hand, the clinical data contributes with the diagnosis and prevention of the disease. Rheumatic fever and RHD are still prevalent in diverse regions of the world (1–3). The correct diagnosis is very important as well as the clinical treatment. Briefly, in Brazil, both RF and RHD are still important diseases in different regions (4). A RF prevention program involving more than 700 children with RF/RHD was performed. This program evaluated the long-term evolution and outcomes after the control of recurrences. These data are presented and discussed by Mota et al. (5). Another interesting article, done by Spina et al., discusses the clinical diagnosis of acute rheumatic myocarditis in asymptomatic RHD patients (6). As RF and RHD are autoimmune diseases, the mechanisms leading to autoimmune reactions involve several molecules that play a role in the immune response against the bacteria (7, 8). As we know, protective response involves several molecules that are genetically controlled, from both innate and adaptive immune response, in order to eradicate an infection (8). Among these molecules, complement plays an important role in the immune response against S. pyogenes. The lectin pathway of complement and RHD is discussed by the group de Messias-Reason (9). Another interesting article on autoimmune targets is presented by Root-Bernstein. In his article, a parallel between molecular mimicry reactions on RHD and autoimmune myocarditis is established (10). Rheumatic fever and RHD are considered as prototypes of human autoimmune diseases, and no animal models could reproduce accurately the disease. In the past few years, however, experiments on Lewis rat showed some similar autoimmune reactions in the myocardium of S. pyogenes immunized mice. The article by the group of Ketheesan presented a historical overview of animal models that were used to investigate the pathogenesis of RF/RHD (11). I hope the readers enjoy this collection, and I and my co-editors are grateful of having the opportunity to prepare this Research Topic for Frontiers in Pediatrics. Conflict of Interest Statement The authors declare that the research was conducted in the absence of any commercial or financial relationships that could be construed as a potential conflict of interest.

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          Prevalence of rheumatic heart disease detected by echocardiographic screening.

          Epidemiologic studies of the prevalence of rheumatic heart disease have used clinical screening with echocardiographic confirmation of suspected cases. We hypothesized that echocardiographic screening of all surveyed children would show a significantly higher prevalence of rheumatic heart disease. Randomly selected schoolchildren from 6 through 17 years of age in Cambodia and Mozambique were screened for rheumatic heart disease according to standard clinical and echocardiographic criteria. Clinical examination detected rheumatic heart disease that was confirmed by echocardiography in 8 of 3677 children in Cambodia and 5 of 2170 children in Mozambique; the corresponding prevalence rates and 95% confidence intervals (CIs) were 2.2 cases per 1000 (95% CI, 0.7 to 3.7) for Cambodia and 2.3 cases per 1000 (95% CI, 0.3 to 4.3) for Mozambique. In contrast, echocardiographic screening detected 79 cases of rheumatic heart disease in Cambodia and 66 cases in Mozambique, corresponding to prevalence rates of 21.5 cases per 1000 (95% CI, 16.8 to 26.2) and 30.4 cases per 1000 (95% CI, 23.2 to 37.6), respectively. The mitral valve was involved in the great majority of cases (87.3% in Cambodia and 98.4% in Mozambique). Systematic screening with echocardiography, as compared with clinical screening, reveals a much higher prevalence of rheumatic heart disease (approximately 10 times as great). Since rheumatic heart disease frequently has devastating clinical consequences and secondary prevention may be effective after accurate identification of early cases, these results have important public health implications. Copyright 2007 Massachusetts Medical Society.
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            The Lectin Pathway of Complement and Rheumatic Heart Disease

            The innate immune system is the first line of host defense against infection and is comprised of humoral and cellular mechanisms that recognize potential pathogens within minutes or hours of entry. The effector components of innate immunity include epithelial barriers, phagocytes, and natural killer cells, as well as cytokines and the complement system. Complement plays an important role in the immediate response against microorganisms, including Streptococcus sp. The lectin pathway is one of three pathways by which the complement system can be activated. This pathway is initiated by the binding of mannose-binding lectin (MBL), collectin 11 (CL-K1), and ficolins (Ficolin-1, Ficolin-2, and Ficolin-3) to microbial surface oligosaccharides and acetylated residues, respectively. Upon binding to target molecules, MBL, CL-K1, and ficolins form complexes with MBL-associated serine proteases 1 and 2 (MASP-1 and MASP-2), which cleave C4 and C2 forming the C3 convertase (C4b2a). Subsequent activation of complement cascade leads to opsonization, phagocytosis, and lysis of target microorganisms through the formation of the membrane-attack complex. In addition, activation of complement may induce several inflammatory effects, such as expression of adhesion molecules, chemotaxis and activation of leukocytes, release of reactive oxygen species, and secretion of cytokines and chemokines. In this chapter, we review the general aspects of the structure, function, and genetic polymorphism of lectin-pathway components and discuss most recent understanding on the role of the lectin pathway in the predisposition and clinical progression of Rheumatic Fever.
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              Continuing education and environment in the center of action.


                Author and article information

                Front Pediatr
                Front Pediatr
                Front. Pediatr.
                Frontiers in Pediatrics
                Frontiers Media S.A.
                28 October 2015
                : 3
                : 91
                [1] 1Laboratory of Immunology, Heart Institute (InCor), School of Medicine, University of São Paulo , São Paulo, Brazil
                Author notes

                Edited by: Oswin Grollmuss, Centre Chirurgical Marie Lannelongue, France

                Reviewed by: Teresa M. Coque, Hospital Universitario Ramón y Cajal, Spain

                *Correspondence: Luiza Guilherme, luizagui@ 123456usp.br

                Specialty section: This article was submitted to Pediatric Cardiology, a section of the journal Frontiers in Pediatrics

                Copyright © 2015 Guilherme, Köhler and Faé.

                This is an open-access article distributed under the terms of the Creative Commons Attribution License (CC BY). The use, distribution or reproduction in other forums is permitted, provided the original author(s) or licensor are credited and that the original publication in this journal is cited, in accordance with accepted academic practice. No use, distribution or reproduction is permitted which does not comply with these terms.

                : 08 May 2015
                : 14 October 2015
                Page count
                Figures: 0, Tables: 0, Equations: 0, References: 11, Pages: 2, Words: 949

                rheumatic fever,rheumatic heart disease,autoimmune diseases,valvular damage,innate and adaptive immune response,animal model


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