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PubMed1984

Acute rheumatic fever in north Florida.

Because of the declining incidence and severity of acute rheumatic fever reported in the United States and Europe, we reviewed our clinical experiences with 128 cases of acute rheumatic fever. No decline in the rate of admissions for patients with rheumatic fever was found in our hospital. The most common major manifestation of rheumatic fever in our patients was carditis, followed by arthritis. Chorea, erythema marginatum, and subcutaneous nodules were rare. Carditis was severe in 26% of the patients, moderate in 27%, and mild in 47%. A high percentage of our patients had residual cardiac lesions, and the mortality was higher than what has been reported in the rest of the country. We conclude that primary prevention of rheumatic fever in areas like ours should be continued.

Acute Disease↗
PubMed1994

Rheumatic fever prophylaxis in South Africa--is bicillin 1,2 million units every 4 weeks appropriate?

Rheumatic fever is a major health problem in South Africa. Although intramuscular benzathine penicillin (bicillin) 1.2 million units (MU) every 4 weeks is widely used for secondary prophylaxis, studies in other countries have shown a recurrence rate of 3-8% over 5-6 years in patients on this regimen. It has been recommended that serum penicillin concentrations should be maintained above 0.02 mg/ml to prevent such recurrences. The World Health Organisation (WHO) and the American Heart Association have recommended since 1988 that patients in high-risk areas for the development of rheumatic fever should receive benzathine penicillin 1.2 MU every 3 weeks rather than every 4. The aims of this study were, firstly, to determine the prevalence of serum penicillin concentrations below 0.02 micrograms/ml in rheumatic fever patients on benzathine penicillin 1.2 MU 4-weekly and, secondly, to study the effect of increasing the dose of 1.8 MU 4-weekly in patients with subtherapeutic concentrations. Forty-five of 51 rheumatic fever patients (88%) in this study on benzathine penicillin 1.2 MU 4-weekly had low serum penicillin concentrations (< 0.02 micrograms/ml) at the end of the 4th week after the injection. Penicillin was detected in the urine of 30 of the 45 patients (67%) with low concentrations, suggesting that such patients have tissue-bound penicillin which might be important in preventing rheumatic fever. The 15 patients (33%) with subtherapeutic serum penicillin concentrations and no detectable penicillin in the urine could be at very high risk for recurrent attacks of rheumatic fever. Fourteen of 29 patients (48%) given the higher dose of benzathine penicillin (1.8 MU 4-weekly) had subtherapeutic serum penicillin concentrations at the end of the 4th week after the injection, but in all 29 penicillin was detected in the urine. Review of our present policy of secondary prophylaxis for rheumatic fever is necessary. Concentrated preparations of benzathine penicillin (600,000 U/ml) are not available in South Africa; administration of a higher dose (1.8 MU) 4-weekly would therefore require a double injection, which could affect compliance adversely. We recommend that rheumatic fever patients in our area should receive benzathine penicillin 1.2 MU 3-weekly as recommended by the WHO until strategies for secondary prophylaxis have been evaluated further.

Adolescent↗
PubMed1978

Dermatoglyphic alterations associated with acute rheumatic fever in children.

The dermatoglyphic configurations of 78 children with acute rheumatic fever were compared with those of 46 first-degree relatives and 1,310 normal subjects. Of the children with acute rheumatic fever, 75% had an ulnar deviation of the axial triradius. In about 40% of this group, the ulnar deviation was associated with a concomitant distal displacement, which resulted in a significantly higher mean maximal angle atd (P less than .001) and significantly lower mean ab and td ridge counts (P less than .001) relative to normal control values. The palmar dermatoglyphics of patients with acute rheumatic fever were more closely related to the configurations of first-degree relatives than to normal controls. The dermatoglyphic profiles of six patients were nearly identical to those of their first-degree relatives, all of whom had a history of acute rheumatic fever. Presence of abnormal dermatoglyphic profiles in a large proportion of children with acute rheumatic fever supports the hypothesis that certain individuals have a genetic predisposition to this disease.

Acute Disease↗
PubMed2000

Culturing the throat to protect the heart: Dr. Milton Markowitz and the prevention of rheumatic fever.

The purpose of this article is to briefly review how the understanding of rheumatic fever has evolved over the last 50 years. Particular emphasis is given to the identification of the Group A streptococcus as the causative agent of rheumatic fever and the use of antibiotics to treat and prevent rheumatic fever. Throughout his 50-year career, Dr. Milton Markowitz, former chairman of the department of pediatrics at the University of Connecticut School of Medicine, has been involved in these advances as an international expert on rheumatic fever prevention. Using archival materials, including some of the earliest literature descriptions of rheumatic fever, as well as an extended interview with Dr. Markowitz, the authors present a unique personal perspective both on the history of rheumatic fever and the context within which the scientific advances that have led to contemporary treatment and prevention strategies have evolved.

Connecticut↗
PubMed1993

Acute rheumatic fever.

During the first half of this century rheumatic fever was a common disease with significant morbidity and mortality in the United States. In the 1980s, when many clinicians were hoping this disease was a disease of the past, anxieties were renewed when outbreaks were reported in several areas around the country. Although the etiology still eludes us, insight has been gained. Environmental and genetic factors are believed to play a role in the epidemiology of this disease. Additionally, the implicated organism, the group A streptococcus, has many strains, and differences in its many proteins may determine their potential for rheumatic fever. The mechanisms leading to disease are not clear, but the streptococcus has been implicated as a source of antigens with cross-reactivity with human tissues and has been shown to modify immune mechanisms. Clinical aspects are briefly reviewed and physicians are reminded to consider rheumatic fever as a diagnostic possibility in the appropriate settings.

Acute Disease↗
PubMed1986

Recurrent abdominal and cervical pains. An unusual clinical presentation of acute rheumatic fever.

Most cases of acute rheumatic fever (ARF) present with arthritis, carditis or choreiform movements. However, a variety of clinical manifestations which are not included in the modified Jones criteria can be the presenting symptoms of the disease. We describe a case of a 10-year-old boy with ARF who presented with recurrent episodes of abdominal and cervical pain who later developed an active carditis which established the diagnosis of ARF. A high degree of suspicion and an awareness of the less common manifestations of ARF are necessary to make an early diagnosis and initiate appropriate treatment in certain cases of ARF.

Abdomen↗
PubMed2000

Rheumatic fever in the Nazareth area during the last decade.

BACKGROUND: Acute rheumatic fever is considered a relatively uncommon disease in developed countries. Although cases are encountered in the Nazareth area in Israel, no systematic study of this disease has been done in the last 20 years. OBJECTIVE: To study the incidence and characteristics of acute rheumatic fever cases in the Nazareth area during the last decade. METHODS: Cases of acute rheumatic fever diagnosed according to the modified Jones criteria were identified in two hospitals in the Nazareth area during the 10 years. These two hospitals receive about 75% of non-obstetric referrals to the emergency room. Clinical, laboratory and treatment data of these patients were documented and the incidence of disease calculated. The population census in the Nazareth area was obtained from the Central Bureau of Statistics. RESULTS: Forty-four patients, with a mean age of 18 years, were identified. The mean annual incidence was 5 cases/100,000 population. Arthritis was found in 98% of the patients (migratory in 52%) and carditis in 34%, but only one patient had a subcutaneous nodule, and none had either erythema marginatum or chorea. Only one patient with carditis developed heart failure a few months later due to severe mitral stenosis. CONCLUSION: Rheumatic fever in the Nazareth area is still manifest. The mean age of the patients was higher than found previously. In only half of the patients was the arthritis migratory, with other major manifestations of rheumatic fever found only rarely.

Acute Disease↗
PubMed1964

IMMUNOLOGIC RELATION OF STREPTOCOCCAL AND TISSUE ANTIGENS. III. PRESENCE IN HUMAN SERA OF STREPTOCOCCAL ANTIBODY CROSS-REACTIVE WITH HEART TISSUE. ASSOCIATION WITH STREPTOCOCCAL INFECTION, RHEUMATIC FEVER, AND GLOMERULONEPHRITIS.

Sera from patients with recent streptococcal infection or non-suppurative sequelae exhibit with variable frequency a precipitin reaction in agar gel with a partially purified streptococcal antigen which has been shown previously to be immunologically related to human heart tissue. This precipitin could be absorbed from sera with normal human heart tissue homogenates but not with homogenates of other organs. Demonstration of this cross-reaction by heart absorption was found dependent both upon the serologic properties of individual sera and the nature or state of purification of the streptococcal product employed as test antigen. Evidence was obtained of a close association of heart-related and non-heart-related antigenic determinants in partially purified preparations of the streptococcal antigen by both gel diffusion and immunoelectrophoresis. On immunoelectrophoretic analysis, cross-reactive antigen exhibited a more rapid mobility toward the anode than M protein. It was destroyed by digestion with trypsin, pepsin, and chymotrypsin. Based on specific absorption tests with a Type 5 and Type 19 strain, the antigen was localized to cell walls and to a lesser extent to cell membranes of these strains. Precipitating activity related to cross-reactive antibody was localized to the immunoglobulin zone in immunoelectrophoresis. Reactive sera showed diminution or loss of serological activity following heat inactivation at 56 degrees C or after prolonged storage at 4 degrees C. Sera containing cross-reactive precipitating antibody exhibited an immunofluorescent reaction with sarcolemma of cardiac myofibers, which was inhibited by streptococcal cross-reactive antigen. By this inhibition test, the immunofluorescent reaction related to cross-reactive antibody could be distinguished from that due to other heart-reactive factors. Antibody to streptococcal cross-reactive antigen defined by precipitation-absorption tests was observed in 24 per cent of patients with recent history of uncomplicated streptococcal infection and in the majority of patients with acute rheumatic fever, rheumatic heart disease, or acute glomerulonephritis. It was observed rarely in patients with non-streptococcal related disease. These data provide evidence that induction of cross-reactive autoantibody to heart in certain individuals is associated with streptococcal infection.

Aged↗
PubMed1981

Diagnostic criteria of rheumatic fever in a New Zealand community.

The diagnostic criteria of acute rheumatic fever are reviewed from experience in a New Zealand community. Two-hundred-and-sixty-one first attacks labelled rheumatic fever and 209 other episodes occurred in a defined geographic population during 1962--76. The major importance of heart, joint and brain manifestations is confirmed. Criterior levels for fever, P-R interval prolongation, erythrocyte sedimentation rate, white cell count and antistreptolysin-O titre are suggested. Nodules and evidence of established rheumatic heart disease were not found to be of diagnostic significance because they tended to be equally common among patients with rheumatic fever and patients considered to have another disease. Separate criteria for cases with and without pre-existent rheumatic heart disease are given, to enable assignment of episodes to probable rheumatic fever status.

Adult↗
PubMed1976

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.

Acute Disease↗
PubMedDate not supplied

The decline of acute rheumatic fever in Israel.

INTRODUCTION AND METHODS. In Israel, as in most economically developed countries, rheumatic fever has become uncommon, although cases continue to be reported each year, particularly in the Arab population. In this observational survey, based on cases notified to, and investigated by, the Ministry of Health, we review the epidemiology of acute rheumatic fever in Israel in the decade 1981-90. RESULTS AND RECOMMENDATIONS. Where it persists in Israel, acute rheumatic fever remains commoner in non-Jews than in Jews and in males rather than in females, in children aged 5-14, and in children from relatively overcrowded and deprived homes. Despite the steady decline in rheumatic fever incidence in the past decade, its continued occurrence in Israel, and reports of outbreaks of acute rheumatic fever and virulent streptococcal infection in North America and Europe, demand that clinicians continue to treat streptococcal sore throats with full curative antibiotic doses, that confirmed cases receive extended chemoprophylaxis, and that surveillance and notification of acute rheumatic fever be continued.

Acute Disease↗
PubMed1993

Acute rheumatic fever.

A funny thing has happened on our way to the elimination of rheumatic fever from the United States. It is quite clear that, at least in some areas, rheumatic fever has made a dramatic resurgence. Although all the factors accounting for this are not clearly understood, there are several obvious conclusions that must be drawn: 1. Rheumatic fever is no longer the province of the poor and overcrowded. 2. Clinical manifestations, as embodied in the Jones criteria, may have changed somewhat over the years. 3. New insights into the genetics and immunology of rheumatic fever may lead to a vaccine and/or effective therapy in the future. 4. Rheumatic fever will continue to occur unless and until we resume an aggressive approach to the treatment and prophylaxis of pharyngitis, probably utilizing benzathine penicillin as our primary drug.

Acute Disease↗
PubMed1995

Echocardiographic diagnosis of subclinical carditis in acute rheumatic fever.

The diagnosis of carditis in acute rheumatic fever traditionally depends on characteristic auscultatory findings. The advent of pulsed and colour Doppler echocardiography provides a method of detecting minor degrees of pathological regurgitation without characteristic clinical signs. Using strict criteria, pathological left heart regurgitation can be differentiated from physiological regurgitation: colour Doppler must show a substantial colour jet in two planes extending well beyond the valve leaflets; pulsed Doppler must confirm a high velocity signal, holosystolic for mitral regurgitation, or holodiastolic for aortic regurgitation. Several centres have observed subclinical carditis in children with acute rheumatic fever. We are confident that we are not overdiagnosing valvulitis, having tested this in a blinded fashion. Subclinical valvulitis should be accepted as evidence of carditis, a major diagnostic criterion for acute rheumatic fever.

Child↗
PubMed2001

Can subclinical valvitis detected by echocardiography be accepted as evidence of carditis in the diagnosis of acute rheumatic fever?

AIM: Subclinical valvar insufficiency, or valvitis, has recently been identified using Doppler echocardiography in cases of acute rheumatic fever with isolated arthritis or chorea. The prognosis of such patients with acute rheumatic fever and subclinical valvitis is critical when determining the duration of antibiotic prophylaxis. We aimed, therefore, prospectively to investigate the association of silent valvitis in patients having rheumatic fever in the absence of clinical evidence of cardiac involvement, and to evaluate its prognosis. METHODS AND RESULTS: Between November 1998 and September 1999, we identified 26 consecutive patients with silent valvitis in presence of rheumatic fever but in the absence of clinical signs of carditis. The patients, eight female and 18 male, were aged from 6 to 16 years, with a mean of 9.9+/-2.7 years. Major findings were arthritis in 16, chorea in 7, and arthritis and erythema marginatum in 1 patient. Two cases had arthralgia with equivocal arthritic signs and Doppler echocardiographic findings of pathologic mitral regurgitation. Silent pathologic mitral regurgitation was found in 12 cases, and aortic regurgitation in 2 cases. All patients with arthritic findings were treated with acetylsalicylic acid with one exception, this patient receiving both prednisone and acetylsalicylic acid. No antiinflammatory treatment was given to patients with chorea. After a mean follow-up of 4.52 months, valvar regurgitation disappeared in 4 patients, including the one with migratory arthralgia and no other major criterions. All six patients with chorea and silent carditis still have mitral insufficiency. CONCLUSION: Acute rheumatic fever without clinical carditis is not a benign entity. Doppler echocardiographic findings of subclinical valvar insufficiency, therefore, should be considered as carditis when seeking to establish the diagnosis of acute rheumatic fever.

Acute Disease↗
PubMed2000

Association of HLA-DR7 with rheumatic fever in the Brazilian population.

OBJECTIVE: Rheumatic fever (RF) is a multisystem inflammatory disease that develops as a sequel of untreated throat infection by the group A beta-hemolytic streptococcus. As HLA antigens are known to be important in controlling immunological responsiveness, studies have investigated HLA antigen association with RF. Studies with Caucasians, Black Americans, and Indians showed associations with HLA-DR4, DR2, and DR3, respectively. One study on a Brazilian population suggested an association with HLA-DR7 and HLA-DR53. We investigated the association between RF and antigens HLA-DR7 and DR53 in the white Brazilian population. METHODS: Thirty-five patients and 209 healthy individuals living in the northern region of the state of Parana, Brazil, were used as test and control groups, respectively. Classical statistical methods were used to compare HLA frequencies between these groups. Results. Data confirmed positive association with HLA-DR7 (46.7 vs. 25.7%; p = 0.015), but not with HLA-DR53 (54.3 vs. 44.5%; p = 0.28). The relative risk and etiologic fractions were 2.4 and 0.27%, respectively. CONCLUSION: Positive association between HLA-DR7 specificity and RF was observed in the white Brazilian population by 2 independent studies, supporting the hypothesis of the involvement of genetic factors in susceptibility of rheumatic fever.

Brazil↗
PubMed1998

Does rheumatic fever occur usually between the ages of 5 and 15 years?

It has long been considered that rheumatic fever usually occurs in children between the ages of 5 and 15 years. However, supporting data from the developing countries are insufficient. It is important to know the age of occurrence of rheumatic fever for clinical and public health purposes. To describe the age distribution of Bangladeshi subjects, we have reviewed the records of all patients who attended with acute rheumatic fever in the outpatient department of the National Center for Control of Rheumatic Fever and Heart Diseases, Dhaka, during June 1990 through November 1995. During this period 630 subjects were diagnosed to have acute rheumatic fever defined by the revised Jones criteria. Of them, 535 (84.9%) presented with first attack. Their age ranged from 3 to 30 years, and a skewness to the right of the age distribution was observed. Thus, we used percentile distribution to determine reference range of age. The 2.5 and 97.5 percentiles were 5 and 22 years, respectively. Their mean (standard deviation) and median age were 12.7 (4.4) and 12 years, respectively. Slightly more than 22% of subjects were older than 15 while less than 1% were younger than 5 years. This finding was supported by multiethnic data from other developing countries. The current analysis warrants reevaluation of the prevailing conviction for age of occurrence of rheumatic fever. In conclusion, the age reference range for occurrence of rheumatic fever in Bangladesh should be considered to be 5 to 22 years but not 5 to 15 years.

Adolescent↗