The first attack of acute rheumatic fever in childhood--clinical and laboratory profile

One hundred consecutive cases of 'first attack' of acute rheumatic fever were studied. There were 52 males and 48 females, constituting 1.12% of total hospital admissions. Nearly 10% of children were below the age of 5 years, stressing the early onset of rheumatic fever in tropics. Only 47% gave a definite history of overcrowding at home. Sore throat was present in 67%, overt arthritis in 66%, carditis in 57%, arthralgia alone in 22% of which 45.45% had carditis. Small joint involvement was noticed in 23% of cases of which 73.91% had carditis. Only 33.33% had congestive cardiac failure. Ten per cent of children had chorea, while subcutaneous nodules were seen in 4% of cases, all of whom had associated carditis. Erythrocyte sedimentation rate (ESR) showed good correlation with clinical profile. Throat cultures were positive for beta hemolytic streptococci only in 12% of cases. Anti-streptolysin 'O' (ASO) titre showed significant titres on 68% of cases, anti-deoxyribonuclease "B" (ADN-B) in 69.32%, antibody to group A carbohydrate (ACHO) in 70.65%. ASO, ADN-B, and ACHO titres together gave 87.5% positivity while estimations in paired sera showed ASO 79.54%, ADN-B 82.27% and ASO, ADN-B together 99.92% significant titres. Study of blood groups showed A group children to be more vulnerable to rheumatic fever (37.5%) and rheumatic carditis (47.37%). Mortality in the present study was nil.     

Acute rheumatic fever in western Pennsylvania and the tristate area

Acute rheumatic fever is reported to have declined and perhaps be vanishing. Prompted by the occurrence of 17 cases of acute rheumatic fever in an 18-month period in 1985 and 1986, we reviewed the records of 243 children with acute rheumatic fever who were cared for at Children's Hospital of Pittsburgh or Mercy Hospital between 1965 and 1986. Acute rheumatic fever was diagnosed using the modified Jones criteria and cases were classified by major criteria as arthritis, arthritis and carditis, carditis alone, carditis and chorea, chorea alone, and arthritis and chorea. Among the 17 recent patients, 59% had carditis, 30% had chorea, and 24% had arthritis alone. The proportion of children who had particular major manifestations was similar in the last two decades and in 1985 to 1986. The recent children with acute rheumatic fever ranged in age from 6 to 13 years with a mean and median age of 10 years. There were 16 white children and one Asian child. Only four children lived in an urban setting. When demographic features of the children were contrasted with those in the previous two decades, a decrease in the proportion of children who lived in urban areas and who were black was noted. Four children had a history of preceding sore throat but only three sought medical care; nine children had no memorable illness and four had either a nonrespiratory illness or a respiratory infection without sore throat. This resurgence of rheumatic fever serves as a reminder that a diligent approach to the diagnosis and therapy of streptococcal infections remains essential.     

Acute rheumatic fever in New York City (1969 to 1988): a comparative study of two decades

One hundred fifteen attacks of acute rheumatic fever were observed during the past two decades (1969 to 1988) at the Columbia-Presbyterian Medical Center; 104 were initial attacks and 11 were recurrences. The maximum number of cases (18) occurred during the first year of this period, 1969; thereafter the annual incidence decreased until a resurgence was apparent in 1985 to 1986, with 25 cases. There were no differences in the frequency of major manifestations nor the severity of carditis in the 1970s compared with the 1980s. Polyarthritis alone was present in 51 cases, carditis alone in 31, and combined carditis and polyarthritis in 28; chorea was diagnosed in 5. Congestive heart failure occurred in 17 attacks of carditis, including one death from fulminant disease in 1982. In contrast to recent reports, the majority of the affected population was urban, low income, and from crowded communities: more than half were Hispanic, predominantly from Dominican Republic families. The prevalence of acute rheumatic fever underscores the need for early detection and treatment of streptococcal pharyngitis. The observation that 9.5% of the attacks were recurrent reflects failure to comply with antimicrobial prophylaxis and reaffirms the need for more effective secondary prevention programs.     

A case of rheumatic fever with multiple recurrences of carditis

Rheumatic fever is still one of the major public health problems in developing countries. Recurrences have been reported during continuous regular prophylaxis. Here, we describe a 13-year-old girl who had four apparent recurrences of carditis while on a regimen of intramuscular benzathine penicillin G (BPG) every three weeks. The interesting and special features of this case were the presence of frequent recurrences of carditis with no evidence of arthritis despite regular BPG injections every three weeks, the requirement of long-term corticosteroid therapy due to these recurrences, and the shortness of time periods between attacks after withdrawal of steroid therapy. In this case, the administration of supraphysiologic doses of glucocorticoids caused iatrogenic Cushing syndrome with decreased bone mineral density and bilateral avascular tibial necrosis mimicking the symptoms of arthritis. Thus, this is a rare case. In similar cases with recurrences of either arthritis or carditis, instead of long-term corticosteroid therapy, we suggest a regimen of BPG prophylaxis every two weeks rather than every three weeks or monthly BPG injections because of the superiority of the every two weeks schedule in the adequate control of rheumatic fever recurrences, as shown in some previous studies.     

Acute rheumatic fever during childhood in Saudi Arabia.

During a 5-year period ending in December 1989, 73 episodes of acute rheumatic fever in 67 children aged 4-14 years were prospectively studied to ascertain the clinical profile of the disease in initial attacks and recurrences, and to compare the findings with those from other countries. Among 51 children with a first episode of acute rheumatic fever, 76% had arthritis and 43% had carditis. In 22 children with recurrences, arthritis was present in 45% and carditis in 91%. Carditis was more severe among the cases with recurrences. Mitral insufficiency was the most common valvular lesion, but no case of mitral stenosis was detected. Chorea, subcutaneous nodules, and Erythema marginatum were relatively rare. The demographic, clinical and laboratory findings of this study resemble those from Western countries, in contrast with data from tropical countries. Efforts aimed at prompt recognition and adequate treatment of streptococcal pharyngitis and maintenance of anti-streptococcal chemoprophylaxis would be rewarding in reducing the incidence of this disease and its sequelae.     

Rheumatic chorea in northern Australia: a clinical and epidemiological study.

To describe the epidemiology and clinical features of Sydenham's chorea in the Aboriginal population of northern Australia a review was conducted of 158 episodes in 108 people: 106 were Aborigines, 79 were female, and the mean age was 10.9 years at first episode. Chorea occurred in 28% of cases of acute rheumatic fever, carditis occurred in 25% of episodes of chorea, and arthritis in 8%. Patients with carditis or arthritis tended to have raised acute phase reactants and streptococcal serology. Two episodes lasted at least 30 months. Mean time to first recurrence of chorea was 2.1 years compared with 1.2 years to second recurrence. Established rheumatic heart disease developed in 58% of cases and was more likely in those presenting with acute carditis, although most people who developed rheumatic heart disease did not have evidence of acute carditis with chorea. Differences in the patterns of chorea and other manifestations of acute rheumatic fever in different populations may hold clues to its pathogenesis. Long term adherence to secondary prophylaxis is crucial following all episodes of acute rheumatic fever, including chorea, to prevent recurrence.     

Rheumatic chorea in northern Australia: a clinical and epidemiological study

To describe the epidemiology and clinical features of Sydenham's chorea in the Aboriginal population of northern Australia a review was conducted of 158 episodes in 108 people: 106 were Aborigines, 79 were female, and the mean age was 10.9 years at first episode. Chorea occurred in 28% of cases of acute rheumatic fever, carditis occurred in 25% of episodes of chorea, and arthritis in 8%. Patients with carditis or arthritis tended to have raised acute phase reactants and streptococcal serology. Two episodes lasted at least 30 months. Mean time to first recurrence of chorea was 2.1 years compared with 1.2 years to second recurrence. Established rheumatic heart disease developed in 58% of cases and was more likely in those presenting with acute carditis, although most people who developed rheumatic heart disease did not have evidence of acute carditis with chorea. Differences in the patterns of chorea and other manifestations of acute rheumatic fever in different populations may hold clues to its pathogenesis. Long term adherence to secondary prophylaxis is crucial following all episodes of acute rheumatic fever, including chorea, to prevent recurrence.     

Acute rheumatic fever in the young: changing prevalence and pattern

Acute rheumatic fever (ARF) is rare in young people. A group of 28 patients is described who were hospitalized during the years 1960–1984. Patients were divided into two groups: children who were admitted between 1960 and 1966 and those admitted between 1966 and 1984. The annual incidence of ARF declined from 1.17 to 0.44 cases per 10,000 population at risk. The incidence of arthritis declined in parallel to the decrease in number of children presenting with ARF, whereas the incidence of carditis remained the same.Abbreviations ARF acute rheumatic fever - RF rheumatic fever     

Acute rheumatic fever without early carditis: an atypical clinical presentation

The original Jones criteria, first introduced in 1944, have been modified four times and updated-revised criteria were published in 1992. A variety of clinical manifestations, which may be the presenting signs and symptoms of acute rheumatic fever, are not included in the updated-revised Jones criteria. A retrospective study was conducted on all children previously diagnosed to have acute rheumatic fever between September 1998 and September 2002. Review was focused on clinical presentation; out of 60 medical records reviewed, 4 patients with unusual clinical presentation were recognised and are reported here to highlight the potential diagnostic problems of acute rheumatic fever. They presented with atypical articular involvement, silent carditis and low-grade fever in the presence some time of a positive family history for rheumatic fever. Conclusion:a high index of suspicion and an awareness of the absence of early carditis are necessary to make the diagnosis of acute rheumatic fever.Abbreviations ARF acute rheumatic fever - PSReA post-streptococcal reactive arthritis - RF rheumatic fever     

Childhood acute rheumatic fever in Ankara, Turkey

Patients with acute rheumatic fever (ARF) admitted to a tertiary hospital in Ankara between January 1999 and July 2002 were studied cross-sectionally to verify the clinical profile and were followed during the acute period. All patients were investigated for throat culture, streptococcal serologic study, C-reactive protein, and erythrocyte sedimentation rate, and telecardiograms, electrocardiograms and echocardiographic study were done. During the study period, 129 attacks of ARF were observed: 118 were initial attacks and 11 were recurrences. Age on admission was 11.2 +/- 2.73 years (mean +/- SD, range: 6-21 years). Polyarthritis alone was present in 42 cases (33%), carditis alone in 33 (25%), combined carditis and polyarthritis in 36 (28%), combined carditis and chorea in 15 (12%) and chorea alone in 3 (2%). Nineteen (14%) patients with isolated arthritis and pure chorea had silent mitral and/or aortic regurgitation. Carditis was a dominant presenting manifestation, but appeared to be mild or moderate. The present study indicates that ARF is still a significant problem in Turkey. The observation that 8.5% of the attacks were recurrent reaffirms the need for more effective secondary prevention programs.     

Rheumatic fever: the relationship between clinical manifestations and laboratory tests

OBJECTIVES: To determine the relationship between the severity of clinical features of rheumatic fever (RF), and antistreptolysin O titre (ASOT) and/or erythrocyte sedimentation rate (ESR). METHODS: Clinical and laboratory data from 102 children with RF who had been admitted at a university hospital in Tehran between 1992 and 2002 were reviewed retrospectively. In order to categorize the severity of clinical manifestations of disease, patients were divided into three groups. Those with arthritis alone were defined as group A, carditis with or without arthritis as group B and carditis (with or without arthritis) with congestive heart failure as group C. RESULTS: Thirty-one cases were enrolled in group A, 39 in group B, and 32 in group C. We didn't find a significant relationship between the severity of clinical presentation of disease and ASOT (P = 0.89) and ESR (P = 0.24). Seventy-two patients presented with first attack and 30 had recurrences. The frequency of congestive heart failure (CHF) in first attacks was 31.2%vs 68.8% in recurrences (P < 0.0001). No Significant relationship was found between the number of involved valves and ASOT (P = 0.4) or ESR (P = 0.8). CONCLUSION: Variable clinical presentation of disease and increasing intensity of cardiac involvement is not related to the ASOT or ESR levels.     

Atrioventricular conduction in children with acute rheumatic fever.

Atrioventricular conduction was quantitatively evaluated in 118 children with acute rheumatic fever. The mean PR index in children with acute rheumatic fever, 1.06 +/- 0.38, was significantly higher than normal children or children who had febrile illness of nonrheumatic or nonstreptococcal origin (P is less than .001). Among 35 children with rheumatic fever and an abnormal PR index, the disease presented as carditis in 21, arthritis in ten, and chorea in four. The mean PR index and the frequency distribution of abnormal PR indices were significantly higher in children with carditis (P is less than .001). Five children who initially had an abnormal PR index and arthritis or chorea subsequently developed carditis. These observations suggest that children with acute rheumatic fever and abnormal PR index warrant close observation for possible clinical evidence of myocardial involvement during subsequent course of the illness.     

儿童风湿热及风湿性心瓣膜病85例临床分析

为观察儿童风湿热及风湿性心瓣膜病临床动态变化趋势,分析本院90年代诊治的85例风湿热及心瓣膜病发生状况、临床特点和治疗预防复发情况。结果示本组风湿热造成急性心瓣膜炎占77.65%,首诊慢性心瓣膜病占22.35%,其中三分之二患儿以往无急性风湿热病史。首次发病复发率为14.12%,风湿活动复发常以不同程度心力衰竭（心衰）症状就诊,激素抗风湿治疗能够迅速缓解急性心脏炎和改善心功能。1例反复风湿活动顽固性心力衰竭接受二尖瓣置换术效果甚佳。结果表明,减少儿童风湿性心瓣膜病形成不仅要控制急性风湿热,而且需要及时发现潜隐的风湿活动。     

Clinical-echocardiographic correlations in acute rheumatic fever

Thirty-three children with acute rheumatic fever were studied using echocardiography to characterize heart involvement in this disease. Among 26 subjects with a first episode of acute rheumatic fever, 18 had a clinical diagnosis of carditis and six had heart failure. Heart failure usually resulted from valvular incompetence rather than from myocardial failure in these patients. Conversely, among seven subjects with recurrent rheumatic fever, five had a clinical diagnosis of carditis and four had heart failure. Severe left ventricular dysfunction noted on echocardiography probably contributed significantly to the appearance of heart failure in two of these four subjects. Ten patients were initially believed not to have carditis: a diagnosis of mitral valvulitis was made in two of these ten on the basis of the results of the echocardiographic examination. Echocardiography, which provides important information on the cardiac status of patients with acute rheumatic fever, may help in assessing the prognosis and may be useful in the therapy of these patients.     

Acute rheumatic fever with three major criteria: polyarthritis, carditis and chorea. A case report

An eight-year-old girl is presented with three major criteria of acute rheumatic fever: polyarthritis, carditis and chorea. The diagnosis was confirmed with a history of pharyngitis 15 days prior to admission and with the findings of positive acute phase reactants such as elevated erythrocyte sedimentation rate (ESR) and C-reactive protein (CRP), elevated anti-streptolysin-O (ASO) titration, and clinical findings of polyarthritis, carditis and chorea. Patient responded well to salicylate and phenobarbital treatment. The rare association of these three major criteria in acute rheumatic fever is emphasized.     

Rheumatic fever presenting as monoarticular arthritis

BACKGROUND: Monoarticular arthritis in children is most often suppurative septic arthritis (SA) of bacterial origin. We recently cared for 3 patients with monoarticular arthritis who developed carditis while receiving antibiotics for SA. Distinguishing SA from rheumatic fever (RF) is critical to avoid lifelong cardiac complications associated with RF. METHODS: We compared the 3 cases of RF presenting with monoarticular arthritis with 12 cases of culture-confirmed SA to assess the clinical and laboratory differences between the 2 groups. RESULTS: Erythrocyte sedimentation rate, C-reactive protein and mean synovial fluid white blood cell counts were elevated in both groups. Mean antistreptolysin O (ASO) and anti-DNase B titers were elevated in patients with RF. CONCLUSIONS: The clinical and laboratory features of RF and SA demonstrate substantial overlap. Patients with monoarticular arthritis and sterile synovial fluid cultures should have RF included in their differential diagnoses. Patients with an elevated ASO and/or anti-DNase B titer should have a careful cardiac examination looking for clinical evidence of carditis. Echocardiogram should be considered if clinical carditis associated with RF is suspected.     

Clinical, laboratory and echocardiographic profile of acute rheumatic fever in Nepali children

BACKGROUND: Acute rheumatic fever (RF) is a common, preventable health problem in developing countries. Sporadic outbreaks and the prevalence in some indigenous populations have renewed interest in RF in developed countries also. AIMS: To describe the clinical, laboratory and echocardiographic features, outcome and value of echocardiography in detecting valvular disease in RF. METHODS: A prospective, cross-sectional study was conducted over 2 years. Patients under 14 years admitted to the cardiology unit of Kanti Children's Hospital, Kathmandu with RF using the Jones criteria were recruited consecutively. RESULTS: The median age (range) of the 51 patients was 11 (5-14) years, the male:female ratio was 1.6:1 and 39% had a history of a sore throat. Clinical and laboratory features detected were as follows: carditis 92%, arthritis 33%, chorea 8%, subcutaneous nodules 4%, fever 51%, arthralgia 37%, elevated antistreptolysin O titre 94%, elevated CRP 78%, prolonged PR interval 45%, pericardial effusion 22% and cardiac failure 28%. In total, 36 patients (71%) complained of joint pains. A murmur on auscultation was significantly associated with underlying diseased valves confirmed by echocardiography (p=0.001). A murmur was audible in 78.4% and diseased valves were confirmed by echocardiography in 88.2%. The mitral valve was the most commonly involved valve (82%) and mitral regurgitation the commonest lesion (24%). A thickened mitral valve predicted carditis (p=0.007). Five (10%) patients died. CONCLUSION: Inclusion of echocardiographic evidence of carditis and possibly arthralgia as major criteria would improve case detection.     

Outbreak of acute rheumatic fever in northeast Ohio

An outbreak of acute rheumatic fever in 23 patients was seen in northeastern Ohio in 1986, with polyarthritis (78%) and carditis (30%) the major manifestations. Results of a questionnaire completed by the parents of all 23 patients indicated that they were generally nonindigent, with good access to medical care, and from a nonurban setting. Records of inpatients with acute rheumatic fever were reviewed for the 10 years prior to 1986. The clinical manifestations in inpatients from 1976 to 1985 were not significantly different from those of inpatients seen during 1986. Similar outbreaks have been reported elsewhere, and physicians should be aware of a possible resurgence of classic acute rheumatic fever and ensure compliance with an adequate course of therapy for patients with streptococcal pharyngitis.     

Pneumonia associated with acute rheumatic fever

Pulmonary involvement in acute rheumatic fever is rare. Clinical and laboratory findings in rheumatic pneumonia are non-specific. Most of the cases of rheumatic pneumonia described in recent years have had a fulminant course often resulting in death. Autopsy findings are suggestive of but not specific for rheumatic pneumonia. In this article a case of pneumonia associated with acute rheumatic fever is described. Pneumonia and carditis occurred simultaneously in a 10-year-old boy treated without steroids, which resulted in recovery. The clinical spectrum of rheumatic pneumonia can range from mild to fulminant.