A systemic inflammatory disease of childhood, acute rheumatic fever develops after infection of the upper respiratory tract with group A beta-hemolytic streptococci.

Rheumatic fever principally involves the heart, joints, central nervous system, skin, and subcutaneous tissues. It commonly recurs.

The term rheumatic heart disease refers to the cardiac involvement of rheumatic fever - its most destructive effect. Cardiac involvement develops in up to 50% of patients and may affect the endocardium, myocardium, or pericardium during the early acute phase. It may later affect the heart valves, causing chronic valvular disease.

The extent of damage to the heart depends on where the disorder strikes. Myocarditis produces characteristic lesions called Aschoff's bodies in the acute stages as well as cellular swelling and fragmentation of interstitial collagen, leading to formation of a progressively fibrotic nodule and interstitial scars. Endocarditis causes valve leaflet swelling, erosion along the lines of leaflet closure, and blood. platelet, and fibrin deposits. which form beadlike vegetation. It strikes the mitral valve most commonly in females and the aortic valve in males. In both, it affects the tricuspid valves occasionally and the pulmonic valve rarely.

Long-term antibiotic therapy can minimize the recurrence of rheumatic fever, reducing the risks of permanent cardiac damage and valvular deformity.

Although rheumatic fever tends to be familial, this tendency may reflect contributing environmental factors. For example, in lower socioeconomic groups, incidence is highest in children between ages 5 and I5, probably resulting from malnutrition and crowded living conditions. Rheumatic fever strikes most commonly during cool. damp weather in winter and early spring. In the United States, it's most common in the northern states.

Causes

Rheumatic fever appears to be a hypersensitivity reaction in which antibodies produced to combat streptococci react and produce characteristic lesions at specific tissue sites. How and why group A streptococcal infection initiates the process are unknown. Because few people infected with Streptococcus ever contract rheumatic fever (about 0.3%), altered host resistance probably is involved in its development or recurrence.

Signs & Symptoms

Some of the most common symptoms are: breathlessness, fatigue, palpitations, chest pain, and fainting attacks.

Diagnostic Tests

No specific laboratory tests determine the presence of rheumatic fever, but the following test results support the diagnosis:

White blood cell count and erythrocyte sedimenration rate may be elevated (during the acute phase); blood studies show slight anemia caused by suppressed erythropoiesis during inflammation.
Cardiac enzyme levels may be increased in severe carditis.
Antistreptolysin-O titer is elevated in 95% of patients within 2 months of onset.
Electrocardiography reveals no diagnostic changes, but 20% of patients show a prolonged PR interval.
Chest X-rays show normal heart size (except with myocarditis, heart failure, and pericardial effusion).
Echocardiography helps evaluate valvular damage, chamber size, ventricular function, and the presence of a pericardial effusion.
Cardiac catheterization is used to evaluate valvular damage and left ventricular function in severe cardiac dysfunction.

Treatment

Effective management eradicates the streptococcal infection, relieves symptoms, and prevents recurrence, thus reducing the chance of permanent cardiac damage. During the acute phase, treatment includes penicillin or erythromycin (for patients with penicillin hypersensitivity). Salicylates, such as aspirin, relieve fever and minimize joint swelling and pain; if the patient has carditis or if salicylates fail to relieve pain and inflammation, the doctor may prescribe corticosteroids.

Supportive treatment requires strict bed rest for about 5 weeks during the acute phase with active carditis, followed by a progressive increase in physical activity. The increase depends on clinical and laboratory findings and the patient's response to treatment.

After the acute phase subsides, a monthly l.M. injection of penicillin G benzathine or daily doses of oral sulfadiazine or penicillin G may be used to prevent recurrence. Such preventive treatment usually continues for 5 to 10 years.

Heart failure requires continued bed rest and diuretics. Severe mitral or aortic valvular dysfunction that causes persistent heart failure requires corrective surgery, such as commissurotomy (separation of the adherent, thickened leaflets of the mitral valve), valvuloplasty (inflation of a balloon within a valve), or valve replacement (with prosthetic valve). Corrective valvular surgery seldom is necessary before late adolescence.

Prevention Tips

The best defense against rheumatic heart disease is to prevent rheumatic fever from ever occurring. By treating strep throat with penicillin or other antibiotics, doctors can usually stop acute rheumatic fever from developing.

People who've already had rheumatic fever are more susceptible to attacks and heart damage. That's why they're given continuous monthly or daily antibiotic treatment, maybe for life. If their heart has been damaged by rheumatic fever, they're also given a different antibiotic when they undergo dental or surgical procedures. This helps prevent bacterial endocarditis, a dangerous infection of the heart's lining or valves.

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