Rheumatic Fever

What is rheumatic fever?

Rheumatic fever is an inflammatory illness that can develop a few weeks after a throat infection caused by a bacterium called group A streptococcus (the germ behind strep throat and scarlet fever). It is not an infection in itself. Instead, it happens when the body’s immune system, while fighting the bacteria, mistakenly attacks the body’s own tissues. The joints, heart, skin, and brain are the areas most often affected.

The condition is most common in children and adolescents between about 5 and 15 years of age, although it can occasionally occur in younger children and in adults. It is now uncommon in many high-income countries, largely because strep throat is usually recognized and treated with antibiotics. It remains a significant health problem in parts of the world where access to timely medical care is limited.

The most serious long-term concern with rheumatic fever is damage to the heart valves, a condition known as rheumatic heart disease. Because of this, people who have had rheumatic fever are usually followed closely by a heart specialist. In hospital groups such as Acibadem, this follow-up is typically managed through the Cardiology Department, often alongside pediatric and infectious disease teams.

Rheumatic fever symptoms

Rheumatic fever symptoms usually begin two to four weeks after a strep throat infection. In some cases the original sore throat was mild or went unnoticed, which can make the connection harder to spot. Symptoms vary a great deal from person to person, and not everyone develops every feature. Commonly reported signs include:

• Fever, which may be mild or high
• Painful, swollen, warm joints, most often the knees, ankles, elbows, and wrists, with pain that tends to move from one joint to another over days
• Chest pain, breathlessness, or a racing heartbeat, which may suggest inflammation of the heart (carditis)
• Tiredness and feeling generally unwell
• Jerky, uncontrollable movements of the hands, feet, or face, known as Sydenham chorea, sometimes with emotional outbursts or unusual behavior
• A painless, flat or slightly raised rash with ragged edges, called erythema marginatum, usually on the trunk or limbs
• Small, painless lumps under the skin (subcutaneous nodules) near joints or along the spine
• Nosebleeds and abdominal pain, which are less specific but sometimes occur

Doctors often group these features into “major” and “minor” signs. The major signs are heart inflammation, arthritis affecting several joints, chorea, the characteristic rash, and skin nodules. Minor signs include fever, joint aches without swelling, and certain blood test or heart tracing changes. The way the illness presents can differ by stage:

• Early or acute stage: Fever and joint pain are usually the first and most obvious complaints. Heart inflammation may cause few or no symptoms at this point and is sometimes found only on examination or an ultrasound of the heart.
• Delayed features: Chorea often appears later than the other signs, sometimes months after the original infection, and may occur on its own without joint or heart involvement.
• Long-term stage: If the heart valves are scarred, symptoms such as breathlessness on exertion, swelling of the ankles, or palpitations may develop years later as rheumatic heart disease progresses.

Joint symptoms and the rash generally settle over a few weeks without lasting damage. Heart involvement is the feature that determines the long-term outlook, which is why it receives the most attention during diagnosis and follow-up.

Causes and risk factors

The direct cause of rheumatic fever is an abnormal immune response to a group A streptococcal infection of the throat. When the immune system produces antibodies (protective proteins) to attack the bacteria, some of these antibodies also react against proteins in the body’s own tissues that look similar to those on the bacteria. This process, sometimes described as molecular mimicry, leads to inflammation in the joints, heart, skin, and nervous system.

It is important to understand that rheumatic fever does not follow every case of strep throat, and it does not usually follow strep infections of the skin. Only a small proportion of people who have an untreated or incompletely treated throat infection go on to develop the condition. Factors that increase the likelihood include:

• Untreated or partially treated strep throat, including stopping antibiotics early
• Age between roughly 5 and 15 years
• Repeated strep throat infections
• Crowded living conditions, which make the spread of streptococcal bacteria more likely
• Limited access to medical care, so infections are not diagnosed or treated promptly
• A family history of rheumatic fever, suggesting some people may be genetically more susceptible
• A previous episode of rheumatic fever, which greatly raises the risk of another episode after a new strep infection

Rheumatic fever itself is not contagious. However, the strep throat that triggers it spreads easily from person to person through coughing, sneezing, and close contact.

Rheumatic fever diagnosis

There is no single test that confirms rheumatic fever. Doctors make the diagnosis by combining the history of a recent throat infection, the pattern of symptoms, a physical examination, and the results of several tests. Internationally, many clinicians use a set of criteria known as the Jones criteria, which require evidence of a preceding streptococcal infection together with a particular combination of the major and minor signs described above.

Tests and assessments that are commonly used include:

• Throat swab: A rapid antigen test or a throat culture can detect group A streptococcus if the bacteria are still present. By the time rheumatic fever develops, the swab is often negative because the infection has already cleared.
• Blood antibody tests: Tests such as anti-streptolysin O (ASO) and anti-DNase B measure antibodies the body has made against streptococcal bacteria. Rising or high levels support the diagnosis of a recent infection.
• Inflammation markers: Blood tests such as erythrocyte sedimentation rate (ESR) and C-reactive protein (CRP) show how much inflammation is present in the body.
• Electrocardiogram (ECG): This tracing of the heart’s electrical activity can reveal changes such as a prolonged PR interval, a delay in the signal traveling through the heart, or abnormal rhythms.
• Echocardiogram: This ultrasound scan of the heart is the main way to detect inflammation of the heart muscle or valves and to check for leaking valves. It may show valve involvement even when there is no heart murmur on examination.
• Chest X-ray: This may be used to look for an enlarged heart or fluid in the lungs when heart failure is suspected.

Because joint pain and fever have many possible causes, doctors also consider and rule out other conditions such as viral infections, juvenile arthritis, Lyme disease, and other forms of heart inflammation. In children with chorea, other neurological causes may be considered as well. In people who have had rheumatic fever before, a repeat episode can be diagnosed with fewer criteria, since the risk of recurrence is known to be higher.

Rheumatic fever treatment options

Treatment aims to clear any remaining streptococcal bacteria, reduce inflammation and relieve symptoms, protect the heart, and prevent future episodes. The exact approach depends on which parts of the body are affected and how severely. Rheumatic fever treatment options generally fall into the following groups.

Antibiotics

Even if the throat swab is negative, doctors usually prescribe a course of antibiotics, most often penicillin or a related drug, to make sure the streptococcal infection is fully eliminated. People who are allergic to penicillin may be given an alternative antibiotic.

Anti-inflammatory medication

Joint pain, swelling, and fever are commonly treated with anti-inflammatory medicines such as aspirin or non-steroidal anti-inflammatory drugs (NSAIDs). Aspirin is generally avoided in children for most other illnesses because of a rare complication called Reye syndrome, but it has traditionally been used in rheumatic fever under close medical supervision. Your doctor will decide which medicine is appropriate. In cases of severe heart inflammation, corticosteroids (strong anti-inflammatory hormones) may be used.

Managing heart involvement

If the heart is inflamed, doctors may recommend rest and limited physical activity until the inflammation settles. If heart failure develops, meaning the heart cannot pump effectively, medicines such as diuretics (water tablets) and other heart medications may be needed. Regular echocardiograms help the care team track how the valves are recovering.

Treatment of chorea

Sydenham chorea usually improves on its own over weeks to months. When the movements are severe or interfere with daily life, medicines that calm abnormal movements, such as certain anti-seizure or sedating drugs, may be prescribed. A quiet, low-stress environment is often recommended.

Long-term preventive antibiotics

One of the most important parts of treatment is preventing another episode. Each recurrence increases the risk of permanent heart damage. For this reason, doctors usually recommend ongoing preventive antibiotics, known as secondary prophylaxis. This is most often given as a penicillin injection every three to four weeks, or sometimes as daily oral tablets. The recommended duration varies: it often continues for at least several years after the last episode or until early adulthood, and it may be lifelong for people with significant valve damage. Your doctor will advise on the duration that suits your situation.

Surgery and procedures

Surgery is not needed for the acute illness itself. However, if rheumatic heart disease develops and a valve becomes badly narrowed or leaky over time, procedures such as balloon valvotomy (widening a narrowed valve with a balloon), valve repair, or valve replacement may be considered. These decisions are made by a cardiology and heart surgery team based on symptoms and echocardiogram findings, usually many years after the original episode.

Follow-up and rehabilitation

Recovery from the acute illness is typically gradual, with a return to normal activity as symptoms allow. Regular check-ups, including heart examinations and periodic echocardiograms, are usually recommended even when the person feels well. People with damaged valves may also be advised about dental care and other measures to reduce the risk of infection settling on the valves.

Living with rheumatic fever and outlook

Most people recover from the acute phase of rheumatic fever within weeks to a few months. Joint symptoms, the rash, and nodules almost always resolve completely. Chorea usually disappears as well, although it can occasionally return.

The long-term outlook depends mainly on whether, and how badly, the heart was affected. Many people who had no or mild heart inflammation during the acute illness go on to lead normal lives without lasting problems. When the valves are damaged, the effects may range from a mild leak that never causes symptoms to progressive narrowing or leaking that requires medication or surgery later in life. Valve damage can worsen slowly over years, which is why regular monitoring is advised even in the absence of symptoms.

Taking preventive antibiotics consistently is the single most effective step a person can take to protect their heart, because repeated episodes are the main driver of worsening valve disease. Many families find that setting reminders and coordinating injections with routine appointments makes long-term treatment easier to maintain.

Day-to-day life is generally not restricted once the acute illness has settled, although people with significant heart involvement may receive individual advice about exercise. Women who have had rheumatic heart disease are usually advised to discuss any planned pregnancy with their doctor, as pregnancy places extra demands on the heart. Good dental hygiene and prompt treatment of any future sore throats are also commonly recommended.

Frequently asked questions

What is rheumatic fever and is it the same as scarlet fever?

Rheumatic fever and scarlet fever are related but different. Scarlet fever is a direct streptococcal infection that causes a sore throat and a characteristic rash. Rheumatic fever is a delayed immune reaction that can follow strep throat or scarlet fever a few weeks later, affecting the joints, heart, skin, and nervous system. Treating the original infection promptly is thought to lower the chance of rheumatic fever developing.

What are the first rheumatic fever symptoms to look out for?

The earliest signs are often a fever and painful, swollen joints that appear two to four weeks after a sore throat. The joint pain frequently moves from one large joint to another. Some children develop chest discomfort, breathlessness, or tiredness that may indicate heart involvement, while others show unusual jerky movements. Because these features are not unique to rheumatic fever, a medical assessment is needed to confirm the cause.

What causes rheumatic fever to develop after strep throat?

Rheumatic fever causes are linked to the immune system rather than to the bacteria directly damaging tissue. Antibodies made to fight group A streptococcus can cross-react with similar proteins in the heart, joints, and brain, producing inflammation. Only a minority of people with untreated strep throat develop this reaction, and researchers believe inherited factors influence who is affected.

How is rheumatic fever diagnosis confirmed?

There is no single confirmatory test. Doctors combine evidence of a recent strep infection, obtained from a throat swab or blood antibody tests, with a recognized pattern of clinical signs such as arthritis, heart inflammation, chorea, rash, or nodules. Blood tests for inflammation, an ECG, and an echocardiogram are commonly used to support the diagnosis and assess the heart.

What are the main rheumatic fever treatment options?

Treatment usually includes antibiotics to clear the streptococcal infection, anti-inflammatory medicines for joint pain and fever, and rest. Corticosteroids may be used for severe heart inflammation, and specific medicines may help control chorea. Long-term preventive antibiotics are typically recommended to reduce the risk of recurrence, and heart valve procedures may be considered years later if rheumatic heart disease develops.

Can rheumatic fever come back?

Yes. People who have had rheumatic fever are at higher risk of another episode if they catch strep throat again, and each recurrence raises the likelihood of permanent heart valve damage. This is the main reason doctors recommend regular preventive antibiotic injections or tablets for several years, and sometimes longer, after the first episode.

Is rheumatic fever contagious?

Rheumatic fever itself cannot be passed from one person to another. However, the strep throat infection that triggers it is contagious and spreads through respiratory droplets and close contact. Prompt diagnosis and treatment of sore throats in household members can help limit the spread of the bacteria.

When to see a doctor

Anyone with a sore throat accompanied by fever, swollen glands, or a rash, especially a child, should be assessed by a doctor so that a streptococcal infection can be identified and treated. If joint pain, fever, or unusual symptoms develop in the weeks after a sore throat, a medical review is advisable even if the throat has recovered. People with a history of rheumatic fever should seek advice promptly for any new sore throat or fever, and should not stop preventive antibiotics without discussing it with their doctor.

Seek urgent medical attention if any of the following occur:

• Chest pain or pressure, particularly if it worsens with breathing or lying down
• Severe or sudden breathlessness, or difficulty breathing when lying flat
• Fainting, near-fainting, or a very fast or irregular heartbeat
• Blue or gray lips or fingertips
• Rapidly increasing swelling of the legs, ankles, or abdomen
• High fever with confusion, drowsiness, or a stiff neck
• Sudden, severe, or worsening uncontrolled movements that affect swallowing, speech, or safety
• A joint that is hot, extremely painful, and cannot be moved, which may signal a joint infection rather than rheumatic fever

These signs may indicate serious heart or neurological complications that need immediate assessment. When in doubt, it is safer to be examined promptly than to wait.