Background

Acute rheumatic fever (ARF) and rheumatic heart disease (RHD) were once common across the world, including in Australia, Europe, Great Britain, and North America.1^,2^,3 Rates of disease declined in most of these countries during the mid-20th century, primarily due to improved socioeconomic conditions and reduced household crowding, better access to healthcare, and the availability of penicillin to treat streptococcal infections.4^,5 

Today, there are small populations of older people living with RHD who developed the disease when they were young, and before improvements in socioeconomic conditions and antibiotics. ARF and RHD are also common among young people living in many low- and middle-income countries despite the availability of penicillin, particularly where widespread poverty persists and access to health care is difficult.6^,7

ARF and RHD in Australia

In Australia, ARF and RHD continue to cause significant ill-health and premature death among Australia's Aboriginal and Torres Strait Islander peoples and New Zealand Maori and Pacific Islanders. These groups also experience considerable inequalities across a wide range of social, educational and health outcomes compared with the general population.8^,9^,10

• Since the early 1990s, ARF has occurred almost exclusively in young Aboriginal and Torres Strait Islander peoples, with highest rates among children.11 
• Females are more likely to be diagnosed with ARF and RHD than males, and rates of disease are highest across northern Australia.12 
• The number of Aboriginal and Torres Strait Islander peoples affected by ARF and RHD appears to be increasing, however the exact reasons for this are not clear.13 
• People who have had ARF are at high risk of having it again, with one in five people having a recurrent episode of ARF within 10 years of their first illness.14
• The burden of disease often lasts most of a person’s lifetime, starting with ARF in childhood and progressing to RHD for the duration of adult life.
• Complications from RHD are common, and include atrial fibrillation, endocarditis, heart failure and stroke.
• Aboriginal and Torres Strait Islander people with RHD are more likely to die compared with non-Indigenous Australians with RHD; however, the death rate for both groups is decreasing.15

Data collection and reporting systems

Understanding the true burden of ARF and RHD in Australia, that is, the number of people with disease and where they live, is difficult. State and territory based ARF/RHD disease registers are established in the Northern Territory, Queensland, Western Australia, South Australia, and New South Wales. Data from these registers are collected and reported by the Australian Institute of Health and Welfare.16

While these reports are very useful, there are still challenges to fully understanding ARF and RHD in Australia. For example:

1. Each ARF/RHD register was developed at a different time, and the processes for collecting data vary. 
2. In some areas, there are only small numbers of people with ARF and RHD, and reporting small numbers is difficult.
3. Indigenous population estimates are not stable below state and territory level, and this can make reporting difficult.
4. There are various other sources of ARF and RHD information (e.g., hospital admission records, surgery records, death registers), however, these systems are not linked together and are not linked with the ARF/RHD registers, so an accurate number of people with disease is hard to predict. 

End RHD in Australia: Study of Epidemiology (ERASE)

In 2020, researchers working on a linked ARF/RHD data project published the End RHD in Australia: Study of Epidemiology (ERASE) study.17 ARF/RHD data were collected, linked and analysed from the five ARF/RHD disease registers,18 as well as data from hospital admissions, death databases, cardiac surgery databases, emergency department presentations, primary care presentations, and maternity notification data systems. The ERASE study provided the most comprehensive description of the epidemiology and management of ARF and RHD available in Australia.

As well as being reported through a series of publications,19^,20^,21^,22^,23^,24^,25 results from the ERASE study are being used to guide health policy and clinical practice care to support people with ARF and RHD in Australia. For example, the results

• provided evidence for the 3rd edition of RHDAustralia's Australian ARF/RHD Guidelines.
• informed policy recommendations for the RHD Strategy Endgame Report.26
• were used to project future costs associated with health interventions for ARF and RHD. 
• are being used to provide meaningful information to Aboriginal and Torres Strait Islander communities about ARF and RHD risk, prevention, diagnosis, and management.

A series of maps showing the rates of ARF and RHD across Australia from the ERASE study data have been developed for the health, research, and scientific community. These maps are available to use in reports and presentations. Interpretations have been included to help provide context and highlight important information. 

Download Maps  Select Read Only

(Not all maps appear in the ERASE publications)

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• 18.  Bond-Smith D, Seth R, de Klerk N, et al. Development and evaluation of a prediction model for ascertaining rheumatic heart disease status in administrative data. Clinical Epidemiology. 2020;12: 717-730.
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• 20.  Katzenellenbogen JM, Bond-Smith D, Cunneen R, et al. The End Rheumatic Heart Disease in Australia Study of Epidemiology (ERASE) Project: data sources, case ascertainment and cohort profile. Clin Epidemiol 2019;11: 997-1010.
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• 26. Wyber R, Noonan K, Halkon C, et al. The RHD Endgame Strategy: The blueprint to eliminate rheumatic heart disease in Australia by 2031. Perth: The END RHD Centre of Research Excellence, Telethon Kids Institute, 2020.