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Rheumatic fever in Minnesota. II. Evaluation of hospitalized patients and utilization of a State Rheumatic Fever Registry.

We studied the hospital records of 124 patients with a discharge diagnosis of acute rheumatic fever who were hospitalized in 21 Minneapolis-St. Paul hospitals during 1975 and 1976. After careful review of the hospital records, we found that 83 (67 per cent) of these patients did not have an acute illness. Seventeen (41 per cent) of the 41 cases with an acute illness were thought to adequately fulfill the Jones' Criteria for acute rheumatic fever. Upon review of the rheumatic fever registry of the Minnesota State Health Department, we found that less than one-half of the hospitalized patients had been reported to the registry. Cases that fulfilled and did not fulfill the Jones' Criteria were reported with equal frequency, indicating significant underreporting and overreporting of rheumatic fever. Evaluation of secondary rheumatic fever prophylaxis, both in those patients with acute rheumatic fever as well as in those with rheumatic heart disease, indicated that many patients who, in theory, should be receiving prophylaxis were not receiving it. These studies indicate a need for more thorough evaluation of the current epidemiology of rheumatic fever and the role of a rheumatic fever registry, and imply a need for reevaluation of these programs. (Am J Public Health 69:767-771, 1979).

Acute Disease↗

Towards understanding the pathogenesis of rheumatic fever.

Acute rheumatic fever results from an immunological response to group A streptococcal infection, but the exact nature of this response, and of the underlying host and organism characteristics, continues to evade researchers. Earlier models of rheumatic fever pathogenesis emphasised the importance of humoral immunity, but more recent work suggests that cellular immunity may play a primary role. Greater understanding of these disease mechanisms is allowing researchers to move towards the development of a vaccine for rheumatic fever.

Humans↗

The reemergence of serious group A streptococcal infections and acute rheumatic fever.

Acute rheumatic fever and life-threatening group A streptococcal infections have reemerged during the past 15 years to once again become a serious health threat in the developed countries of the world. Reports of outbreaks of acute rheumatic fever in many parts of this country and others have shattered the complacency that the health-care community had acquired related to this devastating sequela of streptococcal pharyngitis. Invasive streptococcal infections, often associated with loss of limbs of life despite optimal antibiotic therapy, have underscored the potential virulence of these organisms. A new clinical entity, streptococcal toxic shock syndrome, has emerged as a consequence of the new invasive strains of group A streptococci. In this article, the authors summarize the recent changes in the epidemiology of group A streptococcal infections and rheumatic fever and review the potential reasons for the increased virulence of these organisms. In addition, they discuss prospects for a streptococcal M protein vaccine designed to control these infections and their sequelae.

Anti-Bacterial Agents↗

Familial Mediterranean fever and acute rheumatic fever: a pathogenetic relationship?

The frequency of acute rheumatic fever (ARF) in patients with familial Mediterranean fever (FMF) was documented and the effects of preceding streptococcal infections on the exacerbation of FMF were determined. In the first part of the study, 162 individuals with FMF were investigated for a history of ARF in a retrospective study. In the second part of the study, antistreptolysin-O (ASO) titres were measured in 130 individuals with FMF. Thirty-six patients had an arthritic attack (group A1), 55 patients had a typical FMF attack without arthritis (group A2) and 39 patients were in the attack-free interval (group B) during the investigation. Nine patients with FMF (5.5%) were considered to have ARF and three of them (1.85%) also had rheumatic heart disease. This prevalence of rheumatic heart disease in FMF is higher than that of the normal population (0.65%) reported in Turkey. Elevated ASO titres were found in 75%, 42% and 38% of the patients in groups A1, A2 and B, respectively. These percentages were found to be significantly higher in group A1 than in both groups A2 (p<0.01) and B (p<0.01). We concluded that patients with FMF might be more prone to the late complications of streptococcal infections.

Acute Disease↗

Treatment of acute streptococcal pharyngitis and prevention of rheumatic fever: a statement for health professionals. Committee on Rheumatic Fever, Endocarditis, and Kawasaki Disease of the Council on Cardiovascular Disease in the Young, the American Heart Association.

Primary prevention of acute rheumatic fever is accomplished by proper identification and adequate antibiotic treatment of group A beta-hemolytic streptococcal (GAS) tonsillopharyngitis. Diagnosis of GAS pharyngitis is best accomplished by a throat culture. Penicillin (either oral penicillin V or injectable benzathine penicillin) remains the treatment of choice, because it is cost effective, has a narrow spectrum of activity, has long-standing proven efficacy, and GAS resistant to penicillin have not been documented. Various macrolides, oral cephalosporins, and other beta-lactam agents are acceptable alternatives, particularly in penicillin-allergic individuals. The individual who has had an attack of rheumatic fever is at very high risk of developing recurrences after subsequent GAS pharyngitis and needs continuous antimicrobial prophylaxis to prevent such recurrences (secondary prevention). The duration of prophylaxis depends on the number of previous attacks, the time lapsed since the last attack, the risk of exposure to streptococcal infections, the age of the patient, and the presence or absence of cardiac involvement. Penicillin is again the agent of choice for secondary prophylaxis, but sulfadiazine or erythromycin are acceptable alternatives in penicillin-allergic individuals. This report is an update of a 1988 statement by this committee. It expands on the previous statement, includes more recent therapeutic modalities, and makes more specific recommendations for the duration of secondary prophylaxis.

Acute Disease↗

[Mortality trends for acute rheumatic fever and chronic rheumatic heart disease in Venezuela, 1955-1994]

This study reports on deaths due to acute rheumatic fever (ARF) and chronic rheumatic heart disease (CRHD) in Venezuela from 1955 to 1994. Census publications and other official sources provide the population data. Yearly age -and sex- specific mortality rates per 100,000 inhabitants were calculated and adjusted by the direct method to the WHO's standard population. The RF-adjusted mortality rate (AMR) declined: total from 7.68 to 1.08 (-85.9%); male from 7.53 to 0.84 (-88.8%); and female from 7.83 to 1.33 (-83.0%). In all cases female RF-AMRs were higher than male. There was a reduction of 88.5% in the ratio of RF deaths to cardiovascular deaths (ICD-6330-334,400-447; ICD-7330-334,400-447; ICD-8 390-438; ICD-9 390-438). ARF-AMR declined (-93.5%) as did CRHD-AMR (-85.1%). Proportional death by ARF and CRHD in patients 45 years old and younger increased. Venezuela experienced a continuous decline in RF-AMR from 1955 to the l980s, followed by a period of stabilization lasting until l994. The reduction could be the consequence of improvement in socioeconomic status and of RF prevention with penicillin, a standard practice in the health care system. The 1980s and 1990s RF-AMR stabilization could be associated with economic setbacks in Venezuela and changes in the agent's virulence patterns.

Journal Article↗

Guidelines for the diagnosis of rheumatic fever. Jones Criteria, 1992 update. Special Writing Group of the Committee on Rheumatic Fever, Endocarditis, and Kawasaki Disease of the Council on Cardiovascular Disease in the Young of the American Heart Association.

The Jones Criteria for guidance in the diagnosis of acute rheumatic fever were first published by T. Duckett Jones, MD, in 1944 and have been revised over the years by the American Heart Association. The current guidelines are an update of these criteria. For the first time, the guidelines are designed to establish the initial attack of acute rheumatic fever. Major manifestations, minor manifestations, and supporting evidence of antecedent group A streptococcal infection are discussed. These updated guidelines expand on the available tools to diagnose streptococcal pharyngitis and clarify the available antibody tests for detecting antecedent group A streptococcal infection. At the present time echocardiography without accompanying auscultatory findings is insufficient to be the sole criterion for valvulitis in acute rheumatic fever. Finally, this article addresses overdiagnosis of rheumatic fever and lists exceptions to the Jones Criteria, including recurrent attacks in individuals with a history of rheumatic fever.

Acute Disease↗

[Epidemiologic characteristics of streptococcal pharyngitis, scarlatina and rheumatic fever in the area of Belgrade 1981-1987].

The paper deals with the epidemiological data on streptococcal diseases (pharyngitis, scarlet fever and rheumatic fever) over the period from 1981-1987. The results of epidemiological investigations revealed a continuous decrease of rheumatic fever in the population, aged from 0 to 19 years. The maximum mortality rate of streptococcal diseases was registered in 1982 with 4,259.59 cases of streptococcal pharyngitis, 544.08 cases of scarlet fever and 9.17 of rheumatic fever on 100,000 inhabitants. The minimum morbidity rate for rheumatic fever was registered in 1987 (3,31%). Over the period from 1976-1987 the morbidity rate of rheumatic fever was twice and four times decreased in the Belgrade population, aged 0-19 years. According to the conclusions of the Vith Republican Meeting on Rheumatic Fever (Belgrade, 1980) a success in primary prophylaxis of rheumatic fever was achieved. But, there are still some questions without answer, as for instance, what is the relation between the carrier and the risk of rheumatic fever; why the human alone is susceptible to rheumatic fever; is the immunisation a future alternative approach, ets. In view of current epidemiological data on rheumatic fever in the world, it is necessary to start with a longterm prospective epidemiological study to determine the prevalent M-types of group A streptococci directly related to rheumatic fever.

Adolescent↗

[An approach to the diagnosis of recurrent attack of rheumatic fever in patients with rheumatic heart disease].

In order to evaluate recurrent attack of rheumatic fever (RARF) in patients with rheumatic heart disease (RHD), 97 patients with diagnosis of RHD for two years and more were included in this study. Progression of the lesions of cardiac valves was found in every case by comparing the echocardiographic and Doppler findings at present and two years ago. The history of the initial attack of acute rheumatic fever or the appearance of RHD were reviewed. The present complaints at admission and necessary laboratory procedures were studied. The data showed that 75 of the 97 patients had history of initial attack of ARF and the remaining 22 were symptomless with indolent carditis or carditis of insidious onset two years ago. In this admission, only 25 of the 97 patients had ARF in the Jones criteria were followed strictly, while the remaining 72 did not fulfill the criteria. It is suggested that if there is evidence of recent group a streptococcal infection, the conditions for diagnosis of RARF are as follows: 1) When one major or more than two minor criteria are present in a patient with RHD, a definite diagnosis of RARF may be made. 2) A presumptive diagnosis of RARF may be made, when a patient presents one minor criteria and several other manifestations such as anemia, abdominal pain, rapid sleeping pulse rate, tachycardia out of proportion to fever, malaise, epistaxis, precordial pain and an elevated level of IgG, IgA, C3 and circulating immune complexes.

Adolescent↗

Differentiation of patients with rheumatic fever from those with inactive rheumatic heart disease using the artificial subcutaneous nodule test, myocardial reactive antibodies, serum immunoglobulin and serum complement levels.

Thirty patients with acute rheumatic fever, and 20 with inactive rheumatic heart disease were studied in order to determine parameters which differentiate the two groups. Subcutaneous nodules developed following the subcutaneous injection of autologous blood in 16 of 26 patients with rheumatic fever but in none of the 20 with inactive rheumatic heart disease (P = 0.00001). Myocardial reactive antibodies were found in 14 of 30 patients with rheumatic fever, and in 4 of 20 with inactive rheumatic heart disease (P = 0.0505). Significantly higher values of IgG (P less than 0.001), IgA (P less than 0.01), C3 (P less than 0.001) and C4 (P less than 0.001) were observed in rheumatic fever patients. There was no significant difference in IgM levels. A combination of three variables (artificial subcutaneous nodules, IgA, and C3) had a sensitivity of 87% and a specificity of 100% for rheumatic fever. A combination of two variables (artificial subcutaneous nodules and IgA) had a sensitivity of 84% and a specificity of 100% for rheumatic fever.

Adolescent↗