Introduction

What is acute rheumatic fever?

Acute rheumatic fever (ARF) refers to an autoimmune response to infection of the upper respiratory tract—and possibly of the skin (McDonald et al. 2004)—by group A streptococcus (GAS) bacteria. Not all people who have a streptococcal infection develop ARF but, in those affected, it usually develops within 2–3 weeks of the infection (Webb 2015). ARF can affect the heart, joints, brain and subcutaneous tissues (inner most layer of skin) (Parnaby & Carapetis 2010) and can be extremely painful. While there is no lasting damage caused to the brain, joints and skin, ARF may cause lasting damage to the heart.

The risk of ARF recurrence is relatively high after an initial ARF episode and repeated episodes increase the likelihood of long-term heart valve damage, known as ‘rheumatic heart disease’ (Carapetis et al. 2016). As each episode of ARF can worsen the damage to the heart, the priority in disease management is to prevent ARF recurrences using long-acting penicillin treatment, which is known as secondary prophylaxis.

What is rheumatic heart disease?

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

Figure 1: Diagram of the heart, emphasising the heart valves.

The diagram is of the heart, emphasising the pulmonary valve, the tricuspid valve, the aortic valve and mitral valve.

Symptoms of RHD include fatigue, chest pain, swelling of legs and face and shortness of breath. Diagnosis may be difficult as symptoms are shared with other cardiac diseases. The type of valve affected and severity of damage, along with a history of ARF, are important clinical indicators for a RHD diagnosis. Many patients can remain asymptomatic despite having moderate or even severe RHD. Untreated RHD can cause arrhythmias (when the heart beats too fast, too slow, or irregularly), stroke, endocarditis (infection of the inner lining of the heart or its valves) and complications of pregnancy, and may be fatal.

Management of RHD includes treating symptoms and preventing worsening of disease, which requires regular echocardiography (echo) to identify and monitor which valves are damaged and how badly. Management of an RHD diagnosis is complex and involves coordination of multiple services such as primary health care (for secondary prophylaxis with penicillin and monitoring of heart medications such as anticoagulation therapy), oral healthcare services, echo, specialist medical care, and other cardiothoracic and interventional cardiology services (RHD Australia 2012).

ARF and RHD are preventable diseases

RHD is a preventable and treatable disease. It is common in low- and middle-income countries (Wyber 2014, Webb 2015), and only in socioeconomically disadvantaged populations in high-income countries. Both ARF and RHD are linked with overcrowding, socioeconomic deprivation, and low levels of functioning ‘health hardware’ (for example, toilets, showers, taps etc.) and lack of access to health care services (Webb 2015, Sims et al. 2016). Prevention measures that improve the living and environmental health conditions to the extent that ARF and RHD are no longer common in affected communities are known as primordial prevention measures. Improved living conditions and access to functional health hardware can reduce high rates of Group A streptococcal (GAS) infections and progression to ARF (Katzenellenbogen et al. 2017). 

After a GAS infection, progression to ARF is preventable through early treatment. This is called primary prevention of ARF and relies on correct diagnosis and treatment within 9 days of onset of GAS throat infection (Gerber et al. 2009). People not seeking medical attention for a throat infection, or misdiagnosis, can affect timeliness of treatment, as well as a failure to prescribe treatment as recommended in clinical guidelines (RHD Australia 2012).

Secondary prevention of the progression from ARF to RHD relies on correct diagnosis of ARF, to enable commencement of regular antibiotic preventive medication. Correct diagnosis is challenging as there is no specific laboratory test for ARF. Diagnosis is based on clinical criteria outlined in the Australian modification of the Jones criteria (Appendix A), which takes into account Australia’s high-risk groups, particularly Aboriginal and/or Torres Islander people (Carapetis et al. 2016). Guidelines recommend admission to hospital for clinical investigation and confirmation of the diagnosis (RHD Australia 2012).

For people with suspected or clinically confirmed ARF, benzathine penicillin G (BPG) is recommended in order to prevent further GAS infections and thereby reduce the risk of developing RHD or of RHD progression (Stollerman et al. 1955). BPG prophylaxis is clinically effective and cost-effective for RHD control at both individual and community levels (Webb 2015, Wyber 2015 & RHD Australia 2012).

References

Carapetis JR, Beaton A, Cunningham MW, Guilherme L, Karthikeyan G, Mayosi BM et al. 2016. Acute rheumatic fever and rheumatic heart disease. Nature reviews. Disease Primers 2(15084)84.

Gerber MA, Baltimore RS, Eaton CB, Gewitz M, Rowley AH, Shulman ST et al. 2009. Prevention of rhematic fever and diagnosis and treatment of acute Streptococcal pharyngitis: a scientific statement from the America heart Association Rheumatic Fever, Endocarditis and Kawasaki Disease Committee of the Council on Cardiovascular Disease in the Young, the Interdisciplinary Council on Functional Genomics and Translational Biology, and the Interdisciplinary Council on Quality of Care and Outcomes Research: endorsed by the American Academy of Pediatrics. Circulation 119(11):1541–51.

Katzenellenbogen JM, Ralph AP, Wyber R & Carapetis J 2017. Rheumatic heart disease: infectious disease origin, choric care approach. BMC health services research 17(1)793.

McDonald M, Currie BJ & Carapetis JR 2004. Acute rheumatic fever: a chink in the chain that links the heart to the throat? Lancet Infect Dis 4(4):240–5.

Parnaby M & Carapetis J 2010. Rheumatic fever in Indigenous Australian children. Journal of Paediatrics and Child Health 46:527–33.

RHD Australia (ARF/RHD writing group), National Heart Foundation of Australia and the Cardiac Society of Australia and New Zealand 2012. The Australian guideline for the prevention, diagnosis and management of acute rheumatic fever and rheumatic heart disease (2nd edn). Northern Territory: RHD Australia, Menzies School of Health Research.

Sims SA, Colquhoun S, Wyber R & Carapetis JR 2016. Global disease burden of Group A Streptococcus. In: Ferretti JJ, Stevens DL, Fischetti VA, editors: Streptococcus pyogenes: Basic biology to clinical manifestations. Oklahoma City (OK): University of Oklahoma Health Sciences Centre.

Stollerman GH, Rusoff JH & Hirschfeld I 1955. Prophylaxis against group A streptococci in rheumatic fever—the use of single monthly injections of benzathine penicillin G. BMJ 252(19):787–792.

Webb RH, Grant C & Harnden A 2015. Acute Rheumatic Fever. BMJ 351(8017). doi:https://doi.org/10.1136/bmj.h3443

Wyber R & Carapetis J 2015. Evolution, evidence and effect of secondary prophylaxis against rheumatic fever. Journal of Practice of Cardiovascular Sciences 1(1)9–14.