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Rheumatic Fever

By Geoffrey A. Weinberg, MD

Rheumatic fever is inflammation of the joints, heart, skin, and nervous system, resulting from a complication of untreated streptococcal infection of the throat.

  • This condition is a reaction to an untreated streptococcal throat infection.

  • Children may have a combination of joint pain, fever, chest pain or palpitations, jerky uncontrollable movements, a rash, and small bumps under the skin.

  • The diagnosis is based on symptoms.

  • Prompt and complete antibiotic treatment of any streptococcal throat infection is the best way to prevent rheumatic fever.

  • Aspirin is given to alleviate pain, and antibiotics are given to eliminate the streptococcal infection.

Although rheumatic fever occurs after a streptococcal throat infection (strep throat), it is not an infection. Rather, it is an inflammatory reaction to the streptococcal infection. The parts of the body most commonly affected by the inflammation include the

  • Joints

  • Heart

  • Skin

  • Nervous system

Most people with rheumatic fever recover, but the heart is permanently damaged in a small percentage of people.

Rheumatic fever can occur at any age but occurs most often between ages 5 years and 15 years. In the United States, rheumatic fever rarely develops before age 3 or after age 21 and is much less common than in developing countries, probably because antibiotics are widely used to treat streptococcal infections at an early stage. However, the incidence of rheumatic fever sometimes rises and falls in a particular area for unknown reasons. Overcrowded living conditions, undernutrition, and lower social and economic status seem to increase the risk of rheumatic fever. Heredity seems to play a part because the tendency to develop rheumatic fever appears to run in families.

In the United States, a child who has a streptococcal throat infection but is not treated has only a less than 1 to 3% chance of developing rheumatic fever. However, about half of the children who have had rheumatic fever develop it again after another streptococcal throat infection if that infection is not treated.

Rheumatic fever occurs after streptococcal infections of the throat but not after those of the skin (impetigo) or other areas of the body. The reasons why are not known.

Symptoms

Symptoms of rheumatic fever vary greatly, depending on which parts of the body become inflamed. Typically, symptoms begin 2 to 3 weeks after the disappearance of throat symptoms. The most common symptoms of rheumatic fever are

  • Joint pain

  • Fever

  • Chest pain or palpitations caused by heart inflammation (carditis)

  • Jerky, uncontrollable movements (Sydenham chorea)

  • Rash

  • Small bumps (nodules) under the skin

A child may have one symptom or several symptoms.

Joints

Joint pain and fever are the most common first symptoms. One or several joints suddenly become painful and feel tender when touched. They may also be warm, swollen, and red. Joints may be stiff and may contain fluid. Ankles, knees, elbows, and wrists are usually affected. The shoulders, hips, and small joints of the hands and feet also may be affected. As pain in one joint lessens, pain in another joint starts (called migratory pain).

Joint pains may be mild or severe and typically last about 2 weeks and rarely more than 4 weeks.

Rheumatic fever does not cause long-term joint damage.

Heart

Some children with heart inflammation have no symptoms, and the inflammation is recognized years later when heart damage is discovered. Some children feel their heart beating rapidly. Other children have chest pain caused by inflammation of the sac around the heart. Children may have a high fever, chest pain, or both.

Heart murmurs are sounds that occur as blood flows through the heart. Children commonly have quiet heart murmurs. However, murmurs that are loud or have changed sometimes mean the child has a heart valve disorder. When rheumatic fever involves the heart, the heart valves are commonly affected, which causes the development of new, larger, or different murmurs that doctors can hear using a stethoscope.

Heart failure may develop, causing the child to feel tired and short of breath, with nausea, vomiting, stomachache, and a hacking, nonproductive cough.

Heart inflammation disappears gradually, usually within 5 months. However, it may permanently damage the heart valves, resulting in rheumatic heart disease. The likelihood of developing rheumatic heart disease varies with the severity of the initial heart inflammation and also depends on whether recurring streptococcal infections are treated.

In rheumatic heart disease, the valve between the left atrium and ventricle (mitral valve) is most commonly damaged. The valve may become leaky ( mitral valve regurgitation), abnormally narrow ( mitral valve stenosis), or both. Valve damage causes the characteristic heart murmurs that enable a doctor to diagnose rheumatic fever. Later in life, usually in middle age, the valve damage may cause heart failure and atrial fibrillation (an abnormal heart rhythm).

Skin

A flat, painless rash with a wavy edge (erythema marginatum) may appear as the other symptoms subside. It lasts for only a short time, sometimes less than a day.

Small, hard, painless lumps (nodules) may form under the skin in children with heart or joint inflammation. The nodules typically appear near the affected joints and go away after a while.

Nervous system

Jerky, uncontrollable movements, usually of both arms and legs and particularly of the face, feet, and hands, called Sydenham chorea may begin gradually in children with rheumatic fever, but usually only after all other symptoms have subsided. A month may go by before the jerky movements become so intense that the child is taken to a doctor. By then, the child typically has rapid, purposeless, sporadic movements that disappear during sleep. The movements may involve any muscle except those of the eyes. They may begin in the hands and spread to the feet and face. Facial grimacing (a distorted expression on the face) is common. Children may cluck their tongue, or the tongue may dart in and out of the mouth.

In mild cases, children may seem clumsy and may have slight difficulties in dressing and eating. In severe cases, children may have to be protected from injuring themselves with their flailing arms or legs. The chorea lasts between 4 months and 8 months.

Diagnosis

  • Established clinical criteria

  • Throat cultures

  • Blood tests

  • Electrocardiography and often echocardiography

A doctor bases the diagnosis of rheumatic fever on the a combination of symptoms and test results called the Modified Jones Criteria (see How Do Doctors Diagnose Rheumatic Fever?).

Doctors do blood tests to look for high levels of antibodies to streptococci. Doctors also look for streptococci by swabbing the child's throat and sending the swab to a laboratory for examination.

Other blood tests, such as the erythrocyte sedimentation rate (ESR) and C-reactive protein, help doctors determine whether inflammation is present in the body and how widespread it is. The ESR and C-reactive protein are increased when inflammation is present.

Doctors do electrocardiography (ECG—recording the heart's electrical activity) to look for abnormal heart rhythms caused by heart inflammation. Doctors may do echocardiography (producing an image of structures in the heart by using ultrasound waves) to diagnose abnormalities of the heart valves and inflammation of the heart.

If doctors are not sure whether a red, swollen joint is caused by a joint infection, rather than rheumatic fever, they may use a needle to remove fluid from the joint ( joint aspiration) and do tests on the fluid.

Prognosis

Rheumatic fever and some of the problems it causes, such as inflammation of the heart and Sydenham chorea, can return. Episodes of Sydenham chorea usually last several months and resolve completely in most people, but the disorder returns in about one third of people. Joint problems (such as pain and swelling) are not permanent, but the heart inflammation can be permanent and severe, especially if streptococcal infections return and are not treated.

Heart murmurs caused by rheumatic fever eventually disappear in some people, but most people have permanent murmurs and some degree of heart valve damage.

Treatment

  • Antibiotics

  • Aspirin

  • Sometimes corticosteroids

Treatment of rheumatic fever has three goals:

  • Eliminating any remaining streptococcal infection

  • Reducing inflammation, particularly in the joints and heart, and thus relieving symptoms

  • Preventing future infections

Doctors give children with rheumatic fever antibiotics to eliminate any remaining infection. A long-acting penicillin is given as a single injection or penicillin or amoxicillin is given by mouth for 10 days.

Aspirin is given in high doses for several weeks to reduce inflammation and pain, particularly if inflammation has reached the joints and heart.

Some other nonsteroidal anti-inflammatory drugs (NSAIDs), such as naproxen, may be as effective as aspirin, but, in most children, aspirin is preferred for the treatment of rheumatic fever.

If heart inflammation is severe, corticosteroids such as prednisone are recommended in addition to aspirin and may be given by vein (intravenously) or by mouth to further reduce inflammation.

Children should limit their activities if they have joint pain, chorea, or heart failure. Children who do not have inflammation of the heart do not need to limit their activities after the illness subsides. Prolonged bed rest is not helpful, and parents should talk about it with their child's doctor.

Preventive treatment (antibiotic prophylaxis)

The best way to prevent rheumatic fever is with prompt and complete antibiotic treatment of any streptococcal throat infection.

In addition, children who have had rheumatic fever should be given drugs (typically penicillin) by mouth every day or by monthly injections into the muscle to help prevent another streptococcal infection. When antibiotics are given to people who do not yet have an infection, this preventive treatment is called prophylaxis. How long this preventive treatment should be continued is unclear. It depends on the severity of the disease and is usually continued for at least 5 years or until age 21 (whichever is longer). Some doctors recommend that it should be continued for life in certain people, such as those who have lasting heart valve damage and have close contact with young children (because the children may carry streptococcal bacteria, which could reinfect such people).

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