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A case of acute rheumatic fever accompanied by transient aortic regurgitation.

Though acute rheumatic fever (RF) is now rare in Japan, it continues to be an important disease condition that physicians should be prepared to diagnose and treat. We describe a patient with acute RF accompanied by transient aortic regurgitation (AR). The AR was detected only by echocardiography. There were no other indications, and it disappeared after treatment with prednisolone. The changes in cardiac valves in the early phase of RF have been the subject of only a few case studies. Echocardiography is quite valuable in the workup of patients with acute RF and should be performed even if there are no signs of cardiac involvement.

Acute Disease↗

Renal lesions in acute rheumatic fever.

In the past 4 years, four patients with acute rheumatic fever and abnormalities of renal function, urinary sediment, or both have undergone percutaneous renal biopsy at this institution. Distinctly different renal lesions were found and included focal glomerulonephritis, classic acute exudative poststreptococcal glomerulonephritis, mesangioproliferative glomerulonephritis, and severe interstitial nephritis. In each case, the clinical abnormalities were transient. In this report we describe the clinical and pathologic findings in each of these patients to stress the variability of renal lesions that may accompany acute rheumatic fever.

Acute Disease↗

Incidence and diagnosis of acute rheumatic fever in Denmark, 1980 and 1983. A retrospective analysis of the fulfillment of the revised Jones criteria in hospitalized patients.

A review of 1547 official hospital record summaries concerning discharges during the period 1980 through 1983 of patients whose diagnoses had been coded as acute rheumatic fever revealed that in only 61% of the cases had this illness been diagnosed or suspected. A substantial proportion of the remaining patients had had acute non-rheumatic pericarditis diagnosed. The medical records were analyzed for 141 patients diagnosed in 1980 or 1983 by hospital departments as having acute rheumatic fever with regard to the revised Jones criteria. They were fulfilled in 47 patients, 23 of whom were considered unlikely cases of rheumatic fever. Eight patients were considered possible cases, although they did not fulfill the revised Jones criteria. The current annual incidence of acute rheumatic fever was estimated to be at most 0.3 per 100,000 inhabitants.

Acute Disease↗

Advanced atrioventricular conduction block in acute rheumatic fever.

We carried out a retrospective case control analysis to evaluate the outcome, and the need for treatment, of problems with atrioventricular conduction occurring during an acute attack of rheumatic fever, assessing the occurrence of second and third atrioventricular block versus first degree block. We reviewed and analysed the clinical, electrocardiographic and echocardiographic records of all children diagnosed in a single institute as having acute rheumatic fever during a period of seven consecutive years. During the period from October, 1994, through October, 2001, 65 children meeting the modified Jones criterions for acute rheumatic fever were hospitalized in the Soroka University Medical Center, Israel. First-degree atrioventricular block was identified in 72.3% of the children, and resolved with no specific treatment other than non-steroidal anti-inflammatory medications. Second-degree atrioventricular block of Mobitz type I, was observed in one child (1.5%), which progressed from first-degree block, and subsequently resolved. Complete atrioventricuar block was found in 3 children (4.6%), one progressing from Mobitz type I second-degree block, and two being seen as the first presentation. Of the three children with complete atrioventricular block, one patient was not treated, the second was treated with aspirin, and the final one with combined aspirin and steroids. The disturbances of conduction resolved in all three. We conclude that advanced atrioventricular block is rare during acute rheumatic fever. If occurring, block appears to be temporary, and resolves with conventional anti-inflammatory treatment. Specific treatment, such as insertion of a temporary pacemaker, should be considered only when syncope or clinical symptoms persist.

Acute Disease↗

The fall and rise of rheumatic fever in the United States: a commentary.

After a documented decline in the incidence of acute rheumatic fever in the United States during the past three decades, an apparent resurgence has occurred in the mid-1980s. Although standards of living have continued to improve with concomitant decrease in crowding and easier accessibility to medical care, the precise reasons for the decline remain unexplained. Furthermore, the decline has occurred even though there is no epidemiologic evidence to suggest any reduction in the incidence of group A streptococcal pharyngitis. Just as the decline remains unexplained, so also does the "resurgence". Of considerable interest are the facts that the preceding pharyngitis has been mild in the majority of cases, the incidence of documented carditis has been high (over 90% in one series), and the rheumatic fever has been concentrated in middle class families with ready access to medical care. Even more intriguing has been the appearance of very mucoid strains of group A streptococci at the same time. While this simultaneous appearance suggests "rheumatogenicity", this has not been substantiated; no "rheumatogenic factor" has yet been isolated from these strains. This outbreak, although small in comparison with the number of cases occurring in many of the developing countries of the world, has important implications for those countries. Unless and until the pathogenesis of rheumatic fever is fully understood, methods of control will not be optimal.

Cross-Sectional Studies↗

Detection of C-reactive protein, streptolysin O, and anti-streptolysin O antibodies in immune complexes isolated from the sera of patients with acute rheumatic fever.

Circulating immune complexes (IC) of 42 patients with acute rheumatic fever from Santiago, Chile, were studied. The complexes were isolated by polyethylene glycol precipitation and were analyzed for antibodies, antigens, and C-reactive protein. We found the complexes to be enriched in antibody to streptolysin O, particularly in the group of patients with elevated levels of IC. IgM was the predominant class of Ig present in the complexes. Western blots from 12 patients to detect antigens in the complexes showed proteins of m.w. 50,000, 60,000, and 69,000, consistent with the polypeptides of streptolysin O. Such antigens were absent in the complexes from patients with post-streptococcal glomerulonephritis and pharyngitis. Eluted antibodies from these protein bands on the nitrocellulose sheets reacted with the streptolysin O in Western blots and neutralized the hemolytic activity of streptolysin O in a microhemolysin assay. In addition, isolated complexes from several sera showed the presence of C-reactive protein bound to complexes. In vitro experiments demonstrated that [125I]C-reactive protein was not precipitated by polyethylene glycol either alone or when added to monomeric IgG, whereas it precipitated significantly when added to aggregated IgG. The detectable C-reactive protein in isolated complexes and sera samples increased after treatment with sodium dodecyl sulfate. These data suggest that circulating immune complexes in acute rheumatic fever contain streptolysin O and its antibody and raise interesting questions regarding the pathogenetic significance of C-reactive protein in the complexes.

Antigen-Antibody Complex↗

Antibody levels and in vitro lymphoproliferative responses to Streptococcus pyogenes erythrogenic toxin A and mitogen of patients with rheumatic fever.

We investigated the in vitro lymphoproliferative responses to a streptococcal mitogen and erythrogenic toxin A of children with acute rheumatic fever (ARF) and patients with chronic rheumatic heart disease (CRHD). Antibody levels to the streptococcal products were also analyzed in the sera of those with ARF or chronic rheumatic heart disease as well as in the sera of children with streptococcal pharyngitis or poststreptococcal glomerulonephritis. Our results demonstrated that the individuals had depressed lymphoproliferative responses during the active stage of rheumatic fever. The depressed responses were not found either to be induced by time-sensitive mitogen-specific suppressor cells or to be related to a dose-response phenomenon. On the other hand, antibody levels to the extracellular mitogens were significantly elevated in the sera of children with ARF compared with the levels in the rest of the groups. The hyperresponsiveness noted among children with ARF was found to be at a quantitative level and was not due to recognition of more epitopes, as determined by Western blotting (immunoblotting). The profile of immune responsiveness in children with ARF to the streptococcal extracellular mitogens is discussed in relation to the pathogenesis of disease.

Adolescent↗

Acute rheumatic fever in Los Angeles County.

There was a general downward trend in the reported incidence of acute rheumatic fever in Los Angeles County during the years 1954-1963. A survey of hospital records in five large hospitals in 1962 revealed 100 cases diagnosed, 39 of which were reported. Diagnoses in the charts reviewed conformed to the Modified Jones Criteria. Most of the patients were born in Los Angeles County. Mortality rates for acute rheumatic fever during the same period were greatly in excess of those expected from the reported morbidity. The mean crude mortality rate for the period concerned was higher than for New York City, although not as high as for Boston.Acute rheumatic fever appears to constitute a health problem in need of review in Los Angeles County.

Adolescent↗

[Therapy of rheumatic fever].

After making the diagnosis the rheumatic fever as a secondary disease after streptococcal infection is immediately to be treated with penicillin G. Parallel to this or overlapping the rheumatic inflammatory mesenchymal tissue reactions are treated with prednisolone or derivatives, aminophenazone or salicylic acid preparations, in which cases in children prednisolone and aminophenazone are preferred. The combat against the streptococcal infection by means of penicillin transgresses into a recidivation prophylaxis with benzathin-penicillin, which is to be performed up to an age of 5 years. Substitutes for the recidivation treatment with benzathin-penicillin are V-tablopen or erythromycin, perhaps also sulfaclomide. The treatment is to be performed in a hospital up to the time that the patient is free from signs of inflammation and at least 4 weeks without fever, then a gradual rehabilitation begins.

Adult↗

Adherence of group A streptococci to pharyngeal cells: a role in the pathogenesis of rheumatic fever.

We used an assay in vitro to investigate the possible role of streptococcal adherence to human pharyngeal cells in the pathogenesis of acute rheumatic fever. There was no difference in adherence of rheumatic fever-associated and non-associated strains of group A streptococci to pooled pharyngeal cells of normal people. Likewise, streptococci not associated with rheumatic fever adhered equally well to cells taken from normal people and from patients with rheumatic heart disease. However, the pharyngeal cells of all nine rheumatic heart disease patients tested had increased avidity for adherence for a rheumatic fever-associated strain of streptococcus compared to the pharyngeal cells obtained from age- and sex-matched controls. Increased streptococcal adherence to pharyngeal cells of rheumatic fever-prone patients may play a role in the pathogenesis of rheumatic fever.

Adult↗

Acute rheumatic fever: a chink in the chain that links the heart to the throat?

Acute rheumatic fever (ARF) remains a major problem in tropical regions, resource-poor countries, and minority indigenous communities. It has long been thought that group A streptococcal (GAS) pharyngitis alone was responsible for acute rheumatic fever; this belief has been supported by laboratory and epidemiological evidence gathered over more than 60 years, mainly in temperate climates where GAS skin infection is uncommon. GAS strains have been characterised as either rheumatogenic or nephritogenic based on phenotypic and genotypic properties. Primary prevention strategies and vaccine development have long been based on these concepts. The epidemiology of ARF in Aboriginal communities of central and northern Australia challenges this view with reported rates of ARF and rheumatic heart disease (RHD) that are among the highest in the world. GAS throat colonisation is uncommon, however, and symptomatic GAS pharyngitis is rare; pyoderma is the major manifestation of GAS infection. Typical rheumatogenic strains do not occur. Moreover, group C and G streptococci have been shown to exchange key virulence determinants with GAS and are more commonly isolated from the throats of Aboriginal children. We suggest that GAS pyoderma and/or non-GAS infections are driving forces behind ARF in these communities and other high-incidence settings. The question needs to be resolved as a matter of urgency because current approaches to controlling ARF/RHD in Aboriginal communities have clearly been ineffective. New understanding of the pathogenesis of ARF would have an immediate effect on primary prevention strategies and vaccine development.

Acute Disease↗

Penicillin and the marked decrease in morbidity and mortality from rheumatic fever in the United States.

There has been a marked decline in mortality due to rheumatic fever in the United States. We present evidence for the important role of penicillin in changing the severity of rheumatic carditis, beginning about 1946. Since that year, mortality due to rheumatic carditis has rapidly decreased to zero at the hospital we studied (House of the Good Samaritan, Boston), the rate of loss of all murmurs in patients at the study hospital accelerated simultaneously and exceeded 40 percent by 1970, and the rates of decline in national mortality due to rheumatic carditis accelerated fourfold with the advent of antibiotics. These data, together with reports of recent outbreaks of rheumatic fever, emphasize the importance of continued efforts to diagnose and treat Group A streptococcal pharyngitis.

Adolescent↗

[Acute rheumatic fever: problems and outlooks].

The issue related with acute rheumatic fever still remains to be topical at the present stage, which is accentuated by a high prevalence of rheumatic heart diseases. The results of multiple studies point out at the presence of "rheumatogenetic" A-streptococcal strains possessing certain biological properties. Although there were no changes in the disease semiotics, the intensity degree of clinical signs went down, due to which an early diagnosis of the disease became more complicated. The issues related with the nosological classification of post-streptococcal reactive arthritis and with PANDAS syndrome need to be solved. There is also an urgent necessity in creating high-technological domestic benzathine-penicillins intended for secondary prevention of the disease.

Acute Disease↗

[Activity of lipid peroxidation processes in children with rheumatic fever].

Pathogenic mechanism of acute and chronic inflammation is connected to the increased production of superoxide anion, hydrogen peroxide, and other free radicals. Clinical role of lipid peroxidation (LPO) processes was studied in 38 patients in the age from 3 to 15 years old with different variants of Rheumatic Fever (RF). We have investigated the relationship between malonidialdehide (MDA) and RF. We measured the levels of MDA in the plasma in patients with acute rheumatic fever (ARF) and chronic rheumatic heart disease (CRHD). Our study revealed that the levels of MDA in patients with ARF are significantly higher than in patients with CRHD. These levels were also significantly higher in patients with history of disease for up to 2 years, than in patients with history longer than 2 years. Thus, the measurement of MDA in the plasma could be used as a laboratory test for relation of active state of rheumatic fever.

Adolescent↗

[Gallium-67 in rheumatic fever: preliminary report].

PURPOSE: To validate the importance of 67Ga imaging to diagnose activity in rheumatic fever carditis and in the follow-up of treated patients with the disease. PATIENTS AND METHODS: Thirty patients with rheumatic fever were studied. Fifteen males and 15 females aged 7 to 39 years old (median 16 years), were submitted to 67Ga imaging. The patients were divided into two groups: GA--20 patients who received only clinical treatment and GB--11 patients (one from group A) submitted to surgical biopsy. In 12 patients from GA, sequential 67Ga imaging were also realized for the therapeutic follow-up. Fifty-five 67Ga images were reviewed subjectively by experienced observers and a semi-quantitative analysis processed in a dedicated computer was performed with ROI's of 10 x 20 pixels over the projection of the heart and right lung in 22 negative and 33 positive studies. RESULTS: Group A--In 19 patients (95%) the 67Ga uptake matched with the clinical evidence, EGC and echocardiogram results. In one patient (5.0%) the 67Ga study was negative, but there were clinical and EKG evidence of activity of the disease; from these data this case was then considered as false negative. Group B--In 11 patients submitted to surgical biopsy, the clinical-scintigraphic-histologic correlation matched in 10 cases and mismatched in one. The semi-quantitative analysis of the 67Ga uptake with ROI's over the topography of the heart and right lung showed an percentage index heart x right lung of 114.5 +/- 17.5% (2 s.d.) in normals and 158 +/- 30% (2 s.d.) in patients with active carditis: these data had value in the therapeutic follow-up of 12 patients from GA, where occurred good correlation, in every case, of the 67Ga uptake index and clinical evolution and EKG findings. CONCLUSION: The 67Ga imaging is a non invasive diagnostic method with a good sensitivity to the diagnosis of activity in rheumatic carditis, to evaluate the therapeutic results and to define inactivity for surgical cases.

Adolescent↗

[Changes of serum soluble tumor necrosis factor receptor I in patients with rheumatic fever].

OBJECTIVE: To study the changes and the effects of soluble tumor necrosis factor receptor I (sT-NFR I) in patients with rheumatic fever, and to find out the laboratorial marker of clinic activity in patients with rheumatic fever. METHODS: The serum concentrations of sTNFR I in 16 patients with ARF, 16 patients with chronic inactive rheumatic heart disease (CRHD) and 15 healthy controls were measured by methods of ELISA and the relationship between sTNFR I and ESR or CRP were studied. RESULTS: The sTNFR I serum concentrations in patients with ARF (3.92 +/- 1.28) ng.ml-1 were significantly higher than those with CRHD (1.43 +/- 0.66) ng.ml-1 and healthy controls (1.06 +/- 0.42) ng.ml-1 (all P < 0.01). The serum concentrations of sTNFR I in patients with CRHD were almost the same with those in healthy controls (P > 0.05); Their positive correlation was significantly observed between sTNFR I and CRP(P < 0.001), and wasn't observed between sTNFR I and ESR. CONCLUSION: The Patients with ARF have high serum levels of sTNFR I. It may be used as the laboratorial marker in the clinic assessment of activity in patients with rheumatic fever.

Adolescent↗