a. Definition. Acute rheumatic fever is a systemic, immune-mediated disorder that occurs as a sequela to group A streptococcal pharyngeal infection. Remember: streptococcal skin infections are not associated with rheumatic fever.
b. Epidemiology. Acute rheumatic fever most commonly occurs in school-aged children, 2–4 weeks (mean, 19 days) after an acute throat infection.
i. Acute rheumatic fever rarely occurs in patients younger than 5 years or older than 40 years.
ii. The overall prevalence is low (<5%) in the United States but is higher in recent immigrants.
c. Rheumatic fever “bites the heart and licks the joints” (i.e., chronic arthritis is not a sequela of rheumatic fever, but valve disease and congestive heart failure [CHF] can be). The incidence of valve involvement varies depending on the valve.
i. The mitral valve is affected in the majority of cases.
ii. The aortic valve is affected in less than half of cases (and almost never as the sole valve).
iii. The tricuspid and pulmonary valves are affected in less than 5% of cases, usually in association with mitral valve involvement.
a. Jones criteria. Diagnosis of rheumatic fever is based on evidence of a preceding streptococcal pharyngitis (e.g., a positive anti–streptolysin O [ASO] titer or culture), plus two major or one major and two minor Jones criteria.
1. Arthritis. The arthritis takes the form of a migratory polyarthritis that tends to involve the large joints sequentially; however, adults may have only single joint involvement. Arthritis resolves spontaneously within 1 month, and there are no residual joint deformities.
2. Heart involvement. Evidence of carditis may include pericarditis, myocarditis, cardiomegaly, CHF, and mitral or aortic regurgitation.
3. Nodules. Small, firm, nontender subcutaneous nodules occur over areas of bony prominence and over tendons. Nodules are rarely seen in adults.
4. Erythema marginatum is the classic rash but is seen in less than 10% of cases. It is erythematous and nonpruritic; individual lesions are evanescent with a serpiginous border.
5. Sydenham’s chorea (St. Vitus’ dance)—the most diagnostic of the major criteria—is characterized by involuntary choreoathetoid movements of the face, hands, and feet.
The Jones criteria have recently been updated to include subclinical carditis, which is diagnosed by echocardiography.
1. Prolongation of the PR interval on the electrocardiogram (EKG)
2. Increased erythrocyte sedimentation rate (ESR) and/or C-reactive protein (CRP)
b. Clinical presentation. Rheumatic fever usually presents in one of three ways:
i. Insidious carditis
ii. Acute-onset polyarthritis
iii. Chorea (least common)
a. High-dose salicylates will rapidly decrease fever and joint swelling, but do not affect the natural course of the illness. If salicylates do not provide symptomatic relief, steroids can be considered.
i. Patients with acute rheumatic fever should be treated with antibiotics (oral penicillin for 10 days or azithromycin for patients who are allergic to penicillin) to eradicate group A Streptococcus carriage.
ii. Prophylactic antibiotics are recommended to prevent further streptococcal infections. The preferred regimen is intramuscular penicillin every 4 weeks and should be continued for a minimum of 5 years after the last episode and until the patient is in his or her mid-20s.
iii. If permanent valve damage or scarring occurs, prophylaxis during invasive procedures (e.g., dental or other surgical procedures) later in life might be appropriate.
Suggested Further Readings
Gewitz MH, Baltimore RS, Tani LY, et al. Revision of the Jones criteria for the diagnosis of acute rheumatic fever in the era of Doppler echocardiography. Circulation 2015;131:1806.Find this resource:
He VYF, Condon JR, Ralph AP, et al. Long-term outcomes from acute rheumatic fever and rheumatic heart disease: a data-linkage and survival analysis approach. Circulation 2016;134:222.Find this resource:
Webb RH, Grant C, Harnden A. Acute rheumatic fever. BMJ 2015;351.Find this resource: