Rheumatic fever

Rheumatic fever symptoms result from inflammation in the heart, joints, skin or central nervous system. There may be few symptoms or several. Symptoms can change during the course of the disease. The onset of rheumatic fever usually occurs about 2 to 4 weeks after a strep throat infection.

Rheumatic fever signs and symptoms can include:

• Fever
• Painful and tender joints — most often in the knees, ankles, elbows and wrists
• Pain in one joint that migrates to another joint
• Red, hot or swollen joints
• Chest pain
• Fatigue
• Flat or slightly raised, painless rash with a ragged edge
• Heart murmur
• Jerky, uncontrollable body movements (Sydenham chorea) — most often in the hands, feet and face
• Outbursts of unusual behavior, such as crying or inappropriate laughing, that accompanies Sydenham chorea
• Small, painless bumps beneath the skin

When to see a doctor

Make an appointment with your child's health care provider if any of these signs or symptoms of strep throat occur:

• Sore throat that comes on suddenly
• Pain when swallowing
• Fever
• Headache
• Stomach pain, nausea and vomiting

Proper treatment of strep throat can prevent rheumatic fever.

Rheumatic fever can occur after a throat infection from a bacteria called group A streptococcus. Group A streptococcus infections of the throat cause strep throat or, less commonly, scarlet fever.

Group A streptococcus infections of the skin or other parts of the body rarely trigger rheumatic fever.

The link between strep infection and rheumatic fever isn't clear. It appears that the bacteria trick the immune system into attacking otherwise healthy tissue.

The body's immune system typically targets infection-causing bacteria. In rheumatic fever, the immune system mistakenly attacks healthy tissue, particularly in the heart, joints, skin and central nervous system. This faulty immune system reaction results in swelling of the tissues (inflammation).

There's little chance of developing rheumatic fever when a strep throat infection is promptly treated with antibiotics and all the medication is taken as prescribed.

If a child has one or more episodes of strep throat or scarlet fever that aren't properly treated, rheumatic fever may occur.

Things that may increase the risk of rheumatic fever include:

• Genes. Some people have one or more genes that might make them more likely to develop rheumatic fever.
• Specific type of strep bacteria. Certain strains of strep bacteria are more likely to contribute to rheumatic fever than are other strains.
• Environmental factors. A greater risk of rheumatic fever is associated with overcrowding, poor sanitation and other conditions that can cause strep bacteria to easily spread among many people.

Inflammation caused by rheumatic fever can last a few weeks to several months. For some people, the inflammation causes long-term complications.

One complication of rheumatic fever is permanent damage to the heart (rheumatic heart disease). Rheumatic heart disease usually occurs years to decades after the original illness.

However, severe rheumatic fever can start to damage the heart valves while your child still has symptoms of the infection. Damage is most common with the valve between the two left chambers of the heart (mitral valve), but the other valves can be affected.

Rheumatic fever can cause the following types of heart damage:

• Narrowing of a heart valve (valve stenosis). This decreases blood flow.
• Leaky heart valve (valve regurgitation). Blood flows backward across the valve.
• Damage to heart muscle. The inflammation associated with rheumatic fever can weaken the heart muscle, affecting its ability to pump.

Damage to the heart valves or other heart tissues can lead to irregular, chaotic heartbeats (atrial fibrillation) or heart failure later in life.

The only way to prevent rheumatic fever is to treat strep throat infections or scarlet fever promptly and completely with a full course of appropriate antibiotics.

There's no single test for rheumatic fever. Diagnosis of rheumatic fever is based on medical history, a physical exam and certain test results. Tests for rheumatic fever might include:

• Blood tests. Blood tests can be done to check for signs (markers) of inflammation in the blood. These tests include C-reactive protein and the erythrocyte sedimentation rate.

  Sometimes, a blood test that can detect antibodies to the strep bacteria in the blood is done. The actual bacteria might no longer be detectable in the throat tissues or blood.

• Electrocardiogram (ECG or EKG). This test records the electrical activity of the heart. It can help diagnose irregular heartbeats and can help a health care provider determine if parts of the heart may be enlarged.
• Echocardiogram. Sound waves are used to create pictures of the heart in motion. An echocardiogram shows how blood flows through the heart and heart valves.

The goals of treatment for rheumatic fever are to treat the infection, relieve symptoms, control inflammation and prevent the condition from returning.

Treatment for rheumatic fever may include:

• Antibiotics. Penicillin or another antibiotic is typically prescribed to treat the strep bacteria.

  After the first antibiotic treatment is fully finished, a provider typically prescribes another course of antibiotics to prevent recurrence of rheumatic fever. Preventive treatment will likely continue through age 21 or until a child completes a minimum five-year course of treatment, whichever is longer.

  People who have had heart inflammation during rheumatic fever might need to continue preventive antibiotic treatment for 10 years or longer.

• Anti-inflammatory drugs. Aspirin or naproxen (Naprosyn, Naprelan, Anaprox DS) can help reduce inflammation, fever and pain. If symptoms are severe or a child doesn't get better with anti-inflammatory medicines, a corticosteroid might be prescribed. Don't give a child aspirin unless a care provider tells you to do so.
• Antiseizure drugs. Medications such as valproic acid or carbamazepine (Carbatrol, Tegretol, others) may be used to treat severe involuntary movements caused by Sydenham chorea.

Long-term care

Discuss with your care provider what type of follow-up and long-term care your child will need for rheumatic fever.

Heart damage from rheumatic fever, called rheumatic heart disease, might not show up for many years — even decades. Always tell your health care provider about any history of rheumatic fever.

A health care provider might recommend bed rest for a child with rheumatic fever. Activities may be restricted until inflammation, pain and other symptoms have improved. If inflammation affects the heart, strict bed rest may be recommended for a few weeks to a few months.

If your child has signs or symptoms of rheumatic fever, you're likely to start by seeing your child's pediatrician. Your child may be referred to a heart specialist (pediatric cardiologist) for some diagnostic tests.

Here's some information to help you get ready for the appointment.

What you can do

Before the appointment, make a list of:

• Your child's symptoms, including any that seem unrelated to your reason for scheduling the appointment and any that have recently been resolved
• Recent illnesses your child has had
• All medications, vitamins or other supplements your child takes or has recently taken, including doses
• Questions to ask the care provider

For rheumatic fever, basic questions to ask the health care provider include:

• What's likely causing my child's symptoms?
• What other conditions could cause these symptoms?
• What tests will my child need?
• What is the best course of action?
• Will rheumatic fever or its treatment affect my child's other health conditions?
• How much do I need to restrict my child's activities?
• Is my child still contagious? For how long?
• What type of follow-up is needed?
• Are there brochures or other printed material that I can have? What websites do you recommend?

In addition to the questions that you've prepared to ask the health care provider, don't hesitate to ask questions during your appointment.

What to expect from your doctor

The health care provider is likely to ask questions, such as:

• When did your child's symptoms begin?
• How have they changed over time?
• Has your child had a cold or flu recently? What were the symptoms?
• Has your child been exposed to strep throat?
• Was your child recently diagnosed with strep throat or scarlet fever?
• If so, did your child take all of the antibiotics as prescribed?