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Diagnosis of rheumatic fever: current status of Jones Criteria and role of echocardiography.

The original Jones Criteria as proposed by Dr. T. Duckett Jones have been modified four times and the updated revised criteria were published in 1992. According to this latest publication major manifestations are carditis, polyarthritis, chorea, erythema marginatum and subcutaneous nodules. Minor manifestations include fever, arthralgia and laboratory findings of elevated erythrocyte sedimentation rate, C-reactive protein and prolonged PR interval on ECG. For making a diagnosis of acute rheumatic fever, two major, or one major and two minor manifestations must be accompanied by supporting evidence of antecedent group A streptococcal infection in the form of positive throat culture or elevated or rising anti-streptolysin titre. The updated guidelines also highlighted a subgroup of "exceptions to Jones Criteria" for patients with chorea, indolent carditis and previous history of rheumatic fever or "rheumatic heart disease". Role of echocardiography has not been defined in these modifications but may be important, as clinical detection of soft murmurs may be difficult due to tachycardia. Doppler and color flow mapping is more sensitive in picking up minor digress of valvular regurgitation. Several studies have confirmed that the yield of carditis with valvular regurgitation increased with use of echocardiography in patients with acute rheumatic fever. Also echocardiography is of great help in mixed valve lesions to determine the severity of each lesion. Other abnormalities detected on echocardiography in acute carditis include prolapse of the valve, focal nodular thickening of leaflets and pericardial effusion. Jones Criteria are guidelines to assist the physician and should not be substituted for clinical judgement as strictly following them may result in underdiagnosis of this disease in our country.

Biopsy↗

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.

Child↗

[Presence of a non-HLA antigen in B-lymphocytes from patients with rheumatic fever and their relatives defined using monoclonal antibodies].

Numerous investigators have suspected that there is a genetic predisposition to rheumatic fever (RF). In this context a group of investigators at Rockefeller University have produced a monoclonal antibody that identifies an antigen present in 100% of all RF studied at that center. Using this antibody, labeled D8/17, we studied 47 patients with acute rheumatic fever and rheumatic heart disease. Of these, 39 were not receiving steroids at the of the test and 35 were positive for the marker (89.7%). The highest percentage of positive cells was seen in the probands with 34.6 +/- 13.16%, while unaffected mothers, fathers and siblings gave 24.9, 5.2 and 7.3% respectively. The control group had an average of 7.5% of positive cells. This study and previous ones, performed by the Rockefeller University group in which HLA typing was included, suggest an autosomal recessive mode of inheritance, not associated with the MHC system, for the D8/17 antigen. Rheumatic fever; non-HLA antigen in; genetic predisposition in.

Adolescent↗

Diagnosis of rheumatic fever obscured by liver disease.

Two children with acute rheumatic fever had concurrent liver disease which obscured their diagnosis. One patient had evidence of viral hepatitis and the other apparently had liver toxicity from high doses of aspirin. The differential diagnosis of arthritis with liver disease must include acute rheumatic fever.

Adolescent↗

The resurgence of acute rheumatic fever in the United States.

The incidence of acute rheumatic fever (ARF) had been declining in the United States for decades, and by the late 1970s ARF had become a rare disease in most areas of the country. The mid-1980s saw a resurgence of ARF, with reported outbreaks in Utah, Ohio, Pennsylvania, and military recruit camps in Missouri and California, as well as sporadic cases in many other communities. This review summarizes salient epidemiologic data related to the resurgence, explores some possible explanations for its occurrence, and discusses strategies for prevention of rheumatic fever now and in the future.

Acute Disease↗

[Rheumatic fever prevention unit].

The author describes the steps that have been taken recently on the prevention and the magnitude of rheumatic fever in Chile. Likewise, he describes the measures proposed by the Advising Committee on Rheumatic Fever, the evolution this disease has followed and the goals that they expect to reach before the next Pan American Conference on Rheumatic Fever.

Adolescent↗

[Comparative study of clinical and Doppler echocardiographic evaluations of the progression of valve diseases in children and adolescents with rheumatic fever].

OBJECTIVE: Compare clinical and Doppler echocardiographic evaluations in assessing valvular diseases in children and adolescents with rheumatic fever, as well as assess the progression of the disease in light of these assessments. METHODS: This is a longitudinal study of 258 children and adolescents diagnosed with rheumatic fever according to Jones criteria. The follow-up period ranged from 2-15 years. The presence and quantification of valve diseases were determined by means of clinical and Doppler echocardiographic evaluations performed during the acute and chronic phases. The Kappa statistics method was used to estimate the degree of agreement between clinical and Doppler echocardiographic evaluations. Comparisons between clinical and Doppler echocardiographic findings on the progress of carditis and valvulitis, respectively, were made using chi-square test or Fishers exact test, p< 0.05. RESULTS: Of the 109 patients who underwent Doppler echocardiographic evaluation during the acute phase, 31 did not present clinical evidence of carditis, but the Doppler echocardiograms of 17 (54.8%) of them showed valve lesions (subclinical valvulitis). During the chronic phase, 153 of the 258 patients had normal cardiovascular examination results; however, Doppler echocardiograms showed that 81 of them (52.9%) had valve lesions (subclinical chronic valvular diseases). Involution of the valvular lesions, as shown by Doppler echocardiographic evaluations, was less frequent and occurred in 10 (25.0%) patients with mild valvulitis, in only one (2.5%) patient with moderate valvulitis, and in none of the patients with severe valvulitis. CONCLUSION: The identification of rheumatic fever valve lesions can be enhanced when clinical evaluations are supplemented by Doppler echocardiographic examinations; also, clinical examinations are not as suitable to detect valvular lesion regression as the echocardiography. The diagnosis of subclinical valvulitis and valvulopathy influences the secondary prophylaxis of rheumatic fever and endocarditis.

Acute Disease↗

Carditis in acute rheumatic fever in Varanasi, India.

In Varanasi carditis is the most important major manifestation of an acute attack of rheumatic fever and is the only one which results in sequelae. In our country carditis constitutes the largest single manifestation of rheumatic fever in its index attack. The overall picture is most distressing with its high incidence (78%), and severity as evidenced by high mortality (70%) in the initial phase. It is necessary to provide institutional treatment for those seriously infected to ensure them the fairest chance of recovery from their carditis without permanent damage to the heart. The diagnosis of carditis is therefore imperative in all cases of acute rheumatic fever even in the absence of other major criteria which may be subdued.

Adolescent↗

[Is there a rheumatic fever?].

In 1988, 11 patients with a suspicion to rheumatic fever (RF) or acute polyarthritis were admitted to the Department of Internal Medicine, University Hospital, "Dr. M. Stojanović". Eight patients have been extensively treated together with a physiatrist, while one of them on an outpatient basis. Follow-up has been from 3 to 9 months. Rheumatic fever was diagnosed with a certainty in only one patient. Three have had the diagnoses of Reiter's syndrome and individually, of infectional, ankylosing and rheumatoid arthritis. One patient is still followed up because of the symptomatic diagnosis of partially cured oligoarthritis or recurrent RF. The numerical relation of established diagnoses for the followed up patients has been in accordance with the decrease in incidence of RF both in our country and the world some decades ago. The possible causes of frequent diagnoses of RF in adults and the necessity of careful evaluation of clinical and laboratory parameters and the need to follow-up unclear polyarthritis even when the patients are not hospitalized, until the definite diagnosis has not been made is stressed in this paper. Therefore, the administration of corticosteroids has to be avoided in these patients, except in special cases.

Adult↗

A clinical, laboratory and echocardiographic profile of children with acute rheumatic fever.

A retrospective study of 42 children with acute rheumatic fever admitted to Hospital Universiti Sains Malaysia from April 1985 to March 1989 was undertaken to assess the clinical, laboratory, echocardiographic aspects and outcome. The ages of the children ranged from 5 years 9 months to 11 years 11 months. There was no significant sex difference. 69.4% were admitted between November and April with a seasonal low between May and August. Sixteen children (38.1%) were hospitalised for recurrence of rheumatic fever. Carditis was the commonest manifestation and was seen in 28 (66.6%) children, followed by arthritis in 24 (57.1%), and chorea in 3 (7.1%). Echocardiography detected abnormalities in 24 out of 35 cases and the most common echocardiographic findings were poor coaptation of mitral valve (ten) left ventricular dilatation (ten), thickened mitral valve cusps (seven) and pericardial effusion (seven). In those children followed up, there were 2 recurrences while on secondary prophylaxis and complete recovery was seen only in 11 (26.9%).

Child↗

Studies of peripheral blood T lymphocytes in assessment of disease activity in rheumatic fever.

The study included three groups of children: (a) 38 with active rheumatic fever (ARF) and active carditis; 21 seen during their first attack and 17 during recurrence of activity, (b) 47 with inactive rheumatic fever (IARF); the period since activity was less than 3 years in 31 cases and more than 3 years in 16 cases. Using monoclonal antibodies and T lymphocyte blast transformation induced by PHA, we found: (1) low total T lymphocytes, helper-inducer cells and helper-inducer/suppressor-cytotoxic ratio which persisted for years; and (2) reduced lymphoblast transformation in active disease.

Adolescent↗

The incidence of acute rheumatic fever in a suburban area of Los Angeles. A ten-year study.

The incidence of in-hospital cases of acute rheumatic fever in residents of the San Fernando Valley section of Los Angeles (population 1,096,000) from 1971 through 1980 was 0.21 per 100,000 population for all ages and 0.63 per 100,000 for 5 to 17 year olds, both among the lowest ever reported in the world literature. Nevertheless, minority group patients had five times the risk of a white patient for acute rheumatic fever developing and a greater than 20-fold increase in the risk for the development of acute rheumatic fever with carditis. Unexpectedly, a low 30% of cases (8/27) had carditis either alone or with other major criteria, while a high 59% (16/27) had polyarthritis as the only major manifestation. This suggests the possibility of false-positive cases based on polyarthritis as the only major manifestation, and that an incidence of acute rheumatic fever may have been reached that is so low that "background" cases are a significant proportion of all cases.

Acute Disease↗

[The changing clinical pattern of rheumatic fever (author's transl)].

During the last years a remarkable change in the pattern of rheumatic fever has been observed. Severe carditis and polyarthritis have almost disappeared. Accordingly the mortality rate decreased considerably milder forms of rheumatic fever may become a diagnostical problem so that adequate therapy may be delayed. These cases may present as chronic heart disease with an unspecific history. The modified Jones criteria alone have limited value. Laboratory tests are increasingly important. Not only improvement of hygiene or therapy with Penicillin are causes for the declining incidence of rheumatic fever, but also the change in the behaviour of Streptococci. This assumption will be supported if the equally dranged course of scarlet fever and the uniform ASO-titers in children as a measure for contacts with Streptococci are taken into account.

Antistreptolysin↗

Evaluation of DNA damage using the comet assay in children on long-term benzathine penicillin for secondary prophylaxis of rheumatic fever.

BACKGROUND: Benzathine penicillin is the most widely used antibiotic in the prophylaxis of children with rheumatic fever. The aim of the present study was to evaluate the DNA damage in children receiving one dose of 1.2 million units benzathine penicillin every 4 weeks over a long period to prevent recurrences of rheumatic fever. METHODS: Thirty-five children with confirmed rheumatic fever under benzathine penicillin prophylaxis were enrolled in the study and 35 healthy children with similar ages and socioeconomic backgrounds served as controls. To detect any DNA damage, the comet assay was performed on circulating lymphocytes from the study subjects. RESULTS: Damaged (limited and extensive migration) cells in children on prophylactic therapy were higher than those in controls (P<0.001). CONCLUSIONS: It has been suggested that differences in the comet scores were induced by the administration of benzathine penicillin over a long period of time and further investigations are needed to confirm this toxic effect.

Adolescent↗

Acute rheumatic fever without early carditis: an atypical clinical presentation.

UNLABELLED: 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.

Acute Disease↗

Rheumatic fever: clinical profile of the initial attack in India.

The clinical profile of acute rheumatic fever in developing countries is frequently reported to differ from that in developed countries. This probably arose because a distinction was not made between the manifestations of the initial attack and those of a recurrence. Here, we report the patterns of presentation and clinical features of 100 cases of carefully determined initial attacks of rheumatic fever. As many as half the patients had carditis, and, of these, 50% exhibited congestive cardiac failure. This high incidence arises because in developing countries with limited health-care facilities patients continue to be physically active during the long pre-admission period. The study confirms that the clinical profile of the initial attack of rheumatic fever in developing countries is in most respects not unlike that in developed countries.

Adolescent↗

Rheumatic pneumonia: reappearance of a previously recognized complication of acute rheumatic fever.

The clinical diagnosis of acute rheumatic fever (ARF) may be challenging; however, a constellation of signs including new valvular insufficiency, cardiomegaly, and heart failure should readily prompt consideration of the diagnosis of rheumatic carditis. In addition, pulmonary findings are compatible with ARF, as associated pulmonary involvement may represent rheumatic pneumonia. We report the case of a young man with ARF and rheumatic pneumonia, a previously described but rare complication of ARF.

Adult↗

Lack of association between TNFalpha gene polymorphism at position -308 and risk of acute rheumatic fever in Turkish patients.

OBJECTIVE: Acute rheumatic fever (ARF) is a multisystem inflammatory disease process that follows nasopharyngeal infection caused by group A streptococcus (GAS) (Streptococcus pyogenes). Recent studies have demonstrated that allelic variations at the tumour necrosis factor alpha (TNFalpha) locus are involved in the nature of rheumatic diseases such as juvenile idiopathic arthritis and rheumatic heart disease. Thus, TNFalpha polymorphisms at -308 in ARF patients might be useful in contributing to identification of the primary factors associated with pathogenesis of ARF. METHODS: We performed a case-control association study between the common G/A promoter polymorphism at position -308 in the TNFalpha gene and ARF in Turkish patients, investigating whether this locus acts as a risk factor or has a modifying effect. RESULTS AND CONCLUSION: Previous studies have reported that TNFalpha plays a major role in the pathogenesis of a number of autoimmune and inflammatory diseases. Moreover, significantly elevated TNFalpha levels were reported in patients with ARF. However, in our sample of patients with ARF (n = 66), no such association was found. No interactive effect was found between the TNFalpha polymorphism at position -308 and no association was detected with disease progression. These findings suggest that the role of TNFalpha in ARF may be in linkage disequilibrium with some other severity genes not yet genetically determined.

Acute Disease↗