Rheumatic fever

Background

Acute rheumatic fever (ARF) is caused by a reaction to a bacterial infection with particular strains of group A streptococcus. It has long been thought that ARF only follows streptococcal pharyngitis (sore throat), however recent studies from Aboriginal populations in Australia have suggested streptococcal skin infection may precede some cases of ARF. Those who have experienced one episode of ARF are more likely to suffer recurrent attacks with subsequent group A streptococcal infections. The most severe complication of recurrent ARF is permanent damage to heart valves, known as rheumatic heart disease.

ARF usually affects children aged 5-15 years. Most cases of ARF currently occur in developing countries. Worldwide there is an estimated 470 000 new cases of ARF annually (60% of whom eventually develop rheumatic heart disease). In most developed countries ARF is now rare, with a few notable exceptions; the highest documented rates of ARF in the world are in Maori and Pacific people in New Zealand, Aboriginal Australians, and those in Pacific Island nations.

Clinical features

Symptoms of ARF generally develop several weeks after an episode of streptococcal pharyngitis (however many patients do not recall having a sore throat). As well as non-specific symptoms such as fever, abdominal pain, and muscle aches, ARF causes a variety of characteristic clinical features:

• Polyarthritis (multiple inflamed joints)
• Carditis (inflammation of the heart) – This involves the heart valves, heart muscle, and membrane surrounding the heart.
• Sydenham chorea – This is a disorder of the nervous system characterised by personality changes, muscle weakness, and involuntary movements.
• Erythema marginatum (one type of annular erythema) – This is a characteristic skin rash that occurs in about 10% of first attacks of ARF in children; it is very rare in adults. The rash can be difficult to detect in dark-skinned people. When present, it is found on the trunk and upper arms and legs, but almost never on the face. The rash appears as pink or red macules (flat spots) or papules (small lumps), which spread outwards in a circular shape. As the lesions advance, the edges become raised and red, and the centre clears. The lesions are not itchy or painful, and sometimes go unnoticed by the patient. The lesions can fade and reappear within hours and may persist intermittently for weeks to months.
• Subcutaneous nodules (small lumps under the skin) – These are now uncommon, occurring in less than 2% of patients with ARF. The painless nodules are found over joints (such as the elbows, knees, ankles, and knuckles), the back of the scalp, and the vertebrae (backbone). The nodules are firm, round, mobile, and range from 0.5-2 cm in size. The nodules are usually only found when severe carditis is present and resolve within one month.

Erythema marginatum

Diagnosis

The diagnosis of ARF is challenging, as there are no clinical features or diagnostic tests available to confirm or rule out this condition. Instead, the diagnosis is made using the Jones criteria. These criteria require evidence of a preceding group A streptococcus infection, and various combinations of the characteristic features above and other non-specific clinical features. A detailed explanation of these criteria (and modifications for the New Zealand environment) can be found on the National Heart Foundation of New Zealand website.

Tests to confirm evidence of a group A streptococcal infection include:

• Blood tests to look for elevated or rising antibodies to group A streptococcus. The most commonly used tests are the plasma antistreptolysin O and the antideoxyribonuclease B titres.
• Culture of throat swabs and rapid antigen tests for group A streptococcus are less accurate.

Other tests used in the assessment of a patient with suspected ARF include:

• Blood tests – markers of inflammation, such as ESR or CRP, may be raised
• Electrocardiogram and echocardiogram can identify heart involvement

Treatment

Following a diagnosis of rheumatic fever, it is standard practice to treat the group A streptococcal infection that led to the disease with oral penicillin (although this practice has not been proven to alter long-term outcomes).

Following the initial attack, patients are treated with continuous penicillin to prevent further streptococcal colonisation or infection and additional damage to the heart. Continuous penicillin is also recommended for people with established rheumatic heart disease. Continuous penicillin is generally given by injection every four weeks for a minimum of 10 years. Some patients, such as those with severe carditis, may require lifelong treatment.

Patients with rheumatic heart disease may occasionally require heart surgery to repair or replace damaged heart valves.

Prevention

ARF can be prevented with timely treatment of group A streptococcal pharyngitis, particularly in people aged 5-15 years. People with sore throat and fever should see the doctor for advice. Vaccines are currently in development and are eagerly awaited.