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PubMed1998

Advanced atrioventricular block in a 39-year-old man with acute rheumatic fever.

This report describes the development of advanced (2:1 and 3:1) AV block in a 39-year-old man with acute rheumatic fever. AV block progressed from first-degree to type I second-degree and finally to advanced AV block. The latter lasted 5 days. AV block regressed in a stepwise fashion via sustained type I second-degree AV block and eventually first-degree AV block. The PR interval returned to normal several weeks after resolution of second-degree AV block. In view of the resurgence of acute rheumatic fever, the diagnosis of acute rheumatic fever should now be considered in young adults or middle-aged patients presenting with second-degree or third-degree AV block of undetermined etiology.

Acute Disease↗
PubMed1992

Occurrence of valvar heart disease in acute rheumatic fever without evident carditis: colour-flow Doppler identification.

OBJECTIVE: To determine the frequency of occurrence of mitral and aortic valvar regurgitation in rheumatic children in whom there was no evidence of carditis acutely or at an earlier attack. DESIGN: Colour flow Doppler imaging was used in a non-randomised study of sequentially admitted children who met the criteria for acute rheumatic fever without clinically evident carditis and patients in whom the disease was quiescent after a previous attack of rheumatic fever. Two separate control groups were used for comparison of the echocardiographic findings, and a group of patients with confirmed rheumatic carditis was included for comparison of acute phase and antistreptococcal reactants. SETTING: A general hospital with the only paediatric inpatient department in Qatar. PATIENTS: From November 1988 to October 1990, 11 children were studied during the acute rheumatic period. In seven additional children the disease was quiescent when they were studied 18 to 36 months after a documented episode of acute rheumatic fever in which there was no evidence of carditis. The control patients were all studied during the same period. MAIN OUTCOME MEASURE: Detection of mitral and aortic regurgitation in patients without clinical evidence of rheumatic carditis in the acute or quiescent stages of the disease. RESULTS: Mitral or mitral and aortic regurgitation was found in 10 of the 11 children studied in the acute rheumatic period. None had a murmur or other evidence of carditis. In all the cases studied the valvar insufficiency was mild. Four of the children studied late in the quiescent period had either aortic or mitral insufficiency by colour flow Doppler evaluation; two children who had previously had valvar insufficiency no longer showed this, and one child without positive findings in the acute phase remained without insufficiency. None of the non-rheumatic control subjects showed mitral or aortic regurgitation. CONCLUSIONS: Colour flow Doppler imaging is a useful method of identifying subclinical mitral and aortic valvar disease at all stages of rheumatic fever when carditis cannot be otherwise detected and is a valuable addition to current diagnostic criteria.

Acute Disease↗
PubMed1985

Studies of streptococcal membrane antigen--binding cells in acute rheumatic fever.

Blood specimens from patients with rheumatic heart disease in both India and New Mexico were typed for the presence of B cell alloantigen 883 by use of a mouse monoclonal antibody with identical specificity to the original 883 human alloantiserum. Strong relative segregation was recorded for 883 positive B cell typing in patients with rheumatic heart disease in both geographic locations as compared with that in normal unaffected controls. In patients with acute rheumatic fever, studies of actual B-lymphocyte membrane binding by anti-883 monoclonal antibody and sonicated group A streptococcal membrane antigens showed separate but contiguous localization on isolated cell surfaces. Although physically distinct, 883 B cell alloantigen and sonicated group A streptococcal membrane antigens moved together in cell capping studies after incubation at 37 degrees C. These findings reaffirm the apparent close association between 883 B cell alloantigen and rheumatic heart disease. They also demonstrate that the B cell alloantigen 883 itself is physically distinct from but very close to sites on antigen-reactive B cells actually binding to group A streptococcal membrane antigens.

Acute Disease↗
PubMed1992

Anticardiolipin antibodies in acute rheumatic fever.

Recent reports describe the association of antiphospholipid antibodies (aPL) with chorea or severe heart valve lesions in systemic lupus erythematosus, lupus-like disease, or the primary antiphospholipid antibody syndrome. We conducted a case series and a case-control investigation of patients with rheumatic fever with Sydenham chorea or other manifestations of rheumatic fever for anticardiolipin antibodies (aCL) during the acute attack and disease remission. Eighty percent of patients were positive for aCL during the rheumatic fever attack vs 40% when inactive (p = 0.035); IgG and IgM aCL increased significantly with disease activity. Individuals with or without Sydenham chorea were equally positive for aCL (76 and 83%, respectively). A significant association was found between IgM aCL and carditis: All patients with valvulitis had IgM aCL (100%) vs 37% of patients without valvular involvement (p = 0.02). aPL may play a role in the pathogenesis of some clinical manifestations of acute rheumatic fever.

Acute Disease↗
PubMed1975

New Zealand trends in rheumatic fever: 1885-1971.

National statistics of rheumatic fever in New Zealand show falling mortality over the past 30 years. However, morbidity remains high, especially among Maori children. Community-based studies are under way to elucidate this important public health problem.

Adolescent↗
PubMed2002

Molecular analysis of group A Streptococcus type emm18 isolates temporally associated with acute rheumatic fever outbreaks in Salt Lake City, Utah.

Acute rheumatic fever (ARF) and subsequent rheumatic heart disease are rare but serious sequelae of group A Streptococcus (GAS) infections in most western countries. Salt Lake City (SLC), Utah, and the surrounding intermountain region experienced a resurgence of ARF in 1985 which has persisted. The largest numbers of cases were encountered in 1985-1986 and in 1997-1998. Organisms with a mucoid colony phenotype when grown on blood agar plates were temporally associated with the higher incidence of ARF. To develop an understanding of the molecular population genetic structure of GAS strains associated with ARF in the SLC region, 964 mucoid and nonmucoid pharyngeal isolates recovered in SLC from 1984 to 1999 were studied by sequencing the emm gene. Isolates with an emm18 allele were further characterized by sequencing the spa, covR, and covS genes. Peak periods of ARF were associated with GAS isolates possessing an emm18 allele encoding the protein found in serotype M18 isolates. Among the serotype M18 isolates, the difference in the number of C repeats produced three size variants. Variation was limited in spa, a gene that encodes a streptococcal protective antigen, and covR and covS, genes that encode a two-component regulatory system that, when inactivated, results in a mucoid phenotype and enhanced virulence in mouse infection models. Pulsed-field gel electrophoresis showed a single restriction profile for serotype M18 organisms isolated during both peak periods of ARF. In SLC, the incidence of ARF coresurged with the occurrence of GAS serotype M18 isolates that have very restricted genetic variation.

Acute Disease↗
PubMed1984

Acute rheumatic fever below the age of five years: a prospective study of the clinical profile.

The clinical profile of acute rheumatic fever below the age of five years was studied prospectively in 53 children seen over a period of seven years. The majority presented with pain in the joints, manifested as arthritis in 81% and arthralgia in 15%. Four per cent presented with congestive heart failure. Carditis developed in 42%, with a high incidence of pericarditis (6%) and congestive heart failure (15%) and a mortality of 2%. The incidence of Erythema marginatum was 2%, while chorea and subcutaneous nodules were not seen. Data from this prospective study, when compared with those of other prospective studies of acute rheumatic fever throughout childhood, clearly show a similar incidence of arthritis and carditis with a slightly more aggressive nature of carditis in children under five years. These findings are in marked contrast to recent reports describing the clinical profile of acute rheumatic fever in this age group.

Acute Disease↗
PubMed1991

[Rheumatic fever, the idea of progress and the circularity of time].

Rheumatic fever, once virtually "gone" from the western world, has made a come-back in the mid nineteen-eighties. This is disturbing because the control of rheumatic fever was a source of pride to the medical community. Changes in medical practice, such as a less compulsive attitude vis-a-vis streptococcal infections, and social changes, such as "house starvation", probably conspired to bring about the come-back. It's likely that the emergence of more virulent and more rheumatogenic streptococcal strains was facilitated by these changes. Physicians should once again be alert to the streptococcal threat, and accurately diagnose and effectively treat streptococcal pharyngitis.

Adult↗
PubMed1993

Association of HLA class I and class II antigens with rheumatic fever in a Turkish population.

The distribution of class I and class II HLA antigens of 100 Turkish patients with rheumatic fever, 77 of whom had cardiac involvement, was examined. We compared the results with a control group of identical origin. The frequency of HLA A10 and HLA B35 antigens were found significantly higher in patients with rheumatic fever (p < 0.05, p < 0.01, respectively). The frequency of HLA A10 and HLA DRw11 in patients with cardiac involvement were significantly higher than in those without cardiac involvement (p < 0.05, p < 0.01, respectively). On the other hand, HLA Cw2 antigen frequency was found significantly higher in patients without cardiac involvement than in those with rheumatic heart disease (p < 0.05). We support the concept that rheumatic fever is an immunological reaction to group A, beta hemolytic streptococci in individuals who have genetic predisposition.

Acute Disease↗
PubMed1991

Acute rheumatic fever in West Virginia. Not just a disease of children.

Rheumatic fever is a poststreptococcal disease that is receiving renewed attention by the medical community. We describe a recent increase in the number of observed cases of acute rheumatic fever (ARF) in West Virginia. This is the fifth report of a recent increase in the incidence of ARF in the Ohio Valley area in the last 4 years. In contrast to the other reports, nearly two thirds of our cases of ARF were in adults, more than half of whom had suffered previous bouts of ARF. In these adults with recurrences, none was taking prophylactic penicillin at the time of presentation. Carditis was present in seven adults, two without a history of carditis. Arthritis was present in all adult patients. These data indicate a possible geographic phenomenon related to the increased number of observed cases of ARF and document that ARF is not simply a disease of childhood. Furthermore, our findings highlight the need for extended penicillin prophylaxis for secondary prevention of ARF, especially for those with an increased risk of acquiring a streptococcal upper respiratory tract infection.

Acute Disease↗
PubMed2001

Resource utilization and cost of rheumatic fever.

OBJECTIVE: The socioeconomic effects of rheumatic fever (RF) in Brazil, including direct and indirect costs to patients and their families and to society, are largely unknown. We evaluated the utilization of resources and costs related to RF in a tertiary center caring for low income patients in the city of São Paulo, Brazil. METHODS: One hundred patients with RF, younger than 18 yrs, with followup of at least one year, were sequentially selected to provide complete information on a questionnaire. Additional data were collected from patients' charts. The utilization of resources was evaluated for each patient throughout the entire disease course. Costs were determined for patients and their families as well as for the society, using variables from 3 different systems: the national public health system, used by most lower income groups; the Brazilian Medical Association, which regulates charges and fees utilized by health plans and insurance companies; and costs charged by private practitioners, paid directly by patients. RESULTS: The RF population studied belonged to a low socioeconomic level. The mean monthly family income was $625.20 US. The mean disease duration was 3.9 yrs (range 1-10). Patients had a total of 1657 medical consultations, 22 hospital admissions, and 4 admissions to intensive care unit. Work absenteeism among parents was calculated as 22.9%, equivalent to 901 days of missed work; about 5% of the parents lost their jobs. Patients showed a high rate of school failure (22%). Considering the public system as a reference, direct, indirect, and total costs to society per 100 patients throughout the entire disease duration were $105,860 US ($271/patient/yr), $18,803 US ($48/patient/yr), and $124,663 US (US $319/patient/yr), respectively. When health care plan and private systems were taken as reference, the total costs were $423,550 US and $684,351 US, respectively. CONCLUSION: RF and rheumatic heart disease have an important socioeconomic impact in Brazil; costs of RF made up roughly 1.3% of annual family income. The estimated annual cost of RF for society in Brazil is $51,144,347.00 US.

Absenteeism↗
PubMed2006

Overlapping humoral autoimmunity links rheumatic fever and the antiphospholipid syndrome.

OBJECTIVE: Rheumatic fever (RF) and the antiphospholipid syndrome (APS) are autoimmune diseases that share similar cardiac and neurological pathologies. We assessed the presence of shared epitopes between M protein, N-acetyl-beta-D-glucosamine (GlcNAc) and beta2 glycoprotein-I (beta2GPI), the pathogenic molecules engaged in these autoimmune conditions. METHODS: Sera from the APS patients were affinity-purified on beta2GPI and beta2GPI-related peptide columns. Sera from RF patients were affinity-purified on protein G column. The beta2GPI and M protein-related peptides were prepared by conventional solid-phase peptide synthesis. The enzyme-linked immunosorbent assay direct binding and inhibition studies were performed on the RF and APS sera for the presence, and cross-reactivity, of antibodies against beta2GPI, beta2GPI-related peptides, streptococcal M protein, M-derived peptides and GlcNAc. RESULTS: Antibodies (Abs) to beta2GPI were found in 24.4% of 90 RF patients. Antibodies against various beta2GPI-related peptides were found in 1.1-36.7% of the patients. The immunoglobulin G sera from RF patients possessed significant anti-beta2GPI activity, while sera from APS patients contained a considerable anti-streptococcal M protein as well as anti-GlcNAc activity. Furthermore, affinity-purified anti-beta2GPI and anti-beta2GPI-related peptide Abs from APS patients cross-reacted with streptococcal M protein and M5 peptide, while beta2GPI and beta2GPI-related peptides inhibited anti-streptococcal M protein activity from RF patients. The results were confirmed by immunoblot analyses. The beta2GPI also inhibited anti-GlcNAc activity from APS patients with chorea. CONCLUSIONS: The results of our study, showing a considerable overlap of humoral immunity in RF and APS, support a hypothesis that common pathogenic mechanisms underlie the development of cardiac valve lesions and Central Nervous System abnormalities in both diseases.

Acetylglucosamine↗
PubMed1985

Ventricular tachycardia in acute rheumatic fever.

A 19-year-old man presented with fever, migratory polyarthritis, and palpitations; a diagnosis of acute rheumatic fever was made. Twenty-four-hour ambulatory cardiac monitoring (Holter) performed during the patient's hospitalization revealed ventricular tachycardia. Although disturbances in cardiac conduction and rhythm are common during the acute phase of rheumatic fever, malignant ventricular arrhythmias, to our knowledge, have not been described. Recognition of this occurrence emphasizes the need for continuous cardiac monitoring in selected patients.

Acute Disease↗
PubMed1975

Peripheral blood T and B lymphocytes during acute rheumatic fever.

Proportions and total numbers of thymus-derived (T) and bone marrow-derived (B) peripheral blood lymphocytes were studied in 53 patients with acute rheumatic fever, diagnosed on the basis of modifified Jones criteria. An elevation in both proportions and absolute numbers of cells bearing surface Ig was found in most patients, particularly during the first 7 days after onset. Conversely, T-cell proportions and numbers were often found to be depressed early in the acue phases of rheumatic fever. Proportions of cells bearing surface Ig did not correlate with another B-cell marker, the aggregated gamma globulin receptor, suggesting that such cells bearing surface Ig were not all B lymphocytes. Incuvation for 20 h at 37 per cent C of cells showing high proportions of surface Ig-bearing surface Ig in both normal and rheumatic fever subjects, although there was no appreciable increment in proportions of lymphocytes expressing T-cell markers. Patients with initial attacks showed higher percentages and total numbers of Ig-bearing lymphocytes (P smaller than 0.01) than did those with rneumatic fever recurrences. Elevations in numbers and proportions of peripheral blood lymphocytes bearing Ig appeared to correlate with the relative acute nature of the rheumatic fever attack.

Acute Disease↗
PubMed1995

Search for linkage disequilibrium between alleles in the T cell receptor alpha and beta chain loci and susceptibility to rheumatic fever.

A population-based linkage disequilibrium study was conducted to search for associations between alleles in T cell receptor alpha and beta chain polymorphic loci and susceptibility to rheumatic fever. The allele frequencies of four T cell receptor locus restriction fragment length polymorphisms were measured in 47 European and 51 Maori subjects with a history of rheumatic fever. These allele frequencies were compared to the allele frequencies in three or four independently recruited, race-matched control groups totalling 125 Europeans and 117 Maoris with no history of rheumatic fever. The polymorphisms studied were (locus/enzyme/probe) C alpha/Taq1/Y14, V alpha/Taq1/Y14, V beta 7/BAMHI/V beta 7.4 and V beta 8/BAMHI/V beta 8.1. There was no evidence for linkage disequilibrium between rheumatic fever and these Tcr alleles in either the Maori or European subjects.

Adult↗
PubMed1983

Acute rheumatic fever.

The classic dramatic clinical presentation of acute rheumatic fever (ARF), with high fever, severe carditis and acute polyarthritis, is changing to an asymptomatic, milder form. The cardiac damage potential of the disease remains high. It is the responsibility of the primary care nurse practitioner to 1) provide primary prevention of ARF through appropriate treatment of streptococcal pharyngitis; 2) be cognizant of the manifestations of the disease; and 3) provide secondary prevention of subsequent attacks through prophylaxis.

Acute Disease↗