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The genetics of rheumatic fever: relationship to streptococcal infection and autoimmune disease.

A large body of evidence suggests that patients with rheumatic fever immunologically respond abnormally both at a humoral and cellular level to streptococcal antigens cross-reactive with mammalian tissues. Implicit in this concept is that this abnormal immune response is genetically programmed. Using a monoclonal antibody called D8/17, a B cell marker has now been identified in 90-100% of all patients with rheumatic fever tested in 5 different geographical and ethnic populations. This trait appears to be inherited in an autosomal recessive fashion. This recognition of a B cell marker unique to individuals with rheumatic fever has important public health implications with respect to identification of individuals susceptible to rheumatic fever, possible prevention of disease and the recognition of prime candidates for future streptococcal vaccines.

Autoimmune Diseases↗

Rheumatic diseases other than rheumatic fever.

A total of 101 cases of rheumatic diseases collected during a period of six months from a hospital population have been analysed. These did not include rheumatic fever. Paediatric age group up to age of 12 years was not included in the series. Rheumatoid arthritis has been found to be the commonest condition, constituting 60 cases. Cervical spondylosis was next in common (24 cases). Ankylosing spondylitis and osteoarthritis constituted nine and six cases respectively. Emphasis has been laid on the bizarre manifestation of cervical spondylosis. The study, in spite of limitations throws some light on the relative frequency of rheumatic diseases other than rheumatic fever in Bangladesh.

Adolescent↗

[Immunologic studies in active rheumatic fever and infectious endocarditis].

We compared clinical and immunological characteristics of acute rheumatic fever (19 cases) and infectious endocarditis (7 cases), because these two diseases can be confused easily with each other and their differential diagnosis is not simple. In this small series we had cases of acute rheumatic fever with splenomegaly and/or vasculitis, as well as infectious endocarditis with subcutaneous nodules, which exemplifies the diagnostic problem. Using laboratory tests we were able to point out differences which are statistically significant, such as: rheumatoid factor by passive agglutination of IgG sensitized latex particles (X2 4.27 p less than 0M05), and tests which reflects the presence of circulating immune complexes, hemolytic capacity of antigammaglobulin antibodies (X2 3.79 p less than 0.05) and the presence of circulating C3 degradation products (X2 5.92 p less than 0.01), which occurs preferentially or exclusively in infectious endocarditis. Although in the standard patient the clinical assessment is usually sufficient to establish a diagnosis, when differentiation between acute rheumatic fever and infectious endocarditis is not clear, immunologic tests are helpful.

Adolescent↗

Pharyngeal carriage of group C and group G streptococci and acute rheumatic fever in an Aboriginal population.

Acute rheumatic fever (ARF) and rheumatic heart disease (RHD) represent an autoimmune response to group A streptococcal pharyngitis. In the Aboriginal population of the Northern Territory of Australia, some of the highest rates of ARF in the world have been reported, although throat carriage rates of group A streptococcus in this population are extremely low and symptomatic group A streptococcal pharyngitis is uncommon; by contrast, carriage rates of group C and G streptococci are high. Therefore, we questioned the involvement of these groups in ARF and showed that they have the potential to elicit an autoimmune response that may trigger ARF.

Adult↗

With what was rheumatic fever confused?

Follow-up of 427 cases initially diagnosed in Wairoa county during 1962-76 as rheumatic fever and/or rheumatic heart disease showed that 40 had neither condition and 51 had chronic rheumatic heart disease only. Sources of misdiagnosis were cardiac (e.g. congenital heart disease, onset of atrial fibrillation), joint (e.g. rheumatoid arthritis, gout), streptococcal infections not proceeding to rheumatic activity and febrile conditions of childhood. Awareness of the problems, some strengthening of the diagnostic criteria, and the evolution of the illness with time would serve to correct misdiagnosis.

Arthritis, Rheumatoid↗

Rheumatic fever and streptococci: the Wairoa College study.

The decline in incidence of rheumatic fever seen in many countries is not evident in New Zealand. Wairoa county has the highest hospital admission rate within New Zealand, 232 per 100 000 per year, five times the national average, 43 per 100 000. Studies in Wairoa included a cross-sectional survey of the local secondary school. Throat swabbing yielded 13.6% of subjects positive for beta-haemolytic streptococcus group A. Boys had a higher rate than girls, and girls aged 14 and over had a lower rate than younger girls. Discriminant analysis failed to identify ethnic, social or geographic factors as contributing to positivity. Sibling concordance was not significant. ASO titres were high in comparison with another New Zealand study (Rotorua Lakes). There was an association between high ASO titre and throat swab positive for group A and group C streptococci. The prevalence of definite rheumatic fever and rheumatic heart disease was established as 11/715 (1.5%). A wide range of residential geographic, climatic and socioeconomic factors failed to discriminate between cases and noncases. Ethnicity alone was a significant discriminator, the cases being more Maori than noncases.

Adolescent↗

Rheumatic fever: a multicenter study in the state of São Paulo. Pediatric Committee--São Paulo Pediatric Rheumatology Society.

Rheumatic fever is still the most commonly seen rheumatic disease in Brazilian pediatric rheumatology clinics. It remains a significant health problem since subsequent cardiac sequelae represent one of the most important causes of chronic heart disease in children. We reviewed the clinical manifestations of rheumatic fever in 786 patients followed at seven pediatric rheumatology clinics in the state of São Paulo, Brazil. All patients were diagnosed according to revised Jones' criteria. Regarding major criteria, 396 (50.4%) children exhibited carditis, 453 (57.6%) polyarthritis, 274 (34.8%) chorea, 13 (1.6%) erythema marginatum, and 12 (1.5%) subcutaneous nodules. Valvular lesions documented by echocardiography in the absence of accompanying auscultatory findings were found in 144 (18.3%) patients. Migratory polyarthritis was observed in 290 (64.0%) patients with articular involvement. Documented previous streptococcal infection assessed by serum antistreptolysin (ASO) titers occurred in 531 (67.5%) patients. Even though prophylaxis with benzathine penicillin was recommended to all patients, recurrent attacks were observed in 147 (18.7%). We emphasize the high frequency of chorea, silent carditis and recurrences in our series as well as the variable clinical presentation of arthritis in rheumatic fever. Multicenter studies should be encouraged to improve our understanding of the clinical features of rheumatic diseases in children and adolescents.

Adolescent↗

Oxygen free radicals in children with acute rheumatic fever.

We have investigated the relationship between oxygen free radicals and acute rheumatic fever with regard to diagnosis of the disease process. At the time of diagnosis, we measured the levels of reactive oxygen molecules in the plasma, this being a parameter for oxygen free radicals, and discovered the levels to be significantly higher when compared with those measured in a control group (P<0.05). The levels measured in the plasma, however, were not statistically different among patients with and without carditis. We found a progressive decrease in the levels measured in the plasma when patients with acute rheumatic fever were tested on the 15th, 30th and 90th days subsequent to diagnosis. By the 90th day, levels measured in the plasma were still higher, but no longer significantly elevated, when compared with the control group. The present study is preliminary, but raises the possibility that measurement of oxygen free radicals in the plasma could be used as a laboratory test for active state of acute rheumatic fever. Further investigations will be needed, nonetheless, to determine the clinical application of this technique.

Acute Disease↗

A case of acute rheumatic fever: echocardiographic findings for mitral regurgitation in acute rheumatic carditis.

An eight-year-old girl with mitral regurgitation in acute rheumatic fever was examined by echocardiography. The examination showed posterior displacement of the coaptation point of the anterior mitral leaflet, i.e. anterior mitral prolapse, and did not indicate signs of edematous change or verrucous fibrin deposits on the valves. Mitral valve prolapse is thought to be one of the causes of mitral regurgitation in acute rheumatic carditis.

Acute Disease↗

[Genetic variants of acid erythrocytic phosphatase as a risk factor of rheumatic fever].

A study was made of the genetic variants (isoenzymes, phenotypes) of acid erythrocytic phosphatase (AcP) in 120 patients with rheumatic fever. There were 78 women and 42 men aged 16 to 57 years. The population data concerned with distribution of the AcP variants among the population of Moscow were used as control. As compared with control, the patients suffering from rheumatic fever demonstrated the accumulation of the rarely occurring variants of AcP (AC, BC and CC, in particular). A significant direct correlation was established between the activity of isoenzymes and relative risk of rheumatic fever incidence. The definite regularities in the distribution of AcP variants were found to depend on the disease clinical patterns (the articular syndrome, the times of the formation of heart diseases, the character of recurrent rheumocarditis). The data obtained can used for distinguishing the rheumatic fever risk groups and forecasting the rheumatic process (to a certain degree of probability).

Acid Phosphatase↗

[Rheumatic fever among children in the Negev].

Between 1977-1989, 143 children with acute rheumatic fever were hospitalized here. In contrast to western countries, there has been no decline in the absolute number of hospitalizations for this disease here. A high prevalence of rheumatic fever was found among Bedouins and non-Ashkenazi Jews (2.5 and 1.5 times greater than the expected incidence, respectively). The affected children were usually from large families and lived in crowded conditions. Recurrences of rheumatic fever were more frequent among girls, and they were affected at an older age. The manifestations, in order of frequency, were arthritis, carditis and chorea. Chorea was found only among girls.

Acute Disease↗

The changing pattern of rheumatic fever in childhood.

The manifestations of acute rheumatic fever were compared in 231 children admitted to hospital from 1937 to 1940 with those in 252 children admitted from 1957 to 1960. Of the 178 in the early group seen during an initial attack, 82 had cardiac involvement; of these, 12 died early. Of the 218 patients in the later group with an initial attack, 96 had cardiac involvement; of these, two died early. Despite this decrease in early mortality, the incidence of isolated mitral value involvement (73% in the early group and 62% in the later group) and the incidence of congestive failure (20% and 13%) were similar. This reduction in the number of early deaths may be due to lowered virulence of the streptococcus, improved socioeconomic conditions, or therapy with penicillin and corticosteroids. The incidence of chorea and the frequency of recurrences were also less in the later group.

Adrenal Cortex Hormones↗

Acute poststreptococcal glomerulonephritis and acute rheumatic fever: occurrence in the same patient.

Group A streptococcus can cause both acute glomerulonephritis and acute rheumatic fever. The occurrence of characteristic acute poststreptococcal glomerulonephritis and acute rheumatic fever in the same patient is rare. We describe a 10-year-old girl with acute rheumatic fever who presented with the typical clinical and biopsy findings of acute poststreptococcal glomerulonephritis.

Acute Disease↗

Rheumatic fever chemoprophylaxis in adults.

In 2340 adult patients above the age of 26 years with rheumatic valvular disease (RHD) seen since 1987, injection benzathine penicillin (once in 3 weeks) was discontinued and the patients were followed for evidence of rheumatic fever for a period of one to nine years (mean of 3 years). Recurrence of rheumatic fever was seen in 5 patients (0.21%). This study confirms that recurrence of rheumatic fever in patients with RHD above 26 years of age is very low and hence we recommend long term chemoprophylaxis to the patients above the age of 26 on an individual basis.

Adolescent↗

[Contribution of doppler echocardiography to the diagnosis of the first attack of acute rheumatic fever].

Since 1944, the Jones criteria for the diagnosis of acute rheumatic fever have been regularly revised to integrate technical and diagnostic innovations. Echographic and Doppler criteria, however, remain unrecognised due to valvular insufficiency in healthy subjects. The aim of this study was to determine the cardiac lesions occurring in acute rheumatic fever and the diagnostic value of Doppler echocardiography. One hundred patients with an average age of 10 years were admitted to hospital because of a first attack of acute rheumatic fever between January 1991 and September 1992. Eighty-six had articular signs, 5 had chorea, but none had cutaneous lesions. Forty-seven murmurs of mitral insufficiency (MI) and eight of aortic insufficiency (AI) were detected; 10 children had signs of cardiac failure. Conduction defects were recorded in 12 cases. Echocardiography showed 7 pericardial effusions; often, the left heart chambers were dilated without alteration of the fractional shortening. The commonest lesions of the mitral valve were thickening of the two leaflets, the reduced mobility of the posterior leaflet, the rigidity of the anterior leaflet and 2 cases of ruptured chordae tendinae. The Doppler mode showed 73 cases of MI, 26 of which were at least moderately severe. These cases of MI were commonly excentric jets behind the posterior leaflet. There were 47 cases of AI, 10 of which were at least moderately severe. If all cases of moderately severe or mild AI and MI are considered as pathological when associated with suggestive morphological valve changes, the number of cases of carditis increased from 50 without the Doppler mode to over 80 with this mode. Doppler echocardiography validated the Jones criteria in 16 children. The authors propose Doppler echocardiography criteria for the validation of carditis.

Acute Disease↗