Pattern of Hereditary Susceptibility in Rheumatic Fever

Wilson, May G.; Schweitzer, Morton D.

doi:10.1161/01.cir.10.5.699

Cited by 73 publications

(16 citation statements)

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“…Again, individual factor is determinant of disease pattern and is poorly understood. Familiar aggregation [9,10], similarity of disease patterns between siblings and identical twin [11] and HLA correlation studies [8] are evidence for a genetic influence on RF susceptibility and manifestation. The suspect-involved genes fall mainly into those capable of immunologic mediation.…”

Section: Introductionmentioning

confidence: 99%

Understanding rheumatic fever

2011

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Through a comprehensive review of the recent findings on rheumatic fever, we intend to propose a new physiopathologic model for this disease. A Medline search was performed for all articles containing the terms rheumatic fever or rheumatic heart disease in title or abstract from 1970 to 2011. Best evidence qualitative technique was used to select the most relevant. The scientific interest on rheumatic fever has notably diminished throughout the twentieth century as evidenced by the comparison of the proportion of articles in which RF was a subject in 1950 (0.26%) and today (0.03%) [Pubmed]. However, RF remains a major medical and social problem in the developing world and in the so-called hotspots, where it still causes around 500.000 deaths each year, not too different from the pre-antibiotic era. The role of genetic factors in RF susceptibility is discussed. Familiar aggregation, similarity of disease patterns between siblings, identical twin, and HLA correlation studies are evidence for a genetic influence on RF susceptibility. The suspect-involved genes fall mainly into those capable of immunologic mediation. Molecular mimicry explains the triggering of RF, but an intense and sustained inflammation is needed to cause sequels. Also, RF patients vary greatly in terms of symptoms. It is likely that a genetic background directing immune response towards a predominantly Th1 or Th2 pattern contributes to these features. The recent findings on rheumatic fever provide important insight on its physiopathology that helps understanding this prototype post-infectious autoimmune disease giving insights on other autoimmune conditions.

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Section: Introductionmentioning

confidence: 99%

Understanding rheumatic fever

2011

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“…Early but unconfirmed studies suggested that susceptibility to rheumatic fever followed a simple autosomal recessive pattern of inheritance (4)(5)(6), or paralleled the inheritance of ABO blood group or ABO secretor status (7)(8)(9)(10)(11)(12)(13)(14)(15)(16)(17). The pathogenesis of rheumatic fever is also thought to involve an aberrant immunological reaction, either humoral or cellular, or both, triggered by an antecedant group A streptococcal infection (18)(19)(20)(21)(22)(23)(24)(25).…”

Section: Introductionmentioning

confidence: 99%

Association of class II human histocompatibility leukocyte antigens with rheumatic fever.

Ayoub¹,

Barrett²,

Maclaren³

et al. 1986

J. Clin. Invest.

171

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The association of class I and II HLA antigens with rheumatic fever and its manifestations was examined in 72 patients, including 48 blacks and 24 Caucasians. No significant association was found between class I antigens and rheumatic fever. In contrast, HLA-DR2 and HLA-DR4 phenotypes were encountered in a significantly higher frequency in black and Caucasian patients with rheumatic fever, respectively, compared with the control populations (P < 0.005). The most significant association (P < 0.005) of these DR antigens with a major manifestation of rheumatic fever was found for mitral insufficiency. In addition, a significant association was encountered between persistent elevation of antibody to the group A streptococcal carbohydrate and HLA-DR4 in Caucasian patients (P < 0.04) or HLA-DR2 in the black patients (P < 0.001). The frequency of HLA-DR2/ 4 heterozygotes among patients with rheumatic fever did not differ significantly from controls. These findings support the concept of a genetically determined susceptibility to rheumatic fever and, particularly, to rheumatic heart disease. The association of the clinical manifestations of rheumatic fever and the immune hyperresponsiveness to a streptococcal antigen could be ascribed to a disease-associated immune-response gene which is in linkage disequilibrium with the DR2 and DR4 alleles of HLA-DR locus on chromosome six.

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“…D avies and G ery [16] have studied the possibility of patients' serum reaction on antigenic heart tissue in various types of heart diseases and came to the conclusion, that only a limited number of people had ability to form circulating antibody. They expected also that the hereditary factor was of some importance, as W ilson and Schweitzer [17] have described.…”

Section: Discussionmentioning

confidence: 89%