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Rheumatic fever, rheumatic heart disease, and the streptococcal connection: the role of streptococcal antigens cross-reactive with heart tissue.

The role of streptococcal infections in initiating the diverse clinical and pathological manifestations of rheumatic fever and rheumatic heart disease is considered in relation to the multiple cross-reactive relations of group A Streptococcus and tissue antigens. Autoantibodies to the following shared antigens have been demonstrated in sera of patients wit rheumatic fever: (1) cardiac, skeletal, and smooth muscle; (2) heart valve fibroblasts; (3) neurons in basal ganglia; and (4) a group A carbohydrate-related determinant in connective tissues. Circulating autoantibodies to these different antigens were present in higher titer or occurred more frequently in patients with rheumatic fever than in those with uncomplicated streptococcal infections. A direct correlation of the presence of these autoantibodies with carditis could not be established. The pathogenetic mechanisms that link streptococcal infection to rheumatic fever and rheumatic heart disease are not yet clear. Among the possibilities to be considered within the above frame of reference are combined cell-mediated and humoral autoimmune mechanisms directed to one or more cross-reactive antigens in the tissues, selective binding of streptococci to tissues, role of circulating immune complexes, and linkage with histocompatibility antigens.

Antibodies, Bacterial

HLA phenotypes in patients with rheumatic fever, rheumatic heart disease, and Yersinia arthritis.

HLA phenotypes were determined in 109 patients with rheumatic fever (RF), 48 patients with Yersinia arthritis (YA), 86 patients with chronic rheumatic heart disease (RHD), and 326 controls. There was an increased frequency of Bw35 in RF as compared to controls (Pc less than 0.01), while B18 was more common in patients with acute carditis than in those without (P less than 0.02). HLA frequencies in RHD did not differ significantly from those in controls. A significant correlation between B27 and YA was observed (Pc less than 0.001). Carditis or iritis occurred in 10 of 31 B27 positive YA patients but in none of 17 B27 negative patients. Eleven of 31 B27 carriers had signs of urological inflammation vs one of 17 B27 negative patients. In the B27 positive YA group, there were three men with previous ankylosing spondylitis and one with Reiter's syndrome (RS). Also, four patients developed RS during Yersinia infection. This simultaneous occurrence of three B27 positive rheumatic diseases suggests that a patient with one "B27 positive rheumatic disease" is more susceptible to other diseases or symptoms known to be associated with the B27 antigen.

Adult

Awareness about sore-throat, rheumatic fever and rheumatic heart disease in a rural community.

This I.C.M.R. study was conducted in 74 villages of Chiraigaon block, Varanasi, U.P., during the period March 1983 and December 1986. Before and after health education awareness survey about sore throat, rheumatic fever and rheumatic heart disease was carried out by interviewing 315 persons by stratified random sampling. The study shows that there is significant increase in the knowledge about most of the symptoms, causes, consequences and preventive measures of sore throat, rheumatic fever and rheumatic heart disease. This paper highlights the importance of health education as a vital component of rheumatic heart disease control programme.

Awareness

The changing picture of rheumatic fever.

Rheumatic fever has decreased in frequency and severity, but the disease has not been eradicated in this country, especially in low socioeconomic populations. Carditis is less common, and changes in the frequency of other rheumatic manifestations such as chorea has made the clinical diagnosis more difficult. Streptococcal antibody tests are still the most useful laboratory aid, but care must be taken against overinterpretation. The search for the answer to the pathogenesis puzzle continues. Until the nature of the disease is better understood, it is unlikely that rheumatic fever will be eradicated. In the meantime, however, the incidence of the disease can be reduced further by improved medical care for deprived populations.

Adolescent

Juvenile rheumatic fever and rheumatic heart disease at Ramathibodi Hospital, Thailand.

One hundred consecutive cases of rheumatic fever and rheumatic heart disease who were seen at Department of Pediatrics. Ramathibodi Hospital were reviewed. Particular attention was given to the pattern and the outcome of the cardiac status of the patients. The high incidence of severe carditis and tight mitral stenosis was similar to most reports from other developing countries. There was a poor prognosis for the cardiac status of those who came late, had more than valvular lesions, were in congestive heart failure, or had preexisting heart disease and atrial fibrillation. In spite of this, 6 patients had no evidence of heart disease after being followed up for less than 5 years.

Adolescent

HL-A antigens in Europeans and Maoris with rheumatic fever and rheumatic heart disease.

Using a standard microtoxicity technique of tissue typing, the distribution of tissue antigens in 75 Maoris and 514 European disease-free blood donors was determined. Fifty Maori and 50 Europeans with rheumatic fever or rheumatic heart disease were compared with each control group. Normal Maoris had HL-A3 less frequently than Europeans (P less than .0005). HL-A28 was reduced (P less than .005) and HL-A17 increased in European patients (P less than .0005). In Maori patients there were minor differences in the frequency of HL-A3 and 8, which were increased, and HL-A10, which was diminished.

Europe

Rheumatic fever and rheumatic heart disease in developing countries.

Studies on the prevalence and other epidemiological features of rheumatic fever and rheumatic heart disease and pilot prophylactic programmes have been carried out in India for the past 12 years or more. The results of these, together with data from other developing countries, have been taken into account in discussing the problems of these diseases in the developing world. Suggestions for their control, to be modified according to local conditions, are made.

Adolescent

Epidemiologic aspects of rheumatic fever and rheumatic heart disease in Israel.

Rheuamtic fever and rheumatic heart disease are still important public health problems in Israel. Rheumatic fever is a seasonal disease, more common during the cold months of the year. A high incidence of rheumatic fever followed the immigration waves of the 1950s, in contrast with a decline in new cases over the last 10 years among both Jews and non-Jews. In the same period (1950s to 1975), a decline in the numbers of hospitalized patients was also reported. After 1970 mortality rates of rheumatic fever dropped to zero. The prevalence of rheumatic heart disease has declined in children, as shown in school studies, but in adults, a different trend is seen. The number of hospitalized patients has increased, in parallel with the increasing population, to more than 3,000 hospitalized patients per year. While a plateau curve is seen in the mortality rates of males of all ethnic origins studied, among females there is a slight decline in mortality. The discrepancy between the decline in occurrence of rheumatic fever and rheumatic heart disease in children and the increase in prevalence of rheumatic heart disease in adults is explained by the massive immigration from countries with a high prevalence of rheumatic heart disease.

Adolescent

Antimyocardial antibodies in heart diseases. II. Immunofluorescent patterns in rheumatic fever and rheumatic heart disease.

Antibodies against rat myocardium were studied by means of histoimmunfluorescence (indirect technique) in 32 rheumatic fever patients. Antibodies were found in 54% of the cases without carditis or active rheumatic valvulitis and in 83% of those with carditis. The fluorescent patterns observed were : sarcolemmal, sarcoplasmic (intermyofibrillar) and smooth muscle of vessels. The sarcolemmal pattern was more frequently observed in rheumatic carditis while the intermyofibrillar one was more common in rheumatic fever without evidence of carditis as well as rheumatic valvulitis. The serum factors involved in the sarcolemmal fluorescence were inhibitied by the insoluble saline myocardium extract.

Adult

An epidemiological study of rheumatic fever and rheumatic heart disease in Lagos.

In Lagos 12 755 schoolchildren aged between six and 12 years were screened for evidence of rheumatic heart disease and showed a prevalence rate of 0.03%. Group C (27.7%) and group G (47.3%) predominated in the throat and in cases of pharyngitis, while group A predominated on the skin. Two hundred and sixty-six cases of pharyngitis were recorded, 70 (26.4%) were positive for beta-haemolytic streptococci. Half of the cases of streptococci while 28.2% and 21.1% were caused by groups C and G respectively. A diversity of serotypes of group A streptococci, which included types 49 and 55, and C and G streptococci were isolated from impetiginous skin lesions. The main point of interest is the association of group C and G streptococci with sore throat and skin infections. The pathogenicity of C and G in such circumstances merits further investigation. The present study also showed that prophylactic benzathine penicillin (Penadur) given to patients with rheumatic heart disease or rheumatic fever had been effective with no recurrent attack of acute rheumatic fever.

Child

The natural history of rheumatic fever and rheumatic heart disease in the Orient.

Studies published in the past 10 years suggest that group A streptococcal infections are frequent in the Orient and lead to a high incidence of rheumatic fever (RF) and rheumatic heart disease (RHD). In the present study, streptococcal infections were found to be more prevalent in Japan and Taiwan, whereas RF and RHD were more common and severe in the Philippines, Thailand, and Indonesia, particularly among the socioeconomically less privileged populations. The pattern of childhood RF varied: Carditis was the most common manifestation, occurring in 57% to 94% of the patients; polyarthritis was generally atypical and less common in the tropics; chorea minor and erythema marginatum were much more common in Japan, less common in Taiwan and rare in the tropics. RF recurrences were quite common and led to the development of new carditis, and deterioration or persistence of the pre-existing heart disease. The 5 year mortality rates differed greatly, ranging from zero to 42%. There was disappearance of the heart murmur in 16.5% to 37.5% of patients. Such apparent recovery was related to adherence to chemoprophylaxis. The major risk factors adversely affecting survival were the severity of carditis, inadequacy of medical service, non-compliance to chemoprophylaxis, RF recurrence, poor socioeconomic status, and high prevalence of group A streptococci. It is concluded that there is no uniform "Oriental-type" of natural history of RF and RHD. The natural history varies greatly among countries as is true in other parts of the world.

Adolescent