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Rheumatic fever, rheumatic heart disease, and the streptococcal connection: the role of streptococcal antigens cross-reactive with heart tissue.

The role of streptococcal infections in initiating the diverse clinical and pathological manifestations of rheumatic fever and rheumatic heart disease is considered in relation to the multiple cross-reactive relations of group A Streptococcus and tissue antigens. Autoantibodies to the following shared antigens have been demonstrated in sera of patients wit rheumatic fever: (1) cardiac, skeletal, and smooth muscle; (2) heart valve fibroblasts; (3) neurons in basal ganglia; and (4) a group A carbohydrate-related determinant in connective tissues. Circulating autoantibodies to these different antigens were present in higher titer or occurred more frequently in patients with rheumatic fever than in those with uncomplicated streptococcal infections. A direct correlation of the presence of these autoantibodies with carditis could not be established. The pathogenetic mechanisms that link streptococcal infection to rheumatic fever and rheumatic heart disease are not yet clear. Among the possibilities to be considered within the above frame of reference are combined cell-mediated and humoral autoimmune mechanisms directed to one or more cross-reactive antigens in the tissues, selective binding of streptococci to tissues, role of circulating immune complexes, and linkage with histocompatibility antigens.

Antibodies, Bacterial↗

HLA phenotypes in patients with rheumatic fever, rheumatic heart disease, and Yersinia arthritis.

HLA phenotypes were determined in 109 patients with rheumatic fever (RF), 48 patients with Yersinia arthritis (YA), 86 patients with chronic rheumatic heart disease (RHD), and 326 controls. There was an increased frequency of Bw35 in RF as compared to controls (Pc less than 0.01), while B18 was more common in patients with acute carditis than in those without (P less than 0.02). HLA frequencies in RHD did not differ significantly from those in controls. A significant correlation between B27 and YA was observed (Pc less than 0.001). Carditis or iritis occurred in 10 of 31 B27 positive YA patients but in none of 17 B27 negative patients. Eleven of 31 B27 carriers had signs of urological inflammation vs one of 17 B27 negative patients. In the B27 positive YA group, there were three men with previous ankylosing spondylitis and one with Reiter's syndrome (RS). Also, four patients developed RS during Yersinia infection. This simultaneous occurrence of three B27 positive rheumatic diseases suggests that a patient with one "B27 positive rheumatic disease" is more susceptible to other diseases or symptoms known to be associated with the B27 antigen.

Adult↗

Allergic reactions to long-term benzathine penicillin prophylaxis for rheumatic fever. International Rheumatic Fever Study Group.

1790 patients from 11 countries were enrolled in a prospective international study to determine the incidence of allergic reactions to monthly intramuscular benzathine penicillin (penicillin G benzathine) injections to prevent recurrences of rheumatic fever. After 32,430 injections during 2736 patient years of observation, 57 of the 1790 patients (3.2%) had an allergic reaction. 4 had anaphylaxis, an incidence of 0.2% (1.2/10,000 injections), all in patients over 12 years of age, and 1 patient died, a fatality incidence of 0.05% (0.31/10,000 injections). These rates are similar to those described for patients without rheumatic fever who receive short-term treatment with parenteral penicillin. Rheumatic fever recurred in 8 of 1790 patients (0.45%) who received benzathine penicillin prophylaxis compared with 11 of 96 (11.5%) who did not comply with treatment. Life-threatening allergic reactions are rare in patients on long-term parenteral benzathine penicillin to prevent recurrences of rheumatic fever; the long-term benefits of such prophylaxis by far outweigh the risk of a serious allergic reaction.

Adolescent↗

An epidemiological survey of rheumatic fever and rheumatic heart disease in Sahafa Town, Sudan.

STUDY OBJECTIVE: The aim was to determine the prevalence of rheumatic fever and rheumatic heart disease and to initiate a programme of secondary prophylaxis in Sahafa Town, Sudan. DESIGN: The study was a prospective case finding survey, carried out by a specially trained team headed by a cardiologist. SETTING: The study involved high risk school children (5-15 years of age) from Sahafa Town in the period 1986-1989. SUBJECTS: A total of 13,332 children on the school registers (7892 boys and 5430 girls) were examined generally and specifically for evidence of rheumatic fever or rheumatic heart disease. MAIN RESULTS: Out of the 13,322 children screened 351 were suspected cases and 146 were confirmed cases of rheumatic fever or rheumatic heart disease. The prevalence rates for all ages were 10/1000 for boys and 14/1000 for girls. The overall prevalence rate of the whole programme area was 11/1000, prevalence of rheumatic fever was 8/1000, and prevalence of rheumatic heart disease was 3/1000. The prevalence rate was significantly increased among the inner town inhabitants (15/1000) compared to the outer town inhabitants 4/1000 (p < 0.001). Monthly prophylactic benzathine penicillin in a dose of 1,200,000 IU was given to both suspected and confirmed cases. Penicillin coverage rate was 72%. CONCLUSIONS: Rheumatic fever continues to be a serious health problem. With economic pressures causing impending change in socioeconomic conditions in most Third World countries in the immediate future, rheumatic fever will continue to have a high prevalence rate and rheumatic fever and rheumatic heart disease prevention programmes will remain a central goal.

Adolescent↗

Rheumatic fever and rheumatic heart disease.

Rheumatic heart disease poses a major challenge to public health and is the most prevalent heart disease in children. The major determinants of rheumatic fever and rheumatic heart disease are poverty, malnutrition, overcrowding, poor housing, and a shortage of health-care resources. Although cost-effective strategies for the prevention and control of these diseases are available, they remain underutilized in most developing countries. A World Health Organization Expert Consultation reviewed the current scientific knowledge of rheumatic fever and rheumatic heart disease, as well as medical and public-health practices, and revised the Jones diagnostic criteria for rheumatic fever and rheumatic heart disease. This revision facilitates the diagnosis of: primary episodes of rheumatic fever, recurrent attacks of rheumatic fever in patients with or without rheumatic heart disease, rheumatic chorea, insidious onset rheumatic carditis and chronic rheumatic heart disease. The present report provides practical recommendations for an evidence-based approach to the prevention and management of rheumatic fever and rheumatic heart disease. It also provides practical guidance for implementing cost-effective programmes for controlling these diseases. The report will be of interest to clinicians, policy-makers and public-health professionals.

Adolescent↗

Acute rheumatic fever and rheumatic heart disease in the top end of Australia's Northern Territory.

OBJECTIVE: To describe the epidemiological and clinical features of acute rheumatic fever and rheumatic heart disease in the Top End of the Northern Territory. METHODS: A retrospective review (in some instances as far back as the 1960s) of all cases of known or suspected acute rheumatic fever or rheumatic heart diseases, with prospective validation of clinical status where necessary. Cases were ascertained from hospital and community medical clinic records and medical staff; and from records and health staff of 10 rural communities. RESULTS: Three hundred and eighty-six revised Jones criteria-confirmed episodes of acute rheumatic fever were documented in 249 individuals (99% Aboriginal). The annual incidence of confirmed acute rheumatic fever between 1989 and 1993 was 254 per 100,000 Aboriginal people aged 5 to 14 years. A more accurate estimate of 651 per 100,000 came from 10 rural communities with more complete information. As of 1995, there were 286 people living with established rheumatic heart disease (95% Aboriginal). The point prevalence of rheumatic heart disease among Aboriginal people was 9.6 per 1000, with a rate of 24 per 1000 in one large rural community. Sydenham's chorea was common, and associated with later rheumatic heart disease in 49% of cases. There was a preponderance of females with acute rheumatic fever, rheumatic heart disease and chorea. CONCLUSIONS: In Aboriginal people in rural northern Australia the incidence of acute rheumatic fever is higher than that reported anywhere in the world, and the prevalence of rheumatic heart disease is among the highest in the world. While continuing attention must be paid to alleviating the causes of these diseases of poverty, immediate action is needed to improve diagnosis of acute rheumatic fever, adherence to secondary benzathine penicillin prophylaxis regimens, and follow-up of rheumatic heart disease cases.

Chorea↗