Acute rheumatic fever (ARF) is a short illness following a group A streptococcus, or ‘Strep A’ infection. Strep A bacteria can cause infection in the throat (strep throat) and skin (skin sores, pyoderma, impetigo). For some people, the body’s immune system gets confused, and they have an autoimmune response to the infection. The result is a generalised, inflammatory illness called acute rheumatic fever.

Symptoms and signs of ARF

• Fever
• Arthritis, where one or more joints becomes red, hot, painful, swollen. Rheumatic arthritis primarily occurs in the larger joints such as the knees, ankles, wrists, and elbows.
• Sydenham chorea which presents as jerky, uncoordinated, uncontrollable movements, particularly of the hands, legs, tongue, and face.
• Carditis, or inflammation of the heart and the heart valves (which may or may not include symptoms)
• Erythema marginatum, a painless skin discolouration, usually on the trunk of the body, sometimes on the arms and legs, but almost never on the face. 
• Subcutaneous nodules that present as small, round, painless lumps over the elbows, wrists, knees, ankles, and near the spine. 

The severity of the ARF illness can range from very mild, to severe where the person may be bed-bound due to severe joint pain or heart failure. ARF can be difficult to diagnose because the illness does not present the same way for everyone, and symptoms do not necessarily all occur together and may be subtle.1^,2 Regardless of the seriousness of the illness, symptoms associated with ARF typically resolve within a few weeks.

Diagnosing ARF

There is no single test that can be used to diagnose ARF. Diagnosis is based on clinical assessment of the criteria required for ARF diagnosis, which includes specific combinations of the symptoms and signs, plus evidence of a recent Strep A infection.3

In Australia is it strongly recommended that all people suspected to have ARF, regardless of severity, should be admitted to hospital. Hospitalisation helps to maximise the likelihood of an accurate diagnosis, ensures prompt and optimal treatment, and allows a multi-disciplinary team to engage with the patient and family to develop a longer-term management plan and clear arrangements for follow-up. 

A smartphone Application has been developed to support health practitioners in the diagnosis of ARF, using the criteria in the RHDAustralia 2020 Australian guideline for prevention, diagnosis and management of acute rheumatic fever and rheumatic heart disease (3rd edition). The diagnosis algorithm is consistent with the 2015 AHA revision of the Jones Criteria by first determining if the patient is high risk or low risk and then considering presenting symptoms and investigations. 

Managing ARF

Management of ARF is based on confirming the diagnosis, determining whether the heart is involved using an echocardiogram (ultrasound of the heart), excluding other potential conditions that can mimic the features of ARF, treating the Strep A infection, and relieving symptoms. Ongoing care in hospital includes close monitoring, antibiotics, rest, pain management, relief of other symptoms as required (e.g., medications to control joint pain and fever) and heart failure management and planning for surgery if heart failure is present).

Other management in hospital includes:

• notifying the ARF illness to the local Disease Control or Public Health Unit.
• providing education about ARF and its management to the patient and family.
• liaising with the patient’s usual health service to establish ongoing care.

Longer term management focuses on preventing recurrent ARF, which includes delivery of regular, long-term secondary prophylaxis, prompt treatment for sore throats and skin sores, and preventing the development or progression of, rheumatic heart disease.

For more information, see Chapter 6 and Chapter 7 in the RHDAustralia 2020 Australian guidelines for prevention, diagnosis and management of acute rheumatic fever and rheumatic heart disease (3rd edition).

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• 1. Special Writing Group of the Committee on Rheumatic Fever E and Kawasaki Disease of the Council on Cardiovascular Disease in the Young of the American Heart Association, Guidelines for the diagnosis of rheumatic fever. Jones Criteria, 1992 update. Journal of the American Medical Association 1992. 268(15): 2069-73. View Source
• 2. Stewart T, McDonald R, Currie B. Use of the Jones criteria in the diagnosis of acute rheumatic fever in an Australian rural setting. Australian and New Zealand Journal of Public Health 2005; 29(6): 526-9 View Source
• 3. Gewitz MH, Baltimore RS, Tani LY, et al. Revision of the Jones Criteria for the diagnosis of acute rheumatic fever in the era of Doppler echocardiography: a scientific statement from the American Heart Association. Circulation.2015;131(20):1806-1818. View Source