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Rheumatic fever and gonococcal pharyngitis in an adult.

The arthritis of rheumatic fever in adults may mimic acute gonococcal arthritis. To further characterize this clinical picture the features of six patients presenting with the migratory polyarthritis of acute rheumatic fever have been analyzed. There were two men and four women, ranging in age from 18 to 43; in five the initial clinical impression was gonococcal arthritis. In all patients, however, the diagnosis of acute rheumatic fever was subsequently established. Characteristics of acute rheumatic fever not commonly described included tenosynovitis in all six and an erythematous rash in three. In three patients synovial fluid WBC exceeded 28,000/cu mm, with greater than 90% neutrophils. In five of the six, gonococcal arthritis was ruled out by appropriate studies and by failure to respond to antibiotics in all patients. The one patient with serum antigonococcal antibodies had gonococcal pharyngitis and acute rheumatic fever.

Acute Disease↗

Salicylate hepato toxicity in rheumatic fever.

Two children with rheumatic fever developed anicteric hepatitis while on high-dose aspirin therapy. The striking chemical abnormality was an elevation of serum glutamic oxalacetic transaminase (SGOT) [aspartate aminotransferase (AST)]. A percutaneous liver biopsy obtained from one of the patients showed ultrastructural abnormalities consistent with a toxic hepatitis and hepatic congestion. An awareness of this potential complication is important when treating children with aspirin at doses previously considered to be non-toxic.

Aspartate Aminotransferases↗

Acute rheumatic fever in Jordanian children.

Rheumatic fever remains a significant health problem in Jordan. We retrospectively reviewed medical charts of 28 boys and 22 girls (mean age at presentation 10.5 +/- 2.6 years) with confirmed diagnosis based on modified Jones criteria at Queen Alia Heart Institute from February 1999 to February 2002. Arthritis was the commonest major manifestation (88%; 68% migratory), carditis was second commonest (48%; 8% silent carditis) and chorea was seen in 6%. None had subcutaneous nodules or erythema marginatum. The mitral valve was most commonly affected (80%); both mitral and aortic valves were affected in 25%. Pericarditis was seen in 12.5% and acute congestive heart failure in 4%. Practitioners should be aware of diverse clinical presentations and emphasize strict adherence to prophylaxis guidelines.

Acute Disease↗

Inverse relationship between the ratio of ICAM-1 expressing lymphocytes and serum TGF-beta 1 concentrations in acute rheumatic fever.

Autoimmunity in acute rheumatic fever (ARF) is triggered by group-A beta hemolytic streptococci (GAS). Although most of the recent work has focused on the major impact of lymphocytes, the exact immunopathogenesis is still unresolved. Regulation of self-tolerance in response to GAS has been investigated in various animal experiments. This study aimed to associate the ratio of lymphocytes bearing adhesion/costimulatory molecules, Bcl-2/CD95 and serum TGF-beta1 concentrations with clinical stages of ARF. Thirty-five patients were assigned according to the clinical stages. Bcl-2 expression on CD19+ and CD3+ lymphocytes was similar within patient groups and controls. CD62p expression was higher in patients with carditis. The ratio of ICAM-1 bearing lymphocytes was significantly different between patient groups and controls and was increased through acute to remission stages longitudinally. In contrast, a gradual and significant decrease in TGF-beta1 concentrations was observed longitudinally from acute to chronic stages. A positive correlation has been documented between ICAM-1+ lymphocyte ratios and Fas+ cytotoxic T cell ratios supported by a prominent increase in CD95+ T cells. These data draw our attention to the role of ICAM-1, Fas and TGF-beta1 in ARF pathogenesis through the perspective of self-tolerance in a clinical setting.

Acute-Phase Reaction↗

A 45-year perspective on the streptococcus and rheumatic fever: the Edward H. Kass Lecture in infectious disease history.

Rheumatic fever has been considered a major problem among civilians in the United States and elsewhere for 100 years but was not recognized as a concern among the U.S. military until World War II. At that time the only available control measure was antimicrobial prophylaxis of recurrent rheumatic fever. Subsequent studies, conducted primarily by the Streptococcal Diseases Laboratory of the Armed Forces Epidemiological Board, demonstrated that rheumatic fever could be prevented by the treatment of patients with streptococcal pharyngitis and by the administration of penicillin for the prophylaxis of streptococcal infections in large groups. With the use of available preventive measures, rheumatic fever virtually disappeared by the 1970s. In 1985, however, rheumatic fever and severe streptococcal infections reappeared, first in the Rocky Mountain area. It is speculated that this reappearance was due to special strains of group A streptococci and--in severe cases--the production of pyrogenic exotoxins. At present, cases continue to occur but not at the level seen in the late 1980s.

Communicable Diseases↗

Benzathine penicillin G for rheumatic fever prophylaxis: 2-weekly versus 4-weekly regimens.

Rheumatic fever is still one of the major public health problems in Egypt and the developing countries. It is characterized by a high tendency to recur following streptococcal infections. The use of long acting penicillin for prophylaxis against strep infections was a good achievement in this field, yet, recurrences have been reported in patients following monthly prophylactic programs. Clinical experience in Alexandria have shown for a long time that giving penicillin every 2 weeks is followed by less recurrences of rheumatic fever. Recently, reports came showing that effective penicillin levels are not maintained except for 2 to 3 weeks after the injection. In the present study, we compared two regimens of prophylaxis with 190 patients in the 2-weekly regimen, and 170 patients in the 4-weekly regimen being followed up for 2 consecutive years. Two hundred and sixty nine streptococcal infections occurred during this period. Although the streptococcal infection rate was equal in both groups, the rheumatic fever recurrence rate and the RF attack rate were significantly higher in the group of patients on the 4-weekly schedule. The results of this study have shown the superiority of the 2-weekly schedule in the adequate control of RF recurrences. We suggest that this schedule should be implemented for secondary prophylaxis of rheumatic fever in Egypt and other areas with severe RF.

Adolescent↗

Epidemiological approaches to understanding the pathogenesis of rheumatic fever.

Thus, in 1985, over four decades after discovery of the first important clue towards understanding the pathogenesis of rheumatic fever, medical science still has not defined the mechanism(s). Objective evaluation of available data from around the world indicates that penicillin alone will not lead to effective control. Furthermore, in geographical areas where streptococcal infections, rheumatic fever and rheumatic heart disease are most prevalent, it seems unlikely that standards of living will rise quickly enough to impact on the incidence of this disease during the next several decades. There is renewed worldwide interest in and emphasis on the implementation of rheumatic fever control programmes. The World Health Organization (WHO), in collaboration with the International Society and Federation of Cardiology (ISFC), is promoting a strategy to develop national programmes for the prevention of rheumatic fever and rheumatic heart disease. In 1984-5, designated by the ISFC as the Year of the Rheumatic Child, the WHO Cardiovascular Disease Unit initiated plans to collaborate with 15 developing countries in the development and implementation of rheumatic fever control programmes. Because of the inescapable conclusion that control methods are most efficiently applied when pathogenetic mechanisms are understood, additional epidemiological data should be collected to assist both basic scientists and clinicians in understanding more about this unique disease.

Disease Susceptibility↗

Acute rheumatic fever: an update.

The incidence of acute rheumatic fever is increasing in the United States, but many physicians are now unfamiliar with the diagnosis and treatment of this disease. The revised Jones criteria provide guidance for the diagnosis of rheumatic fever; two major criteria or one major and two minor manifestations are required to make the diagnosis. Salicylates are usually the initial treatment of choice for the arthritis of rheumatic fever. Penicillin remains the recommended prophylactic agent for secondary prevention.

Acute Disease↗

Rheumatic fever in college students.

The results of three independent surveys concerned with rheumatic fever and heart disease in students at the University of California were assembled and found to be in close agreement. A full 2 per cent of all students believed they had had rheumatic fever; and several times that proportion gave a history of one of the rheumatic manifestations. Only 0.25 per cent had demonstrable rheumatic heart disease and 0.1 per cent had congenital heart disease. Physiologic murmurs may occur in 3 per cent or more of students entering college. Penicillin prophylaxis is important in persons with rheumatic heart disease, but it is important not to put a label of rheumatic heart disease on persons who think they have had rheumatic fever but who have no demonstrable heart disease. Long term penicillin prophylaxis or other long term prophylactic procedures directed against rheumatic fever are not indicated unless the diagnostic criteria for rheumatic fever are clearly met or unless rheumatic heart involvement is definitely present.

Antibiotic Prophylaxis↗

Streptococcal infections that fail to cause recurrences of rheumatic fever.

Prospective studies of recurrences of streptoccal infection and acute rheumatic fever were conducted among patients attending the acute rheumatic fever prophylaxis clinic (City of Memphis Hospitals, Memphis, Tennessee) between 1965 and 1972. The patient population consisted of 124 rheumatic children and adults, two-thirds of whom had evidence of rheumatic heart disease. A total of 104 immunologically documented streptococcal infections occurred during 235 patient-years of follow-up (44.3 infections per 100 patient-years) without a single recurrence of rheumatic fever. Immune responses tended to be modest, and 80% of the infections were subclinical. The majority of our group A streptococcal isolates were obtained from routine cultures of specimens from asymptomatic individuals. Many of these strains were "pyoderma" serotypes, whereas others exhibited a characteristic (production of opacity factor) recently reported to be associated with decreased immunogenicity. Several factors may have contributed to the low recurrence rate of acute rheumatic fever, including the age range of the population under study and immunologically significant infections with strains of Streptococcus that were not group A, but a major reason may be the possibility that the group A strains prevalent in this population have diminished rheumatogenic potential.

Adolescent↗

Evaluation of subclinical valvar disease in patients with rheumatic fever.

Carditis is the only manifestation of acute rheumatic fever that leads to permanent disability. Hence, its diagnosis is of paramount importance. Recently, it has been reported that Doppler echocardiography has disclosed subclinical valvar regurgitation in some patients with acute rheumatic fever manifested as isolated arthritis or pure chorea. The prognosis of such patients with acute rheumatic fever and subclinical valvitis is not clear. We aimed, therefore, prospectively to investigate the potential to diagnose patients with subclinical carditis. We examined 40 patients, aged from 7 to 16 years, with Doppler evidence of mitral and aortic regurgitation, but in the absence of any pathologic murmur. The major findings satisfying the Jones criterions were arthritis in 29 patients, chorea in 10 patients, and arthritis and erythema marginatum in one patient. Of the patients, 33 had mitral regurgitation, 6 patients had combined mitral and aortic regurgitation, and one patient had aortic regurgitation. The patients were followed over a mean period of 18.1 +/- 13.9 months, the valvar regurgitation disappearing in 23 (57.5%). No significant differences were observed in the resolution of the valvitis between those treated with acetylsalicylic acid, steroids, or those receiving no treatment. It is noteworthy, nonetheless, that patients treated with steroids were the fastest to recover from valvitis (p < 0.05). Based on our study, we suggest that subclinical valvitis demonstrated by echocardiography should now be accepted as adequate evidence for the diagnosis of carditis, and become a major diagnostic criterion for acute rheumatic fever. When managing this group of patients with subclinical disease, treatment with steroids seems to have a role in promoting early resolution of the valvitis.

Adolescent↗

Recurrent acute rheumatic fever: a forgotten diagnosis?

The incidence of acute rheumatic fever has seen a dramatic decline over the last 15 to 20 years in most developed countries and treatment of this disease has changed little since. The ease of travel and immigration and the cosmopolitan nature of many cities mean that occasionally the disease will come to the attention of clinicians not familiar with its presentation, resulting in delayed diagnosis and treatment. We present a case of recurrent acute rheumatic fever in a patient who was initially thought to be suffering from acute bacterial endocarditis on her previously diseased rheumatic aortic valve. This culminated in her undergoing urgent aortic valve replacement during a phase of the illness that should have been treated with high dose anti-inflammatory medication. Therefore, clinicians should be aware of this condition and include it in their differential diagnosis of the febrile patient with a previous history of rheumatic fever. We briefly discuss the diagnostic dilemma of patients suffering from this condition and in differentiating it from acute endocarditis.

Acute Disease↗