Acute rheumatic fever and rheumatic heart disease in the Top End of Australia's Northern Territory

OBJECTIVE: To describe the epidemiological and clinical features of acute rheumatic fever and rheumatic heart disease in the Top End of the Northern Territory. METHODS: A retrospective review (in some instances as far back as the 1960s) of all cases of known or suspected acute rheumatic fever or rheumatic heart diseases, with prospective validation of clinical status where necessary. Cases were ascertained from hospital and community medical clinic records and medical staff; and from records and health staff of 10 rural communities. RESULTS: Three hundred and eighty-six revised Jones criteria-confirmed episodes of acute rheumatic fever were documented in 249 individuals (99% Aboriginal). The annual incidence of confirmed acute rheumatic fever between 1989 and 1993 was 254 per 100,000 Aboriginal people aged 5 to 14 years. A more accurate estimate of 651 per 100,000 came from 10 rural communities with more complete information. As of 1995, there were 286 people living with established rheumatic heart disease (95% Aboriginal). The point prevalence of rheumatic heart disease among Aboriginal people was 9.6 per 1000, with a rate of 24 per 1000 in one large rural community. Sydenham's chorea was common, and associated with later rheumatic heart disease in 49% of cases. There was a preponderance of females with acute rheumatic fever, rheumatic heart disease and chorea. CONCLUSIONS: In Aboriginal people in rural northern Australia the incidence of acute rheumatic fever is higher than that reported anywhere in the world, and the prevalence of rheumatic heart disease is among the highest in the world. While continuing attention must be paid to alleviating the causes of these diseases of poverty, immediate action is needed to improve diagnosis of acute rheumatic fever, adherence to secondary benzathine penicillin prophylaxis regimens, and follow-up of rheumatic heart disease cases.