Introduction
Essentials of Diagnosis

• Uncommon in the United States but may be overlooked.
• Peak incidence ages 5-15 years.
• Diagnosis based on Jones criteria and confirmation of streptococcal infection.
• May involve mitral and other valves acutely, rarely leading to heart failure.

General Considerations
Rheumatic fever is a systemic immune process that is a sequela to ß-hemolytic streptococcal infection of the pharynx. Pyodermic infections are not associated with rheumatic fever. Signs of rheumatic fever usually commence 2-3 weeks after infection but may appear as early as 1 week or as late as 5 weeks. It had become quite uncommon in the United States, except in recent immigrants. However, there have been recent reports of new outbreaks in several regions of the United States. The peak incidence is between ages 5 and 15 years; rheumatic fever is rare before age 4 years and after age 40 years. Rheumatic carditis and valvulitis may be self-limited or may lead to slowly progressive valvular deformity. The characteristic lesion is a perivascular granulomatous reaction with vasculitis. The mitral valve is attacked in 75-80% of cases, the aortic valve in 30% (but rarely as the sole valve), and the tricuspid and pulmonary valves in under 5%.

Clinical Findings
Diagnostic criteria first described by Jones are still employed. The presence of two major criteria - or one major and two minor criteria - establishes the diagnosis.

A. Major Criteria
1. Carditis
Carditis is most likely to be evident in children and adolescents. Any of the following suggests the presence of carditis: (1) pericarditis; (2) cardiomegaly, detected by physical signs, radiography, or echocardiography; (3) congestive failure, right- or left-sided - the former perhaps more prominent in children, with painful liver engorgement due to Tricuspid regurgitation; and (4) mitral or aortic regurgitation murmurs, indicative of dilation of a valve ring with or without associated valvulitis. The Carey-Coombs short middiastolic mitral murmur may be present.

In the absence of any of the above definitive signs, the diagnosis of carditis depends upon the following less specific abnormalities: (1) electrocardiographic changes, including changing contour of P waves or inversion of T waves; (2) changing quality of heart sounds; and (3) sinus tachycardia, arrhythmia, or ectopic beats.

2. Erythema marginatum and subcutaneous nodules
The former begin as rapidly enlarging macules that assume the shape of rings or crescents with clear centers. They may be raised, confluent, and either transient or persistent.

Subcutaneous nodules are uncommon except in children. They are small (= 2 cm in diameter), firm, and nontender and are attached to fascia or tendon sheaths over bony prominences. They persist for days or weeks, are recurrent, and are indistinguishable from rheumatoid nodules.

3. Sydenham’s chorea
Sydenham’s chorea - involuntary choreoathetoid movements primarily of the face, tongue, and upper extremities - may be the sole manifestation; only 50% of cases have other overt signs of rheumatic fever. Girls are more frequently affected, and occurrence in adults is rare. This is the least common (3% of cases) but most diagnostic of the manifestations of rheumatic fever.

4. Arthritis
This is a migratory polyarthritis that involves the large joints sequentially. In adults, only a single joint may be affected. The arthritis lasts 1-5 weeks and subsides without residual deformity. Prompt response of arthritis to therapeutic doses of salicylates or nonsteroidal agents is characteristic.

B. Minor Criteria
These include fever, polyarthralgias, reversible prolongation of the PR interval, rapid erythrocyte sedimentation rate, and evidence of an antecedent ß-hemolytic streptococcal infection or a history of rheumatic fever.

C. Laboratory Findings
There is nonspecific evidence of inflammatory disease, as shown by a rapid sedimentation rate. High or increasing titers of antistreptococcal antibodies (antistreptolysin O and anti-DNase B) are used to confirm recent infection; 10% of cases lack this serologic evidence.

Differential Diagnosis
Rheumatic fever may be confused with the following: rheumatoid arthritis, osteomyelitis, endocarditis, chronic meningococcemia, systemic lupus erythematosus, Lyme disease, sickle cell anemia, “surgical abdomen,” and many other diseases.

Complications
Congestive heart failure occurs in severe cases. In the longer term, the development of rheumatic heart disease is the major problem. Other complications include arrhythmias, pericarditis with effusion, and rheumatic pneumonitis.

Treatment
A. General Measures
The patient should be kept at strict bed rest until the temperature returns to normal without medications, the sedimentation rate is normal, the resting pulse rate is normal (

< 100 beats/min in adults), and the ECG has returned to baseline.

B. Medical Measures
1. Salicylates
The salicylates markedly reduce fever and relieve joint pain and swelling. They have no effect on the natural course of the disease. Adults may require aspirin, 0.6-0.9 g every 4 hours; children are treated with lower doses. Toxicity includes tinnitus, vomiting, and gastrointestinal bleeding.

2. Penicillin
Penicillin (benzathine penicillin, 1.2 million units intramuscularly once, or procaine penicillin, 600,000 units intramuscularly daily for 10 days) is employed to eradicate streptococcal infection if present. Erythromycin may be substituted.

3. Corticosteroids
There is no proof that cardiac damage is prevented or minimized by corticosteroids. A short course of corticosteroids (prednisone, 40-60 mg orally daily, with tapering over 2 weeks) usually causes rapid improvement of the joint symptoms and is indicated when response to salicylates has been inadequate.