Acute rheumatic fever and rheumatic heart disease

ARF most commonly follows an untreated throat infection called ‘strep throat’ caused by Group A Streptococcus germs.

The germs can cause the person’s immune system to fight against its own body tissues (autoimmune response), and in some susceptible people it can lead to ARF.

Remember – strep throat infections are contagious, but ARF is not. People living in overcrowded conditions and having limited access to facilities to wash people, clothes and bedding are at an increased risk of developing Group A Streptococcus.

One or more episodes of ARF can lead to permanent damage to the heart valves.

Repeat episodes of ARF can further damage the heart valves and lead to RHD getting worse.

When the heart valves are damaged, they can affect the heart’s ability to pump blood properly around the body.

This may lead to breathlessness and tiredness and reduce your ability to do some activities.

ARF is relatively rare in developed countries but it remains a significant illness in Australia among Aboriginal people and Torres Strait Islanders, particularly across areas of central and northern Australia (our highest rates in WA are in the Kimberley and Pilbara regions).

Anyone can be infected with the Group A Streptococcus bacteria, but children aged 5 to 14 years are most at risk of getting ARF.

Although rare, recurrences of this illness can occur into a person's 40s.

Other groups at risk include:

• Maori and Pacific Islander people
• migrants and refugees from low and middle income countries.

Signs and symptoms may appear 2 to 3 weeks following a sore throat (strep throat) or skin infections and may include:

• sore or swollen joints (commonly knees, elbows, ankles, wrist)
• fever
• skin rash (rare), particularly over the trunk area which can be difficult to see on some dark skin colours
• skin nodules (rare)
• jerky movements which can involve one or both arms, legs, head or mouth – this is called Sydenham’s chorea.

In more severe cases of ARF, inflammation of the heart (carditis) may develop and present as tiredness and breathlessness.

All symptoms will gradually resolve over several weeks to months, except sometimes permanent damage will occur to the heart valves. It is important to keep getting your regular injections for as long as your health professional advises you, even if the symptoms go away.

Each repeat episode of ARF can lead to progressive damage to the heart. This is rheumatic heart disease (RHD). When the damage to the heart valves is severe then surgery may be required to repair or replace the faulty heart valves.

Currently there is no test to determine if you have ARF. Health care professionals will use the information you provide and results from blood tests to make a diagnosis.

Your doctor may order a special heart test called an echocardiogram which will determine if there is damage to the heart.

See your doctor or go to hospital as soon as possible if you suspect you have ARF, especially if a family member has also had ARF. Early recognition and prompt treatment is necessary to prevent further damage to your heart.

ARF has been a notifiable disease since 2015. This means doctors, nurse practitioners and hospitals must inform the Department of Health of your diagnosis and related treatments.

If doctors think you have ARF you will most likely be sent to hospital for admission while they run more tests.

In most cases your treatment will involve:

• Benzathine benzylpenicillin G injections. These are antibiotic injections given into the muscle to help prevent further Group A Streptoccocus infections, and therefore further episodes of ARF. It is the only available treatment known to reduce the risk of permanent heart damage (RHD).  Injections are given at least every 21 to 28 days, for potentially many years depending on the severity of the disease. Your heart doctor (cardiologist) will determine how long you should continue to receive penicillin injections.
• medicines to stop pain in your joints.
• bed rest may be necessary if symptoms are severe.
• you will also have a scan of your heart valves (echocardiogram) done to check for damage. 

If left untreated

People who have had ARF are more susceptible to repeat ARF episodes if they have further Group A Streptococcus infections. Each episode of ARF can damage the heart valves which can lead to:

• lifelong health problems
• a need for heart surgery
• early death.

It is important to have your benzathine benzylpenicillin G injections every 21 to 28 days until your doctor says you can stop. You must keep your appointments with specialist doctors who will monitor the effects of the disease on your heart.

It is very important to keep your teeth and gums healthy as this will help you to prevent germs getting into your body and blood stream (which could damage your heart valves further).

See a dentist at least once a year and tell them you have ARF or RHD. You may need to take medicine before certain dental procedures to protect the heart from germs that can damage the heart further. Keep up to date with your immunisations to protect you against influenza and see your local doctor regularly. See your doctor immediately if you have RHD and become pregnant  as this can add significant strain to your heart.

People most at risk should have all throat and skin infections checked and treated as soon as possible by a health professional. Antibiotics may be prescribed to treat the Group A Streptococcus infection.

Stopping the spread of Group A Streptococcus is particularly important. Washing your hands and body thoroughly with soap and water, washing clothes and bedding regularly, and reducing overcrowding in houses where possible are some of the best ways to limit the spread of bacteria.

Once you have had ARF, having regular penicillin injections at least every 21 to 28 days is the best way to prevent repeat episodes of ARF. This will also help to prevent long-term damage to the heart valves.

Regular medicine can also prevent any existing damage to the heart valves (RHD) from getting worse. Have regular heart scans (echocardiogram) to check your heart valves.