Introduction to acute rheumatic fever

Acute rheumatic fever is a multisystem inflammatory illness resulting from an autoimmune response to Streptococcus pyogenes (group A streptococcus). It is more likely to occur following recurrent streptococcal infections, in particular pharyngitis and impetigo. Acute rheumatic fever occurs most frequently in children aged 5 to 14 years, although recurrent episodes may continue into the fourth decade of lifeKatzenellenbogen, 2020RHDAustralia (ARF/RHD writing group), 2020.

Development of rheumatic heart disease is the most significant consequence of acute rheumatic fever, with damage to the cardiac valves accumulating over recurrent episodes. Less often, rheumatic heart disease can develop from a single severe episode of acute rheumatic fever. Rheumatic heart disease is the most common type of acquired heart disease in children and young adults worldwideCarapetis, 2005. The prevalence of rheumatic heart disease increases with age and peaks at 35 to 44 years RHDAustralia (ARF/RHD writing group), 2020. Following treatment of the acute episode of rheumatic fever, long-term management and follow-up is essential to prevent recurrent episodes and the development or progression of rheumatic heart disease. Working with patients to ensure acceptable and successful secondary prophylaxis against S. pyogenes infection is the most important intervention in long-term management.

The risk of S. pyogenes infection is increased by poor living conditions, including overcrowding and limited access to facilities for washingRHDAustralia (ARF/RHD writing group), 2020. The incidence of acute rheumatic fever has declined significantly in high-income countries over the last 150 years because of improved living conditions and the widespread use of penicillin to treat S. pyogenes infectionOliver, 2020Quinn, 1989RHDAustralia (ARF/RHD writing group), 2020. However, acute rheumatic fever remains an important contributor to burden of disease in low- to middle-income countries and in disadvantaged minority groups in high-income countriesKatzenellenbogen, 2020Oliver, 2020RHDAustralia (ARF/RHD writing group), 2020. In Australia, the incidence of acute rheumatic fever in Aboriginal and Torres Strait Islander peoples remains more than 100 times higher than in non-Indigenous Australians, resulting in rates of acute rheumatic fever and rheumatic heart disease among the highest in the worldKatzenellenbogen, 2020.

Aboriginal and Torres Strait Islander peoples continue to experience the effects of colonisation, racism and discrimination, resulting in economic and social exclusionParadies, 2016RHDAustralia (ARF/RHD writing group), 2020Vickery, 2007. An approach to healthcare that addresses these social determinants of health is an important strategy to reduce health inequality, and improve the health and wellbeing of Aboriginal and Torres Strait Islander peoplesLowe, 1989. To address the high incidence of acute rheumatic fever in Aboriginal and Torres Strait Islander peoples, health professionals need to consider what advocacy and social interventions will benefit their communities and individual patients, especially in decreasing household crowding and poverty, and improving culturally safe access to primary health care.

For detailed information on the diagnosis, management and prevention of acute rheumatic fever and rheumatic heart disease in Australia, see The 2020 Australian guideline for prevention, diagnosis and management of acute rheumatic fever and rheumatic heart disease.