Rheumatic fever

What is Rheumatic fever?

Rheumatic fever is a disease that causes inflammation in various tissues throughout the body. It occurs in children and young adults following a throat infection with group A Streptococci.

Rheumatic fever is not caused by the presence of the bacteria in the affected tissues. Instead, the bacteria appear to trigger an autoimmune reaction in which the body's immune system mistakenly attacks its own tissues. Joint inflammation occurs, but does not cause crippling effects. The central nervous system may also be affected. Most important is the frequency with which the disease permanently damages the heart. Vigorous and complete antibiotic treatment of streptococcal throat infections will largely prevent the occurrence of rheumatic fever.

Rheumatic fever is rare in most developed countries, although it has been reported to be on the rise again in the US. A small minority of individuals appear to have a genetic predisposition to the development of acute rheumatic fever.

How is it diagnosed?

History: A week or two after a streptococcal throat infection, the initial symptoms of rheumatic fever may suddenly appear.

Symptoms include fever, joint pain, loss of appetite, weakness, and shortness of breath. Inflammation and swelling affects the large joints such as the knees, elbows, ankles and wrists. As inflammation in one joint recedes, symptoms tend to develop in another. Several joints may be affected simultaneously. Individuals may report a transient pink rash with slightly raised edges (erythema marginatum). Pea-sized nodules located under the skin may develop over tendons, joints, and bony prominences. Damage to the heart does not always occur. When it does occur, it develops so insidiously that there may be no symptoms until years later.

Physical exam may reveal red, swollen joints and pea-sized nodules beneath the skin (often over bony prominences). Symptoms of carditis (inflammation of the heart muscle) include a rapid heartbeat (tachycardia) that persists during sleep and markedly increases with slight activity, irregular heart rhythms (arrhythmias), changing quality of heart sounds, and heart murmurs.

Damage to the heart may not be apparent until years later. The most common and most serious related symptom is a thickening and scarring of the heart valves, leading to narrowing (mitral stenosis) or leaking (mitral insufficiency) of the heart valves. These changes are permanent and progressive. Heart valve surgery may be needed.

Tests: There are no specific tests for rheumatic fever. Diagnosis relies on the Duckett Jones criteria: the presence of two or more major clinical manifestations or one major manifestation plus two or more minor features.

• Throat swabs may be cultured for group A Streptococcus.
• Blood tests may reveal serological changes that indicate a recent streptococcal infection (antistreptolysin O and antistreptokinase titers). The elevation of one particular blood test (erythrocyte sedimentation rate or ESR) is a useful way of following rheumatic activity.
• Cardiomegaly (enlarged heart) may be detected through echocardiography (diagnostic method that uses ultrasound to visualize internal cardiac structures).
• Electrocardiogram (EKG) is used to record changes in the electrical activity of the heart.

How is Rheumatic fever treated?

Individuals with fever or active joint inflammation are treated with bedrest until the clinical syndrome has subsided (no fever, normal pulse rate, normal ESR, normal white count). If there is evidence of heart muscle inflammation (carditis), the individual is usually hospitalized for careful observation. Residual streptococcal infections are treated with antibiotics, even if the culture of nasal or throat swabs no longer reveals an infection. High-dose aspirin (salicylate) therapy is used to control joint pain and inflammation.

If carditis is present, corticosteroids may be given to try to minimize heart damage. Recurrences, most common when cardiac damage is present, may be prevented by the continued administration of penicillin until the age twenty years, or for five years after the last attack. Any subsequent streptococcal infection that develops should be treated promptly.

Medications

Information	Brand	Generic	Label	Rating
	Deltasone	Prednisone
	Ilosone	Erythromycin
	Naprosyn	Caproxen

Activity

The patient should stay in bed until studies show the disease has subsided. Bed rest for 2 to 5 weeks is usually required, but some cases require months. Provide a bed pan or bedside commode so the patient won’t have to get up to use the bathroom.

Diet

• A liquid or soft diet in the early stages, progressing to a normal diet high in protein, calories and vitamins.
• A low-salt diet may be recommended if patient has carditis (inflammation of the heart).

What might complicate it?

More than 50% of those who suffer acute rheumatic fever with carditis will later (after ten to twenty years) develop chronic rheumatic heart disease, predominantly affecting the mitral and aortic valves. Other complications include irregular heart rhythm (arrhythmias), inflammation of the sac enclosing the heart (pericarditis), chronic lung inflammation (rheumatic pneumonitis), and congestive heart failure.

Predicted outcome

Prognosis depends on the degree to which the heart has been affected and on whether recurrences can be avoided. The heart inflammation may resolve with no permanent effects.

However, there may be permanent scarring of one or more valves, which may result in obstruction to blood flow (stenosis) or backward (reversed) flow of blood (regurgitation or insufficiency). Sometimes, over a period of months or years, valve function is seriously compromised and surgery may ultimately be required to repair or replace the damaged heart valve(s). In rare instances, the heart muscle itself becomes overwhelmed by the inflammation and death from heart failure occurs.

Alternatives

Conditions with similar symptoms include rheumatoid arthritis, pyogenic arthritis, bacterial endocarditis, systemic lupus erythematosus, atrial myxoma, sickle cell crisis, viral myocarditis, dermatomyositis, influenza, Reiter's disease, osteomyelitis, and Lyme disease.

Appropriate specialists

Cardiologist, internist, infectious disease specialist, rheumatologist, and neurologist.

Last updated 6 October 2011