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PubMed1981

The community control of rheumatic fever and rheumatic heart disease: report of a WHO international cooperative project.

The feasibility and effectiveness of a programme for the community control of rheumatic fever and rheumatic heart disease were studied in a cooperative multicentre project initiated and coordinated by the World Health Organization. The programme was carried out in seven centres in various developing countries of Africa, America, and Asia according to a common protocol, and is under way in a further eight countries in Latin America. Pilot community programmes were shown to be practicable and effective in reducing the burden of rheumatic heart disease in developing countries and their extension to cover entire populations should be encouraged.

Adolescent↗
PubMed2001

Rheumatic fever.

Rheumatic fever is a multisystem inflammatory disease that occurs as a delayed sequel to group A streptococcal pharyngitis. It is less common than it was 50 years ago but is still a major cause of heart disease in developing areas of the world. The relationship between the site of infection, the type of causative organism, and susceptibility of the host is essential in the development of the disease. Its major clinical manifestations include carditis, migratory polyarthritis, chorea, erythema marginatum, and subcutaneous nodules. It can manifest as an acute febrile illness consisting of migratory polyarthritis involving the large joints, as carditis and valvulitis, or as Sydenham's chorea with involvement of the central nervous system. The disorder in its milder form resolves itself without sequelae. Carditis is the condition most associated with increased mortality and morbidity and may be fatal in its severe forms. Penicillin is the most appropriate primary and secondary prophylaxis. Anti- inflammatory agents provide symptomatic relief but do not prevent rheumatic heart disease.

Adolescent↗
PubMed1997

Chorea in fifty consecutive patients with rheumatic fever.

Rheumatic fever (RF) remains a significant public health problem in developing countries. Although its incidence has been declining in developed areas, several outbreaks of RF have been identified in the United States since 1985. To study the prevalence and clinical features of Sydenham's chorea (SC), a well-recognized expression of RF, we examined 50 consecutive patients with RF seen at our Pediatric Cardiology Clinic. Chorea was rated on a scale of 0-4 (0 = no chorea; and 4 = severe chorea, patient unable to walk). Chorea, active or in remission, occurred in 13 patients (26%). The male-to-female ratio was 1.08 among the total population and 0.86 among the SC group. The mean age of the 50 patients at onset of RF was 8.4 years and the standard deviation (SD) 3.1 years. Carditis was diagnosed in 84% of patients with and without chorea, but arthritis was more common in the latter group (83.8%) than in the SC group (30.8%). The mean age at onset of SC was 9.2 years (SD 2.7). Involuntary movement, noticed by nine patients, was the most common initial symptom. Chorea went into remission in eight patients after 9.9 months (SD 13.4). Chorea was rated 3 in ten patients, 1 in two patients, and 4 in one patient. Among the five patients with active SC, three had hemichorea and two had generalized chorea. We recognized abnormal saccades in 80% of patients with active chorea, whereas just 13.5% of patients without chorea displayed this abnormality. SC remains an important cause of chorea in countries plagued by RF.

Age Distribution↗
PubMed1983

Immunopathology of acute rheumatic fever and rheumatic heart disease. The demonstration of Coxsackie group B viral antigen in the myocardium.

Immunopathologic studies were performed on heart tissue of patients with acute rheumatic fever with carditis and chronic rheumatic heart disease. Coxsackie group B viral antigen was demonstrated in 3 heart specimens of patients clinically compatible with active rheumatic fever. In two of these the pathologic findings were compatible with acute rheumatic carditis. Immunoglobulin was detected in 2 and complement in one of these heart specimens. All of the chronic rheumatic heart specimens and the controls were negative. These findings suggested that Coxsackie group B virus may be etiologically related to the pathogenesis of acute rheumatic fever.

Acute Disease↗
PubMed1979

Epidemiologic aspects of rheumatic fever and rheumatic heart disease in Israel.

Rheuamtic fever and rheumatic heart disease are still important public health problems in Israel. Rheumatic fever is a seasonal disease, more common during the cold months of the year. A high incidence of rheumatic fever followed the immigration waves of the 1950s, in contrast with a decline in new cases over the last 10 years among both Jews and non-Jews. In the same period (1950s to 1975), a decline in the numbers of hospitalized patients was also reported. After 1970 mortality rates of rheumatic fever dropped to zero. The prevalence of rheumatic heart disease has declined in children, as shown in school studies, but in adults, a different trend is seen. The number of hospitalized patients has increased, in parallel with the increasing population, to more than 3,000 hospitalized patients per year. While a plateau curve is seen in the mortality rates of males of all ethnic origins studied, among females there is a slight decline in mortality. The discrepancy between the decline in occurrence of rheumatic fever and rheumatic heart disease in children and the increase in prevalence of rheumatic heart disease in adults is explained by the massive immigration from countries with a high prevalence of rheumatic heart disease.

Adolescent↗
PubMed1975

Antimyocardial antibodies in heart diseases. II. Immunofluorescent patterns in rheumatic fever and rheumatic heart disease.

Antibodies against rat myocardium were studied by means of histoimmunfluorescence (indirect technique) in 32 rheumatic fever patients. Antibodies were found in 54% of the cases without carditis or active rheumatic valvulitis and in 83% of those with carditis. The fluorescent patterns observed were : sarcolemmal, sarcoplasmic (intermyofibrillar) and smooth muscle of vessels. The sarcolemmal pattern was more frequently observed in rheumatic carditis while the intermyofibrillar one was more common in rheumatic fever without evidence of carditis as well as rheumatic valvulitis. The serum factors involved in the sarcolemmal fluorescence were inhibitied by the insoluble saline myocardium extract.

Adult↗
PubMed1978

An epidemiological study of rheumatic fever and rheumatic heart disease in Lagos.

In Lagos 12 755 schoolchildren aged between six and 12 years were screened for evidence of rheumatic heart disease and showed a prevalence rate of 0.03%. Group C (27.7%) and group G (47.3%) predominated in the throat and in cases of pharyngitis, while group A predominated on the skin. Two hundred and sixty-six cases of pharyngitis were recorded, 70 (26.4%) were positive for beta-haemolytic streptococci. Half of the cases of streptococci while 28.2% and 21.1% were caused by groups C and G respectively. A diversity of serotypes of group A streptococci, which included types 49 and 55, and C and G streptococci were isolated from impetiginous skin lesions. The main point of interest is the association of group C and G streptococci with sore throat and skin infections. The pathogenicity of C and G in such circumstances merits further investigation. The present study also showed that prophylactic benzathine penicillin (Penadur) given to patients with rheumatic heart disease or rheumatic fever had been effective with no recurrent attack of acute rheumatic fever.

Child↗
PubMed1993

Analysis of costs of acute rheumatic fever and rheumatic heart disease in Auckland.

AIM: This analysis aims to identify the direct costs of rheumatic fever and its sequelae to the Auckland Area Health Board and to describe the indirect and intangible costs to patients. METHODS: The annual cost was estimated using primarily 1987 data costed in 1991 dollars. The cost analysis was undertaken in five sections: (1) acute rheumatic fever admissions; (2) surgical admissions; (3) rheumatic heart disease related admissions (nonsurgical); (4) outpatient clinic appointments; and (5) secondary prophylaxis programme. Non hospital board direct costs and indirect and intangible costs are described. Ethnic distribution and subsequent economic burden were analysed for each section. RESULTS: The total cost to the health board was estimated to be $3.60M. The management of chronic rheumatic heart disease accounts for 71% of the cost. Rheumatic heart disease related nonsurgical admissions cost $1,228,495 (34%), surgical admissions $846,235 (23%) and outpatient clinic appointments $490,060 (14%) respectively. Both Maori (30% of costs, $1.1M) and Pacific Island people (36% of costs, $1.3M) are disproportionately affected by this largely preventable disease. Of the total cost 13% is spent on coordinated secondary prevention programmes. CONCLUSION: An energetic secondary prevention programme over 10 years to prevent recurrences and the development of carditis has only partially reduced the rate of rheumatic heart disease. A targeted primary prevention pilot programme should be actively considered.

Acute Disease↗
PubMed1984

Rheumatic fever and rheumatic heart disease in the Hamilton health district: I. An epidemiological survey.

The establishment of a computer based rheumatic fever register in the Hamilton health district is reported. The mean annual incidence of rheumatic fever over a five period in Maoris and non-Maoris 5-29 years of age was 88.0 and 9.3 per 100 000 respectively, with almost twice the recurrence rate in Maoris. The prevalence of rheumatic heart disease in Maoris and non-Maoris, 5-19 years of age, was 6.5 and 0.9 per 1000 respectively, Maoris being more likely to have severe or multiple valve lesions. Over a 10 year study of under 30 year olds, all 11 cardiac deaths occurred in Maoris. Twenty-seven out of 31 valve surgery cases were Maoris.

Adolescent↗
PubMedDate not supplied

Immunologic and immunogenetic studies in rheumatic fever and rheumatic heart disease.

In order to evaluate all the important limbs of the immune system in the same patient population with rheumatic fever (RF) and rheumatic heart disease (RHD) cellular and humoral immune parameters as well as the immunogenetic profile in 265 North Indian patients with RHD were evaluated. They were studied for class in HLA antigens and 165 of them were also evaluated for the class II (DR locus) antigen profile. Data obtained was compared with 400 and 134 healthy controls respectively of the same ethnicity. Humoral immune parameters (Serum immunoglobulins IgG, IgA; Serum complement fractions C3, C4, C3d; circulating immune complexes and B lymphocyte numbers) and cellular immune parameters (total leucocyte and lymphocyte counts; T lymphocyte sub-populations--CD4, CD8 counts; lymphocyte migration inhibition to an extracellular streptococcal antigen, streptolysin 'O') were studied in 23 patients with RF, 21 patients with "inactive" RHD and 20 normal controls. Patients of RHD were noted to have an increased frequency of DR3 (P less than 0.001; Relative risk = 2.3) and a decreased frequency of DR2 (P less than 0.001; Relative risk = 0.3) as compared to the controls. Patients of RF had evidence of an altered regulatory T cell function (increased CD4/CD8 ratio) and decreased cell mediated immunity to streptolysin 'O'. An increased humoral immune response (increased B cell counts, elevated serum IgG, circulating immune complexes and C3d) was noted in patients of RF as well as "inactive" RHD. An integrated pathogenetic model with immune response associated antigens of the DR locus influencing selection of cardiac cross-reactive antigens by the antigen processing macrophages, an altered regulatory T cell function with decreased suppressor T cell activity leading to an abnormal immune response is proposed to explain the pathogenesis of RF.

HLA Antigens↗
PubMedDate not supplied

Rheumatic fever and rheumatic heart disease: clinical profile of 550 cases in India.

BACKGROUND: The aims and objectives of this paper were to analyze the clinical presentation of children with rheumatic fever (RF) and rheumatic heart disease (RHD), to determine the cardiac sequel of RF and valvular affection due to RF, and to study clinical manifestations in recurrences of RF. METHODS: Our study, a retrospective analysis, was conducted at a tertiary-care teaching hospital with specialty follow-up clinic for patients with RF/RHD. This retrospective study was conducted to include a period of 31 years (from January 1971 through December 2001). Pre-completed case protocols of patients with RF/RHD were analyzed to record the following information: demographic data; clinical features on admission/presentation; relevant investigations; recurrences; cardiac valvular affection, and outcome. Modified Jones' criteria were used for diagnosis of RF. RESULTS: The study included 550 patients. Mean age of presentation was 9.62 years and sex ratio was 1.15:1 favoring males. A total of 250 patients presented with initial/first attack of RF. Arthritis and carditis were seen in 169 (67.6%) and 105 cases (42%), respectively. Chorea was seen in 47 cases (18.8%) and erythema marginatum, in four. A total of 224 patients presented with recurrence of RF (with pre-existing RHD). Arthritis and carditis were seen in 109 (48.66%) and 178 cases (79.46%), respectively, in these patients; 76 cases presented with chronic RHD. Mitral regurgitation was the most common cardiac valvular lesion observed (150 cases) followed by a combination of mitral stenosis and mitral regurgitation (98 cases). Congestive cardiac failure was seen in 201 cases (36.54%) and infective endocarditis in 30 (5.45%). 2-D echocardiography and/or color Doppler was performed in 201 patients (36.54%). Average duration of follow-up was 3.19 years; 74.72% of cases were lost to follow-up. Benzathine penicillin prophylaxis was regular in 42.18% cases. Positive family history of RF/RHD was present in 2% of cases. Twenty three patients (4.18%) died. CONCLUSIONS: Arthritis was the most common manifestation in the initial attack of RF, while carditis was the most common manifestation in patients presenting with rheumatic recurrences (with pre-existing RHD) and mitral regurgitation was the most common valvular lesion. Rate of patient dropout from penicillin prophylaxis was high. Clinical manifestations in initial/first attack of RF have not significantly changed in the last 31 years.

Adolescent↗
PubMed2005

Molecular pathogenesis of rheumatic fever and rheumatic heart disease.

Molecular mimicry between streptococcal and human proteins has been proposed as the triggering factor leading to autoimmunity in rheumatic fever (RF) and rheumatic heart disease (RHD). This article summarises studies on genetic susceptibility markers involved in the development of RF/RHD. It also focuses on the molecular mimicry in RHD mediated by the responses of B and T cells of peripheral blood, and T cells infiltrating heart lesions, against streptococcal antigens and human tissue proteins. The molecular basis of T-cell recognition is assessed through the definition of heart-crossreactive antigens. The production of cytokines from peripheral and heart-infiltrating mononuclear cells suggests that T helper 1 (Th1)-type cytokines are the mediators of RHD heart lesions. An insufficiency of interleukin 4 (IL-4)-producing cells in the valvular tissue might contribute to the maintenance and progression of valve lesions.

Animals↗
PubMed1996

Type-specific antibodies to purified streptococcal M proteins from potentially rheumatogenic M-types in patients with rheumatic fever and rheumatic heart disease.

This study was designed to identify the predominant serotypes of group A streptococci (GAS) responsible for rheumatic fever (RF) and rheumatic heart disease (RHD) in India. RF and RHD sera were screened for antibodies to M proteins retrospectively against known rheumatogenic M types and M types isolated from patients with acute RF. All GAS strains isolated from four patients with acute RF in a short outbreak of RF, were identical - serum opacity factor (SOF) negative, T-pattern 3/13 B3264 and M-non-typable. Because of this, M protein was isolated from only one of these four M-non-typable strain (S-399). This was done by limited pepsin digestion and purification by ion-exchange chromatography on DEAE-Sephadex, followed by gel filtration. Purified M protein was found to be homologous on SDS-PAGE (mol.wt 20 kDa), immunogenic in rabbits and to retain its antigenic structure. This purified M protein from an M-non-typable strain (pep-MNT) was used as an antigen to screen RF and RHD sera retrospectively by ELISA for the prevalence of the M-non-typable GAS strain. The prevalence of the M-non-typable strain was compared with that of the known rheumatogenic M types. Results suggest that the M-non-typable strain could be a provisional new rheumatogenic M type in India and could be a candidate for a multivalent M-protein vaccine to control RF and RHD.

Adolescent↗
PubMed2005

Prevalence of rheumatic fever and rheumatic heart disease in rural Bangladesh.

A community-based study was done on 5923 rural Bangladeshi children aged 5-15 years to determine the prevalence of rheumatic fever (RF) and rheumatic heart disease (RHD). The prevalence was found to be 1.2 (95% confidence interval 0.3-2.1) per 1000 for RF defined by revised Jones criteria and 1.3 (0.4-2.2) per 1000 for Doppler echocardiography-confirmed RHD.

Adolescent↗
PubMed2006

Advances in potential M-protein peptide-based vaccines for preventing rheumatic fever and rheumatic heart disease.

Rheumatic fever (RF) and rheumatic heart disease (RHD) are postinfectious complications of an infection (or repeated infection) with the Gram-positive bacterium, Streptococcus pyogenes (also known as group A streptococcus, GAS). RF and RHD are global problems and affect many indigenous populations of developed countries and many developing countries. However, RF and RHD are only part of a larger spectrum of diseases caused by this organism. The development of a vaccine against GAS has primarily targeted the abundant cell-surface protein called the M-protein. This review focuses on different M-protein-based-subunit vaccine approaches and the different delivery technologies used to administer these vaccine candidates in preclinical studies.

Amino Acid Sequence↗
PubMed1979

The natural history of rheumatic fever and rheumatic heart disease in the Orient.

Studies published in the past 10 years suggest that group A streptococcal infections are frequent in the Orient and lead to a high incidence of rheumatic fever (RF) and rheumatic heart disease (RHD). In the present study, streptococcal infections were found to be more prevalent in Japan and Taiwan, whereas RF and RHD were more common and severe in the Philippines, Thailand, and Indonesia, particularly among the socioeconomically less privileged populations. The pattern of childhood RF varied: Carditis was the most common manifestation, occurring in 57% to 94% of the patients; polyarthritis was generally atypical and less common in the tropics; chorea minor and erythema marginatum were much more common in Japan, less common in Taiwan and rare in the tropics. RF recurrences were quite common and led to the development of new carditis, and deterioration or persistence of the pre-existing heart disease. The 5 year mortality rates differed greatly, ranging from zero to 42%. There was disappearance of the heart murmur in 16.5% to 37.5% of patients. Such apparent recovery was related to adherence to chemoprophylaxis. The major risk factors adversely affecting survival were the severity of carditis, inadequacy of medical service, non-compliance to chemoprophylaxis, RF recurrence, poor socioeconomic status, and high prevalence of group A streptococci. It is concluded that there is no uniform "Oriental-type" of natural history of RF and RHD. The natural history varies greatly among countries as is true in other parts of the world.

Adolescent↗
PubMed1997

[HLA-DQA1 genes involved in genetic susceptibility to rheumatic fever and rheumatic heart disease in southern Hans].

The incidence of rheumatic fever (RF) or rheumatic heart disease (RHD) is high in southern China. We studied the genetic susceptibility of HLA-DQA1 alleles to RF or RHD with emphasis on the mechanisms 106 unrelated healthy individuals and 54 patients with RF or RHD of Chinese Han nationality in Guangdong were included. DNA extraction from various biological material using phenol/chloroform method and HLA-DQA1 genotyping by PCR-PAGE and then with silver dyeing was used to show the electrophoretic patterns. A total 6 alleles of HLA-DQA1 were found. Increased allele frequencies of DQA1*0101 (31.48%, RR = 2.89, P < 0.005, EF = 0.206) and decreased allele frequencies of DQA1*0102 (1.85%, RR = 0.106, P < 0.005, PF = 0.134) were observed. Two increased genotyping of HLA-DQA1 (DQA1*0101/0301, chi 2 = 8.84, P < 0.005 and DQA1*0101/0401, chi 2 = 6.23, P < 0.0025) and decreased genotyping of DQA1*0102/0301 (chi 2 = 11.98, P < 0.005) were also observed. These findings suggested that DQA1*0101 contribute to genetic susceptibility for RF or RHD in Guangdong hans while DQA1*0102 to its genetic resistance. Digesting the genotyping of HLA-DQA1 may provide scientific basis for finding susceptible individuals to RF or RHD. Using PCR-PAGE and silver dyeing technique, a new genotyping method for HLA-DQA1, which is simple sensitive and precise, was established and applied.

Adolescent↗
PubMed1981

Rheumatic fever and rheumatic heart disease in rural South Indian children.

A preliminary pilot study of streptococcal infection and sequelae was initiated in 23 villages in 1973 using trained field workers for case detection. Part of the pilot programme, in 1974-75, involved 374 rural and 664 urban schoolchildren, and revealed that 14.9% of them had streptococcal pyoderma while pharyngitis was seen in 4.2%. The pilot study also revealed a high prevalence of rheumatic fever (RF) and rheumatic heart disease (RHD) in both rural (5.4/1000) and urban (6.0/1000) pupils. A long-term surveillance study was then undertaken in 3890 pupils attending 15 rural schools, from July 1975 to December 1978, when the prevalence of RF was found to be 0.5/1000 and of RHD, 4.4/1000, giving an overall rate of 4.9/1000. The incidence rate was found to be 1.7/1000 per year in 1976, 1.6/1000 per year in 1977, and 0 in 1978. The rate of compliance with primary prophylaxis was 90-100%, and with secondary prophylaxis was 92-93% of the 27 cases.

Adolescent↗