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Rheumatic fever

Definition

Rheumatic fever is an inflammatory disease that may develop after an infection with Streptococcus bacteria (such as strep throat or scarlet fever). The disease can affect the heart, joints, skin, and brain.

Alternative Names

Acute rheumatic fever

Causes

Rheumatic fever is common worldwide and is responsible for many cases of damaged heart valves. It is not common in the U.S., and usually occurs in isolated outbreaks. The latest outbreak was in the 1980s.

Rheumatic fever mainly affects children ages 6 -15, and occurs approximately 20 days after strep throat or scarlet fever.

Symptoms

• Abdominal pain
• Fever
• Heart (cardiac) problems, which may not have symptoms, or may result in shortness of breath and chest pain
• Joint pain, arthritis (mainly in the knees, elbows, ankles, and wrists)
• Joint swelling; redness or warmth
• Nosebleeds (epistaxis)
• Skin nodules
• Skin rash (erythema marginatum)
  • Skin eruption on the trunk and upper part of the arms or legs
  • Eruptions that look ring-shaped or snake-like
• Sydenham chorea (emotional instability, muscle weakness and quick, uncoordinated jerky movements that mainly affect the face, feet, and hands)

Exams and Tests

Because this disease has different forms, no one test can firmly diagnose it. Your doctor will perform a careful exam, which includes checking your heart sounds, skin, and joints.

Tests may include:

• Blood test for recurrent strep infection (such as an ASO test)
• Complete blood count
• Electrocardiogram
• Sedimentation rate (ESR)

Several major and minor criteria have been developed to help standardize rheumatic fever diagnosis. Meeting these criteria, as well as having evidence of a recent streptococcal infection, can help confirm that you have rheumatic fever.

The major criteria for diagnosis include:

• Arthritis in several joints (polyarthritis)
• Heart inflammation (carditis)
• Nodules under the skin (subcutaneous skin nodules)
• Rapid, jerky movements (chorea, Sydenham chorea)
• Skin rash (erythema marginatum)

The minor criteria include:

• Fever
• High ESR
• Joint pain
• Other laboratory findings

You'll likely be diagnosed with rheumatic fever if you meet two major criteria, or one major and two minor criteria, and have signs that you've had a previous strep infection.

Treatment

If you are diagnosed with acute rheumatic fever you will be treated with antibiotics.

Anti-inflammatory medications such as aspirin or corticosteroids reduce inflammation to help manage acute rheumatic fever.

You may have to take low doses of antibiotics (such as penicillin, sulfadiazine, or erythromycin) over the long term to prevent strep throat from returning.

Outlook (Prognosis)

Rheumatic fever is likely to come back in people who don't take low-dose antibiotics continually, especially during the first 3 -5 years after the first episode of the disease. Heart complications may be severe, particularly if the heart valves are involved.

Possible Complications

• Arrhythmias
• Damage to heart valves (in particular, mitral stenosis and aortic stenosis)
• Endocarditis
• Heart failure
• Pericarditis
• Sydenham chorea

When to Contact a Medical Professional

Call your health care provider if you develop symptoms of rheumatic fever. Because several other conditions have similar symptoms, you will need careful medical evaluation.

If you have symptoms of strep throat, tell your health care provider. You will need to be evaluated and treated if you do have strep throat, to decrease your risk of developing rheumatic fever.

Prevention

The most important way to prevent rheumatic fever is by getting quick treatment for strep throat and scarlet fever.

References

Gerber MA. Group A Streptococcus. In: Kliegman RM, Behrman RE, Jenson HB, Stanton BF, eds. Nelson Textbook of Pediatrics. 18th ed. Philadelphia, Pa: Saunders Elsevier; 2007:chap 182.

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