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PubMed1981

Yersinia arthritis mimicking acute rheumatic fever. A case report.

A reactive or post-infectious arthritis is a well-known complication of yersinia enterocolitica infection and tends to occur in patients with the HLA-B27 haplotype. We describe such a patient, who in addition satisfied the revised Jones criteria for a diagnosis of acute rheumatic fever. Our case illustrates that arthritis due to Yersinia may mimic acute rheumatic fever both clinically and serologically. In areas where Yersinia infection is known to occur, infection with this organism should be excluded in all patients who satisfy the revised Jones criteria.

Adult↗
PubMed1981

Rheumatic fever in children. A follow-up study with emphasis on cardiac sequelae.

Eighty-nine children with rheumatic fever between 1964 and 1978 were reviewed. There were 40 males and 49 females. The first attack occurred mostly between 6 and 12 years. Arthritis was present in 62.9%; carditis, 55.1%; chorea, 19.1%. Children were followed from 20 months to 15 years (mean 61 months). Seventeen developed current attacks (19.1%). On final examination, 40 had no heart disease, 30 had mild heart disease, 6 required valve surgery, 4 had died, and 9 had moderate heart disease but not operated. Chorea was present in 20 children. Six of them also had mitral insufficiency, 3 had recurrence after episode of chorea. Prompt diagnosis and treatment of streptococcal infection and penicillin prophylaxis need to be stressed. Service carditis on initial attack and recurrence predispose to the development of severe heart disease.

Adolescent↗
PubMed1990

T lymphocyte subsets, suppressor and contrasuppressor cell functions, and production of interleukin-2 in the peripheral blood of rheumatic fever patients and their apparently healthy siblings.

We studied CD4 and CD8 T cell subsets, suppressor cell function, production of IL-2, and immune contrasuppressor cell activity in 21 patients with rheumatic fever (RF), both at the time of their first acute episode and 3 months later (recovery phase). As controls we studied their healthy sibling nearest in age, as well as age- and sex-matched unrelated normal subjects. In the acute phase we found CD4+ T cells to be high, concanavalin A-induced suppression to be low, and production of IL-2 to be significantly decreased, as compared to the normal unrelated controls. The addition of contrasuppressor cells (VV+) to cell cocultures resulted in an increase in proliferation by mononuclear cells (MNC) in response to streptococcal M antigen but not to C carbohydrate antigen. In the recovery phase, CD4+ T cells became normal, CD8+ T cells rose above normal, and the suppressor cell functions (concanavalin-A-induced and spontaneously expanded), as well as the production of IL-2, fell further. Siblings were found to have increased CD8+ T cells and decreased production of IL-2, as compared to the unrelated controls. These findings indicate that important immunoregulatory disturbances occur during the acute phase of rheumatic fever, some of which persist, accentuate, or change during the recovery phase. The findings in siblings could be related either to streptococcal infection or to a familial immunoregulatory aberration.

Adolescent↗
PubMed2000

[Rheumatic fever--a review of cases].

OBJECTIVE: To analyse clinical presentation of rheumatic fever (RF), with special emphasis on cardiac involvement, electrocardiographic and echocardiographic findings and the outcome of the cases referred to Maria Pia Children's Hospital from January 1990 to September 1999. METHODS: We retrospectively analyzed the clinical files of all cases referred to pediatric cardiology clinics with the suspicion of acute RF (Group 1) or with rheumatic valvular disease and heart failure (Group 2). In group 1 we studied the following: age and sex distribution, year of diagnosis, presence of Jones criteria treatment and outcome. In group 2 we analysed provenance, age of initial onset of RF, age of cardiology referral, treatment and outcome. RESULTS: Thirteen cases were identified, 8 in groups 1 and 5 in group 2. Group 1 included 3 girls and 2 boys, mean age of 10 years. The diagnosis of RF was based in the presence of 2 major and 1 minor manifestation (4/8), 1 major and 2 minor manifestations (1/8) and chorea in 3 cases associated with clinical carditis in one and subclinical carditis in another. Colour Doppler echocardiography showed pathological mitral regurgitation jet in 6 cases, associated with aortic regurgitation in 2 and dilatation of left ventricle in 3. All were treated with penicillin associated with anti-inflammatory drugs in 5 and haloperidol in 3. Group 2 included 3 girls and 2 boys, mean age 9.56 years. Four were from African countries (Angola and Guinea), and one came from the north of Portugal. The elapsed time between the initial acute attack and cardiology referral varied from 5 months to 3 years. All presented severe mitral insufficiency associated with aortic and/or tricuspid valve lesions, and heart failure. All five underwent valve surgery. The secondary prophylaxis was recommended in every patient. There was a recurrence in a child who had interrupted chemoprophylaxis. The patients from African countries were lost for follow-up. CONCLUSIONS: RF still remains a problem in present times, with serious cardiac sequela in African countries. Colour Doppler echocardiography is a valuable tool for the detection of pathological valvular regurgitation and subclinical carditis if strict criteria are used. The need for appropriate treatment of streptococcal pharyngitis and secondary prophylaxis is emphasized.

Adolescent↗
PubMed2001

Left ventricular mechanics during and after acute rheumatic fever: contractile dysfunction is closely related to valve regurgitation.

OBJECTIVES: The purpose of this study was to characterize left ventricular (LV) mechanics during acute rheumatic fever (ARF) and to define factors influencing remodeling after the acute event. BACKGROUND: Acute rheumatic fever is associated with varying degrees of valvulitis and myocarditis, but the impact of these factors on LV mechanics is poorly defined. METHODS: Echocardiograms and clinical data were reviewed in 55 patients aged 11.2 +/- 2.6 years during ARF. Valve regurgitation was absent or mild in 33 (group I) and moderate or severe in 22 (group II). Forty-two children (75%) underwent a further examination after ARF. RESULTS: Group I patients demonstrated a mildly elevated LV size during ARF and had normal indexes at follow-up. Group II patients demonstrated a markedly elevated LV size (end-diastolic dimension z-score 3.6 +/- 1.8, p < 0.01 compared with the normal population) and decreased shortening fraction (z-score -0.8 +/- 1.4, p < 0.05). The stress-velocity index, a z-score describing the velocity of shortening-afterload relationship, was normal in group II patients with mitral regurgitation (-0.2 +/- 1.2, p = NS) but was depressed in those with aortic regurgitation or both (- 1.4 +/- 1.4, p < 0.01). At follow-up the stress-velocity index remained depressed (-1.2 +/- 1.0, p < 0.01) and had deteriorated in those treated nonsurgically compared with those treated surgically (interval change nonsurgical -0.7 +/- 1.2 vs. surgical 1.3 +/- 1.3, p = 0.005). CONCLUSIONS: The evolution of contractile dysfunction during and after ARF is dependent on the degree and type of valve regurgitation and may be influenced by surgical intervention. These findings suggest that mechanical factors are the most important contributors to myocardial damage during and after ARF.

Adolescent↗
PubMed1993

Rheumatic fever: atypical presentation in an adult.

A 38 year old Caucasian lady presented with a history of vague ill health, raised ESR and prolonged P-R interval on ECG. These features became normal within a week. Contrary to the usual presentation, this patient developed fleeting arthritis, one of the major diagnostic features of rheumatic fever, towards the end of her illness. It is important to recognise such variants of rheumatic fever, even in retrospect, for the sake of giving penicillin prophylaxis to prevent cardiac morbidity.

Adult↗
PubMed1987

Study of antibody response to 4 streptococcal antigens in rheumatic fever and Kawasaki disease with or without cardiovascular lesions.

Patients with rheumatic fever (RF) and Kawasaki disease (MCLS) were divided into two groups, one with and the other without cardiovascular lesions, and their sera were examined for antibodies to 4 streptococcal antigens: anti-streptococcal polysaccharide (ASP), anti-deoxyribonuclease B (ADN-B), anti-streptolysin O (ASO) and anti-streptokinase (ASK) in order to clarify the relationship between group A streptococci and these two diseases. In RF the frequency of positive levels of ASP at its early stage was lower than those of the other 3 antibodies, but the ASP titer tended to persist at elevated levels. The frequency of ADN-B in patients with rheumatic heart disease (RHD) was higher than that in those without RHD, but with regard to the other antibodies no difference was found between the two. Of the 4 antibodies the frequency of at least one positive level was 100% in all the sera of RF patients within 3 months from onset. The differences in the frequencies of positive levels of all 4 antibodies in both MCLS and the controls did not prove to be significant. The frequencies of ASP, ADN-B and ASO in the sera of patients without cardiovascular lesions tended to be slightly higher than those in patients with cardiovascular lesions.

Adolescent↗
PubMed2000

Prophylactic efficiency of 3-weekly benzathine penicillin G in rheumatic fever.

Benzathine penicillin G (BPG) is effective for secondary prophylaxis of rheumatic fever (RF). However, interval between injections a remains a controversial matter. In a study population of 74 patients, following the initial diagnosis of RF, 3-weekly BPG (1.2 million units) regimen was started. During the first three-week period, serum penicillin concentrations were examined on the 7th, 14th and 21st days and throat culture done for group-A b hemolytic streptococcal (GABHS) infection. Ten patients (13.5%) at 21st day of injection had low serum penicillin concentration after the first BPG. GABHS was isolated in 5 patients during this period. Although two of these 5 patients had symptoms of respiratory tract infection, according to laboratory data, the other three were accepted as carriers. All 74 patients were then followed-up for rheumatic recurrence (RR) during long-term period (6 to 60 months, mean 25 +/- 5 months). There was no RR among regular (missing no more than one injection a year) group. We concluded that 3-weekly BPG regimen was satisfactory for secondary prophylaxis in RF, even though serum penicillin level was inadequate during the third week in some of the patients.

Adolescent↗
PubMed1993

Acute rheumatic fever in Saudi Arabia: mild pattern of initial attack.

Fifty-one children with the initial attack of acute rheumatic fever (ARF) were studied prospectively to verify the sociodemographic and clinical profile and to compare results with those from other countries. Most children belonged to large families who lived in an urban setting with ready access to medical care. Unlike reports from many developing countries, the clinical manifestations in this study paralleled data from the West and included arthritis in 76% of the cases, carditis in 43%, and chorea in 8%. Among the 22 cases with carditis, 18 had mitral regurgitation, three developed combined mitral and aortic regurgitation, and one had aortic regurgitation. This study demonstrates the mild nature of ARF in Saudi Arabia and supports the concept that climate and geography appear to bear little relationship to the incidence and severity of ARF.

Adolescent↗
PubMed2001

Childhood acute rheumatic fever in Greece: experience of the past 18 years.

UNLABELLED: This study reports on the authors' experience with acute rheumatic fever (ARF) during the years 1980-1997. The objectives were to estimate the incidence of the disease an area of Greece to characterize its epidemiology, to determine the frequency of the antecedent symptoms and to describe its clinical presentation. The medical records of 66 confirmed cases admitted to the First Department of Pediatrics, "Aghia Sophia" Children's Hospital, were reviewed. Two outbreaks occurred during this period. In contrast to the 3-4 cases seen every year, 14 cases were diagnosed during the 6 mo period from October 1989 to March 1990. An additional 10 cases were diagnosed in 1993. Most of the children (76%) were between 8 and 14 y old. The children were predominantly from middle-class families with ready access to medical care. Carditis, evident by auscultation, and arthritis were the dominant major manifestations in 70% and 68% of the cases, respectively. Mild carditis was present in 54% of children with valvular disease. CONCLUSION: ARF exists in the paediatric Greek population with exacerbations and remissions, but the cardiac manifestations appear mild or moderate.

Adolescent↗
PubMed1985

Salicylate-induced hepatitis in rheumatic fever.

In a 4 1/2-year retrospective study of hospitalized patients with rheumatic fever treated with salicylates, we determined that five of 34 children manifested salicylate-induced hepatitis. The average serum salicylate level in those patients with hepatotoxicity was 30.9 mg/dL, while the average serum salicylate level in the rest of the patients was 19.7 mg/dL. An awareness of this potential complication is important when treating children with salicylates at doses previously considered to be nontoxic. Serum salicylate levels should be maintained at approximately 15 to 25 mg/dL during such therapy.

Adolescent↗
PubMed2003

Effects of IgM from rheumatic fever patients on intracellular calcium levels of neonatal rat cardiac myocytes.

Rheumatic fever (RF), a potential sequela of Streptococcus pyogenes pharyngitis, sometimes results in myocarditis and heart failure. Antibodies have been implicated in the pathogenesis of RF and anti-cardiac myosin antibody levels are elevated in RF patients. Since myocarditis is associated with altered cardiomyocyte calcium transients it was of interest to determine the direct effects of RF patient antibodies on calcium transients in cultured myocytes. RF patient polyclonal IgM treatment caused increased calcium retention by neonatal rat heart cells in vitro as determined with isotopically labeled calcium. Therefore, to further characterize this finding, calcium transients were evaluated by real time fluorescence spectroscopy and deconvolution imaging. RF patient polyclonal IgM produced increased calcium retention during the relaxation stage of the contraction cycle leading to a slowing of contraction rate, disorganized calcium transients, and eventual tetany. In contrast, calcium transient studies of cardiomyocytes following treatment with monoclonal anti-myosin antibodies revealed declining intracellular calcium levels, accompanied by disorganized transients and tetany. Treatment with both antibodies led to myocyte dysfunction and these novel findings suggest a role for antibodies in the pathogenesis of the myocarditis associated with rheumatic carditis.

Animals↗
PubMed1979

Epidemiology of rheumatic fever in Japan.

The number of patients with the first attack of rheumatic fever reached a peak in 1960, decreased remarkably until 1968, then has remained unchanged for the last nine years. Approximately 10% of rheumatic patients still develop recurrences.

Child↗
PubMed1995

Scleritis, uveitis, and glaucoma in a patient with rheumatic fever.

PURPOSE: To determine the origin of scleritis, uveitis, and glaucoma in a boy with fever, sore throat, joint pains, and malaise. METHODS: The patient underwent extensive hematologic, serologic, rheumatologic, and radiologic examinations. RESULTS: The patient recovered after extensive medical therapy. He met the Jones criteria for the diagnosis of acute rheumatic fever and had an increased result for antistreptolysin O antibody titer. CONCLUSIONS: Antistreptococcal antibody testing is helpful in diagnosing rheumatic fever as a cause of scleritis, uveitis, and glaucoma.

Acute Disease↗
PubMed2001

Heart-directed autoimmunity: the case of rheumatic fever.

Molecular mimicry was proposed as a potential mechanism for streptococcal sequelae leading to rheumatic fever (RF) and rheumatic heart disease (RHD). CD4(+)infiltrating T cells are able to recognize streptococcal M peptides and heart tissue proteins. We analyzed the M5 peptide- and heart-specific responses, cytokine profile and T cell receptor (TCR) BV usage from peripheral and heart-infiltrating T cell lines and clones from patients across the clinical spectrum of ARF/RHD. The patient with ARF displayed a higher frequency of mitral valve infiltrating T cell clones reactive against M5: 1-25, 81-103 and 163-177 regions and several valve-derived proteins than the post-RF and chronic RHD patient (67%; 20% and 27%, respectively). The presence of oligoclonal BV families indicative of oligoclonal T cell expansion among mitral valve-derived T cell lines was increased in the chronic RHD patient. Furthermore, mitral valve T cell lines from all patients produced significant amounts of inflammatory cytokines interferon-gamma (IFN-gamma) and tumour necrosis factor-alpha (TNFalpha) in response to M5(81-96) peptide, with the highest production attained by the chronic RHD patient. These data are consistent with an important role for M5 peptide and host antigen-driven, T1-type CD4(+)T cells in the pathogenesis of RHD and heart lesion progression after recurrence of the streptococcal infection.

Adolescent↗
PubMed1985

Acute rheumatic fever in Swedish children 1971-80.

Hospital records from all Swedish children 0-15 years old diagnosed as having acute rheumatic fever (ARF) during 1971-80 were studied. Thirty-one children fully met with Jones' modified criterias which gives an incidence of 0.2 cases per 100 000 children and year. Carditis was the most common major manifestation of ARF. In most children the carditis was mild but in three cases there was a persistent cardial affection. 16 of the children received prophylactic antibiotic treatment. No recurrent attacks of ARF were found during the study period.

Acute Disease↗