Acute rheumatic fever (ARF) and rheumatic heart disease (RHD)

What is acute rheumatic fever?#

Acute rheumatic fever (ARF) is an abnormal immune response that, in susceptible people, occurs about two to three weeks after an untreated group A streptococcal infection. The immune reaction causes the symptoms of ARF. People can’t catch ARF from someone else, because it is an immune response and not an infection itself.

A person can be infected with group A Streptococcus many times over their lifetime and can experience repeated episodes of ARF. The immune response can cause inflammation (swelling) in several organs, including the joints, heart, brain and skin.

How ARF leads to rheumatic heart disease#

Damage to the heart can persist after a person recovers from ARF. This permanent damage is known as rheumatic heart disease (RHD).

RHD can develop after a single bout of ARF, but it is more often associated with recurrent or severe episodes. The part of the heart most commonly damaged is the valves, although other parts of the heart can also be affected. Damaged valves can’t open and close properly, which means the heart may be unable to pump blood effectively.

Other complications of RHD include:

• Infection of damaged heart valves (infective endocarditis)
• Heart failure, when the heart cannot pump well enough to meet the body’s needs
• Stroke, caused by clots forming in the heart or on damaged valves

Who is affected#

ARF is more common in children aged 5 to 15 years and is rare in adults. RHD can be diagnosed in children, adolescents and adults. People who have had ARF in the past are more likely to get it again.

ARF and RHD can affect anyone, but they are more common in some populations and in lower-income settings. Risk factors include poverty, overcrowding and reduced access to medical care. ARF is sometimes undiagnosed, and a failure to recognize it — together with limited access to healthcare — can lead to under-diagnosis and a missed chance to prevent RHD.

Symptoms#

Symptoms of ARF#

Symptoms of ARF can include:

• Fever and fatigue
• Swollen, painful joints (arthritis)
• Uncontrollable body movements (chorea)
• Skin changes, including painless lumps or a rash
• Symptoms caused by heart damage, including shortness of breath or a racing heartbeat

Symptoms of RHD#

RHD often does not cause symptoms. When it does, the most common are shortness of breath and a racing heartbeat caused by heart damage.

Diagnosis#

There is no single test for ARF. To diagnose it, a doctor takes a medical history, does a physical examination, and may order:

• Throat or skin swabs to look for group A streptococcal infection
• Blood tests to look for signs of inflammation and a recent group A strep infection
• An electrocardiogram (ECG) or echocardiogram (echo) to check for damage to the heart

RHD is usually diagnosed with an echocardiogram (an ultrasound of the heart that can assess the valves and look for heart failure). Doctors also take a medical history, perform a physical examination, and may order additional tests.

Treatment#

ARF symptoms can be treated with medicines for pain, fever and inflammation, and care may involve admission to hospital.

Treatment of RHD aims to manage symptoms and prevent complications. Management may involve:

• Blood-thinning medicine to prevent stroke after valve replacement, or if an abnormal heart rhythm develops
• Antibiotics before some dental or surgical procedures to prevent infection of the heart
• Good antenatal care during pregnancy
• Vaccinations (such as against pneumococcal infection) to prevent other illnesses that may strain the heart
• Good dental hygiene — tooth brushing, flossing and regular dental check-ups — to stop mouth bacteria entering the blood and infecting damaged heart valves

Prevention#

Preventing ARF and RHD is important because these diseases can have long-term or permanent effects, even when treated.

ARF can be prevented by:

• Improving living conditions for those at risk, including reducing household overcrowding and providing clean facilities for washing hands and bodies
• Promptly diagnosing and treating group A streptococcal throat and skin infections, especially among people at higher risk

RHD can be prevented by the same measures, and by giving regular antibiotics (secondary prophylaxis) to people who have had ARF to prevent further group A streptococcal infections.

Key Points#

• People can’t catch ARF from someone else, because it is an immune response and not an infection.
• ARF most commonly damages the heart valves, although other parts of the heart can also be affected; this lasting damage is called rheumatic heart disease.
• ARF and RHD can affect anyone but are more common in some populations and lower-income settings, where overcrowding and limited access to care play a role.
• ARF can go undiagnosed, which can mean RHD is not prevented or recognized.
• Symptoms of ARF can include fever, swollen painful joints, uncontrollable movements, skin changes such as painless lumps or a rash, and signs of heart damage.