Acute rheumatic fever (ARF) refers to an autoimmune response to infection of the upper respiratory tract (and possibly of the skin) by group A streptococcus (Strep A) bacteria. ARF can affect the heart, joints, brain and subcutaneous tissues (the innermost layer of skin). While there is no lasting damage caused to the brain, joints and skin, ARF can cause lasting damage to the heart.

Rheumatic heart disease (RHD) is caused by damage to heart valves as a result of one or more ARF episodes. An affected heart valve can become scarred and stiffer, obstructing blood flow (stenosis), or it can fail to close properly, causing blood to flow backwards in the heart (regurgitation) instead of forward around the body. Regurgitation due to damage to the mitral valve is common in RHD.

This is the second annual report from the National RHD data collection. It presents information on ARF and RHD in Australia drawn from the established jurisdictional registers. Data in the collection are updated over time as the jurisdictional programs undertake data cleaning and quality improvement activity, so numbers in this report may not match those in previous reports. In addition, rates presented in this report have been calculated using the revised Aboriginal and Torres Strait Islander population estimates based on the 2016 Census, and should not be compared with those in previously published reports.

Jurisdictional RHD control programs and registers

Under the Rheumatic Fever Strategy, the Australian Government provides funding to support RHD control programs in four jurisdictions: Queensland, Western Australia, South Australia and the Northern Territory. These programs are funded to:

  • improve clinical care, including improved delivery of and adherence to secondary prophylaxis antibiotics
  • provide education and training for health care providers, individuals, families and communities
  • collect and provide agreed data annually to the AIHW for national monitoring and reporting of ARF and RHD and measuring program effectiveness in the detection and management of ARF and RHD
  • maintain a dedicated state-wide patient register and recall system for ARF and RHD.

How many people have ARF?

In 2014–2018, a total of 2,076 diagnoses of ARF were recorded in Queensland, Western Australia, South Australia and the Northern Territory, a rate of 4.4 per 100,000 population over the 5 years combined. During this period the number and rate of diagnoses increased each year, with 478 individuals diagnosed in 2018—a rate of 5.0 per 100,000 (compared to 321, or 3.5 per 100,000 in 2014).

During the same period, 1,963 ARF diagnoses were recorded among Indigenous Australians—a rate of 89 per 100,000 population over the 5 years combined. The number and rate of diagnoses increased from 300 (71 per 100,000) in 2014 to 457 (100 per 100,000) in 2018. ARF was more common among Indigenous females than males, and rates were highest among Indigenous people aged 5–14 (964 diagnoses, 195 per 100,000).

How many people have RHD?

As at 31 December 2018, there were 4,993 people living with RHD recorded on the 4 jurisdictional registers. Of these, nearly 9 in 10 diagnoses (4,325) were among Indigenous Australians, 1 in 3 (1,634) were aged under 25, nearly 2 in 3 (3,232) were females, and the greatest number were living in the NT (2,076).

In 2014–2018, 1,314 new RHD diagnoses were made among Indigenous Australians, a rate of 60 per 100,000 population. For this group, new RHD diagnoses were more common among Indigenous females compared to males (76 and 44 diagnoses per 100,000, respectively), and nearly 3 in 5 new diagnoses were among people aged under 25 years (773 diagnoses). The greatest number and highest rate of new diagnoses among Indigenous Australians was in the Northern Territory (525, or 141 per 100,000).

How many people died with RHD?

In 2014–2018, 315 deaths were reported for people living with RHD (cause of death is not recorded in the registers). Of these, 248 people (79%) were Indigenous Australians, 135 (43%) were aged 45–64 years, and 88 (28%) were aged 15–44 years. The median age at death was 56 years.

How many Indigenous Australians are prescribed secondary prophylaxis?

Secondary prophylaxis with regular benzathine penicillin G (BPG) is the only RHD control strategy shown to be both clinically and cost effective at community and individual levels (RHD Australia 2012). Between 2014 and 2018, the recommended regimen to prevent recurrences of ARF, and progression of RHD, involved regular intramuscular injections of BPG every 21–28 days, for a minimum of 10 years.

In 2018, among Indigenous Australians prescribed 4-weekly BPG:

  • 23% (748 people) received 100% or more of their prescribed doses
  • 19% (613) received 80% to 99% of their prescribed doses
  • 32% (1,012) received 50% to 79% of their prescribed doses
  • 26% (836) received less than 50% of their prescribed doses.

In 2018, among more than 3,900 Australians prescribed BPG, there were 111 recurrent ARF episodes. The majority of these recurrences are most likely a result of delayed doses of BPG.