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Skin biopsy of a 10-year-old boy with a 3-week history of recurrent rash and fever demonstrated histologic changes suggesting a diagnosis of erythema marginatum associated with rheumatic fever. Subsequently, the patient developed classic cardiac and arthritic manifestations. Skin biopsy is recommended for the early diagnosis of rheumatic fever.
We report an 11-year-old boy with acute rheumatic fever who presented with gastric perforation while treated with corticosteroids (CS). He had been treated initially with acetylsalicylic acid for 11 days, CS replaced the treatment with acetylsalicylic acid due to deterioration of carditis. The possible pathogenesis is discussed.
Author reviews relations between streptococcal infections and rheumatic fever, particularly biological characteristics of streptococo, genetic and pathogenic factors. Special emphasis is placed on diagnostic criteria to avoid overdiagnosis. Finally, the basis for a program directed to eradicate the disease: screening of susceptible, individuals selection of rheumatogenic serotypes and vaccine preparation against this antigenic strains are reviewed.
The echocardiographic velocity of circumferential fiber shortening and left atrial dimension were measured serially in two groups of children with acute rheumatic fever: Group I, six patients with valve regurgitation without congestive heart failure, and Group II, seven patients with regurgitation and congestive heart failure. In Group I, the initial velocity of circumferential fiber shortening was increased to 1.90 +/- 0.31 circumferences per second (circ/sec) (mean +/- standard deviation). In group II, it was decreased (1.18+/-0.25). In group I velocity of circumferential fiber shortening subsequently decreased but remained above the normal level; in Group II it increased to exceed the expected normal value. Concurrent changes in left atrial dimension were observed in both groups. The initial left atrial dimension of Group I (2.2 +/- 0.75 cm/m2) was slightly increased and returned to normal (1.70 +/- 0.32) on follow-up study. The left atrial dimension of Group II was greatly increased initially (2.70 +/- 0.81 cm/m2) and remained large (2.50 +/- 0.67). Three patients in Group II experienced rebound during corticosteroid withdrawal. In each the velocity of circumferential fiber shortening decreased, suggesting impaired cardiac contractility. The echocardiogram thus facilitates serial assessment of the severity of carditis in acute rheumatic fever.
Differences in the socioeconomic standards of living remain an important and feasible partial explanation for the difference in incidence of rheumatic fever following GABHS infection in Egyptian compared with American children. However, past and ongoing studies of the epidemiology, diagnosis, and treatment of GABHS infections in Egypt, the United States, and elsewhere suggest other significant factors are relevant. Additional knowledge about the rheumatogenic GABHS serotypes that are most prevalent in all countries is an important area where additional research is needed. Streptococcal research will help not only in improving the quality of primary prevention of rheumatic fever, but also in developing streptococcal vaccines.
Seventy five children with acute rheumatic fever (ARF) were hospitalized on Oahu from 1984 to 1988. The annual incidence rate was 9.5 (all rates are per 100,000 children per year). The first attack and recurrent attack rates were 7.9 and 1.6. Polyarthritis occurred in 84%, chorea in 7%, and carditis in 32%. Mitral insufficiency was the most common valvular lesion (88%). Hawaiians/part Hawaiians and Samoans had the highest incidence rates (relative risk 3 and 56, respectively). Polynesian children were 84 times more likely to develop carditis. Five hundred thirty nine ARF cases were hospitalized on Oahu, 1966 to 1974 and from 1976 to 1988. The annual incidence rate of ARF on Oahu has remained fairly constant at about 12.4. The incidence rates in all ethnic groups have decreased except in Samoan children.
Rheumatic fever resulting from group A beta-haemolytic Streptococcus infection continues to be a prevalent disease and an important cause of morbidity and mortality in developing countries. Molecular mimicry and CD4 T lymphocytes, interleukins and adhesion molecules play a crucial role in the pathogenesis of this disease. Arthritis, followed by carditis and chorea, are the main manifestations of the disease. Evidence of asymptomatic carditis has been increasing; however, abnormality identified by echo-Doppler evaluation is not considered as a criterion for diagnosis of rheumatic carditis. Benzathine penicillin is still the best therapeutic option for the treatment of streptococcal infection and secondary prophylaxis, due to its efficacy and low cost.
OBJECTIVE: To determine whether under-reporting of rheumatic fever occurs at hospital, municipal, provincial and national levels of the South African health system. BACKGROUND: Information on the incidence of rheumatic fever (RF) and the prevalence of rheumatic heart disease (RHD) is required for the prevention of valvular heart disease in developing countries. In South Africa, RF was made a notifiable condition in 1989. It has recently been suggested that the reporting of RF cases may be incomplete, possibly because of underreporting by health care professionals and deficient administration of the disease notification system in South Africa. METHOD AND RESULTS: We assessed whether underreporting of RF cases occurs by comparing the numbers of RF cases reported per year at hospital, municipal, provincial and national levels from 1990 to 2004. There was a fall in the number of RF cases reported per year at national and provincial level over the 15 years of observation. A detailed analysis of the number of RF cases reported at hospital, municipal and provincial level for a 5-year period showed that more cases were diagnosed in one hospital (serving a smaller population) than were captured at municipal and provincial level (serving a larger population), suggesting underreporting by health care professionals. There were discrepancies in the number of cases reported at municipal, provincial and national level, suggesting poor administration of the notification system. CONCLUSION: There appears to be underreporting of RF cases by health care professionals, and poor administration of the RF notification system. Health care professionals need to be educated about the statutory requirement to notify all RF cases in South Africa. An effective national disease notification system is required.
From a retrospective study of hospital records, it is calculated that the present incidence of rheumatic fever in Scottish children is 0.6 per 100 000 per year. This is in keeping with recent research from other developed communities. An attempt is made to assess the relative risk of developing rheumatic fever after antibiotic-treated streptococcal sore throats and non-antibiotic-treated streptococcal sore throats. The risk in both cases is low (probably in the order of 1:30 000) and there is no evidence that prescribing antibiotics for prodromal sore throats confers benefit.
We describe five children in whom symptoms and signs fully consistent with a diagnosis of acute rheumatic fever arose in association with infection by Mycoplasma pneumoniae. With the decline of streptococcal disease as a causative agent of acute rheumatic fever, other etiologic possibilities capable of reproducing the clinical syndrome - especially Mycoplasma pneumoniae - should be considered, as this may have therapeutic and prognostic implications.
OBJECTIVE: Our objective was to confirm an observed temporal association of increased numbers of rheumatic fever cases concomitant with the appearance of an increased prevalence of mucoid strains of Streptococcus pyogenes. During the resurgence of rheumatic fever (RF) that has occurred in the Intermountain area surrounding Salt Lake City, Utah, since 1985, the largest number of cases occurred in 1985 and 1986 and 12 years later in 1997 and 1998. During the initial outbreak and the later exacerbation of the resurgence, an increased number of mucoid strains of S pyogenes were present in the community. METHODS: The referred cases of RF that fulfilled Jones criteria have been systematically reviewed by the medical staff at Primary Children's Medical Center yearly since 1985. Before the resurgence of RF, a program was initiated by the microbiology laboratory at Primary Children's Medical Center to store frozen isolates of S pyogenes. All frozen specimens were randomly selected and entered into a log; the coded entry allowed for comments regarding the origin of the isolate and whether the isolate had a mucoid appearance on the blood agar culture plate. This log was reviewed in October 2002 to determine whether the percentage of frozen mucoid isolates stored during the resurgence of RF would support the clinical and epidemiologic suspicion that mucoid isolates seemed to be present with a higher frequency during the 2 periods of high incidence of RF. The percentage of mucoid isolates was compared with the yearly number of cases of RF. A Pearson r correlation analysis was completed to determine whether there was a significant association between the percentage of mucoid isolates and the number of cases of RF. RESULTS: The highest number of cases of RF was temporally associated with the highest percentage of mucoid isolates. There was statistically significant correlation between percentage of mucoid strains and the number of RF cases. CONCLUSIONS: The Utah experience with the resurgence of RF in a civilian population during the last decade and a half of the 20th century confirmed the temporal association of mucoid strains of S pyogenes, primarily M-type 18, with a high incidence of RF.
One hundred and twenty-six children with the initial attack of acute rheumatic fever were followed up prospectively for 6 years. Sixty-six children maintained regular secondary prophylaxis (regular group) and 60 were irregular (irregular group). Two recurrences developed in the regular group with a recurrence rate of 0.005/patient/year follow-up, and 71 recurrences developed in the irregular group with a recurrence rate of 0.2/patient/year follow-up. These findings demonstrate the effect of secondary prophylaxis in reducing the frequency of recurrences. The prevalence rate of rheumatic heart disease in children who had carditis in the initial attack was 42% in the regular group vs 70% in the irregular group (p less than 0.05). These findings demonstrate the deleterious effect of recurrences in the evolution of rheumatic heart disease. The prevalence rate of rheumatic heart disease in children who maintained regular secondary prophylaxis, was 42% in those children who had carditis in the initial attack and 6% in those who had no carditis (p less than 0.05). These findings demonstrate the prognostic significance of presence or absence of carditis during the initial attack, in the subsequent evolution of rheumatic heart disease. The prevalence rate of rheumatic heart disease in the 66 children who maintained regular prophylaxis was 23%. Comparison of these data with those of similarly designed studies shows that the evolution of rheumatic heart disease following the initial attack of acute rheumatic fever, seems to behave similarly in the tropics and subtropics as it did in temperate climates.
A prospective clinical study is reported of the initial attack of acute rheumatic fever in 210 children seen in Kuwait over a period of four years. The main presenting feature was pain in the joints, caused by arthritis in 79% of our patients and by arthralgia in 15%. Five per cent presented with chorea alone and 1% with congestive heart failure alone. Carditis was detected in 46.2%, cardiomegaly in 10%, pericarditis in 1.4% and congestive heart failure in 4.8%. The incidence of chorea was 7.6%, of erythema marginatum 1% and of subcutaneous nodules 0.5%. The mortality rate was 0.5%. Data from this study reflect the mild nature of acute rheumatic fever in Kuwait, in contrast to the aggressive nature of the disease described from neighbouring and developing countries.
In summary, rheumatic fever seems to be on the rise in the United States. It remains to be seen whether this case is an example of a resurgence of ARF in adults in Virginia, but it probably does, since there are a number of cases being seen in the pediatric population.
An eleven-year study of the incidence and consequences of acute rheumatic fever was carried out in a country in which a uniform climate together with national characteristics of insularity, wealth, and unrestricted access to free medical care contribute prominently to the epidemiologic milieu. Study subjects were 86 children, aged four to fourteen years, satisfying criteria for acute rheumatic fever. Study methods included clinical evaluation, standard laboratory studies, and echocardiography. A declining incidence of rheumatic episodes, ranging from 1.06 to 18.6/100,000 population (average 11.2/100,000), was identified. The course of the episode was generally mild. Arthritic findings predominated (92%), followed by carditis identified clinically in 43% and, with the addition of echocardiography, in 71%. Residual valvular regurgitation, as a longer term consequence, persisted in 46% of those with auscultatory confirmation of valvulitis. No recurrences were identified. Comparison with countries of similar socioeconomic status revealed relatively unimportant differences. Comparison with nearby disadvantaged countries identified striking contrast. It may be concluded that among the contributing factors, for the improvement in the incidence and sequelae of a rheumatic episode, are an advantaged socioeconomic environment and accessibility to unlimited medical care.
From 1974 to 1987 we have diagnosed thirty three patients with rheumatic fever. The age of onset ranged from three to sixteen years with a mean age of nine years and six months. Carditis was the most frequent major criteria (27/33) followed by arthritis (14/33) and Sydenham's chorea (6/33). All the patients with carditis had the mitral valve affected. The usual treatment in the acute phase was penicillin and aspirin. The prophylaxis recommended to the patients was penicillin G benzatine every 21 days if they had carditis and every 28 days if they didn't. Two patients allergic to penicillin are receiving erythromycin. We have observed that the incidence of rheumatic fever has remained unchanged that the age of onset increased and that the antiinflammatory drug of choice in most patients with carditis is aspirin.
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.
BACKGROUND: Patients with acute rheumatic fever (ARF), who were admitted to Pediatric Cardiology Unit of Selçuk University Faculty of Medicine from July 1993 to 1998, were studied retrospectively to verify the clinical profile of the disease and to compare the results with those from other countries. METHODS: All patients were examined by one of the two pediatric cardiologists in our study group. Every patient had a chest X-ray, electrocardiogram and an echocardiographic investigation. Throat culture, antistreptolysin O test, C-reactive protein and sedimentation rates were investigated for each patient. RESULTS: During the study period, 274 cases with ARF were identified among patients admitted to the present institution. There were 8032 visits during the study period, giving an occurrence rate of 3.4%. Arthritis was the most common major manifestation (81.4%). It was followed by carditis (60.9%) and chorea (17.9%). Subcutaneous nodules (0.7%) and erythema marginatum (0.4%) were both seen in patients with carditis. The mitral valve was the most commonly affected valve (95.8%), followed by the aortic valve (40.1%). Two patients died and regurgitation disappeared in 21% of patients with mitral regurgitation. Fifteen patients (14%) with isolated arthritis and pure chorea had mitral regurgitation demonstrated by echocardiographic investigation but without any significant murmur. CONCLUSION: The present study indicates that ARF is still a significant problem in Konya and that recurrences can be prevented by administering a 3-week benzathine penicillin G regimen.