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Rheumatic fever

Rheumatic fever is an inflammatory disease that can develop as a complication of inadequately treated strep throat or scarlet fever. Strep throat and scarlet fever are caused by an infection with group A streptococcus bacteria.

Rheumatic fever is most common in 5- to 15-year-old children, though it can develop in younger children and adults. Although strep throat is common, rheumatic fever is rare in the United States and other developed countries. However, rheumatic fever remains common in many developing nations.

Rheumatic fever can cause permanent damage to the heart, including damaged heart valves and heart failure. Treatments can reduce tissue damage from inflammation, lessen pain and other symptoms, and prevent the recurrence of rheumatic fever.

Symptoms Causes Risk factors Complications Prevention

Rheumatic fever symptoms may vary. Some people may have several symptoms, while others experience only a few. The symptoms may also change during the course of the disease. The onset of rheumatic fever usually occurs about two to four weeks after a strep throat infection.

Rheumatic fever signs and symptoms — which result from inflammation in the heart, joints, skin or central nervous system — may include:

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

When to see a doctor

Your child should see a doctor if he or she has signs or symptoms of strep throat. Proper treatment of strep can prevent rheumatic fever. Call your doctor if your child has any of the following signs or symptoms:

  • A sore throat without cold symptoms, such as a runny nose
  • A sore throat accompanied by tender, swollen lymph glands (nodes)
  • A red rash that starts at the head and neck, then expands to the trunk and extremities
  • Difficulty swallowing anything, including saliva
  • Thick or bloody discharge from the nose, which is more likely in children under 3 years of age
  • A bright red tongue with bumps all over it, known as strawberry tongue

Call your doctor about a fever in the following situations:

  • Newborns up to 3 months old with a fever of 100.4 F (38 C) taken rectally
  • Children ages 3 to 6 months with a temperature of 102 F (38.9 C) or higher
  • Children ages 6 months to 2 years with a fever of 102 F (38.9 C) or higher that doesn't respond to medication or lasts more than one day
  • Children ages 2 to 17 years with a fever of 102 F (38.9 C) or higher (taken rectally for children younger than 3 and orally for children older than 3) that doesn't respond to medication or lasts more than three days

Also, see your doctor if your child shows any other signs or symptoms of rheumatic fever.

Rheumatic fever can occur after an infection of the throat with a bacterium called Streptococcus pyogenes, or 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 exact link between strep infection and rheumatic fever isn't clear, but it appears that the bacterium "plays tricks" on the immune system. The strep bacterium contains a protein similar to one found in certain tissues of the body. Therefore, immune system cells that would normally target the bacterium may treat the body's own tissues as if they were infectious agents — particularly tissues of the heart, joints, skin and central nervous system. This immune system reaction results in inflammation.

If your child receives prompt and complete treatment with an antibiotic to eliminate strep bacteria — in other words, taking all doses of the medication as prescribed — there's little to no chance of developing rheumatic fever. If your child has one or more episodes of strep throat or scarlet fever that aren't treated or not treated completely, he or she may — but won't necessarily — develop rheumatic fever.

Factors that may increase the risk of rheumatic fever include:

  • Family history. Some people may carry a gene or genes that make them more likely to develop rheumatic fever.
  • 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 may easily result in the rapid transmission or multiple exposures to strep bacteria.

Inflammation caused by rheumatic fever may last for a few weeks to several months. In some cases, the inflammation may cause long-term complications.

Rheumatic heart disease is permanent damage to the heart caused by the inflammation of rheumatic fever. Problems are most common with the valve between the two left chambers of the heart (mitral valve), but the other valves may be affected. The damage may result in one of the following conditions:

  • Valve stenosis. This condition is a narrowing of the valve, which results in decreased blood flow.
  • Valve regurgitation. This condition is a leak in the valve, which allows blood to flow in the wrong direction.
  • Damage to heart muscle. The inflammation associated with rheumatic fever can weaken the heart muscle, resulting in poor pumping function.

Damage to the mitral valve, other heart valves or other heart tissues can cause problems with the heart later in life. Resulting conditions may include:

  • Atrial fibrillation, an irregular and chaotic beating of the upper chambers of the heart (atria)
  • Heart failure, an inability of the heart to pump enough blood to the body

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

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