Rheumatic Fever

Key points

• Congestive heart failure is a rare complication that requires immediate treatment
• Initial treatment involves eradication of residual group A streptococci, reduction of inflammation, and treatment of congestive heart failure if present
• Prophylaxis against additional group A streptococcal infections is necessary for at least 5 years (length of treatment reflects degree of cardiac involvement)

Background

Description

• Delayed sequela of upper respiratory infection with group A streptococcus; acute episodes occur 2 to 3 weeks after infection
• Characterized by nonsuppurative inflammatory lesions involving primarily the heart, joints, subcutaneous tissues, and central nervous system
• Classic form is acute, febrile, and self-limited
• Damage to heart valves can occur, be chronic and progressive, and lead to heart failure and death many years after the initial episode
• Clinical presentation is highly variable and may include carditis, Sydenham chorea, migratory polyarthritis, erythema marginatum, and subcutaneous nodules

Epidemiology

Incidence

• The incidence of rheumatic fever is very low in Western countries (eg, in Scotland, 6 cases per 1 million persons per year; in the U.S., <2 cases per 100,000 persons). In developing countries, it may exceed 100 per 100,000 persons
• Overall incidence is 18 per 100,000 among persons aged 5 to 17 years; rarer in adults
• Peak incidence in Europe and the U.S. is in the spring
• An estimated 3% of military recruits with untreated streptococcal pharyngitis develop rheumatic fever, compared with 0.4% of untreated children

Prevalence

• 680 per 100,000 persons
• Affects approximately 1 in 1,800,000 persons in the U.S.

Demographics

Age:

• In children, peak incidence is between age 5 to 15 years
• In adults, the peak incidence of primary adult disease is at the end of the second to the beginning of the third decade of life
• Adults are more likely than children to have recurrent rather than primary disease and to have accompanying severe arthritis
• Adults are less likely than children to have accompanying chorea

Gender:

• Girls and women are more often affected

Race:

• Incidence is higher among African Americans, Puerto Rican persons, Hispanic Americans, Native Americans, and Native Hawaiians

Socioeconomic status:

• Outbreaks have occurred at military bases
• Historically linked to poor inner-city areas; however, risk among this population has decreased owing to improved access to medical care
• Outbreaks in the 1980s affected middle-class populations in urban and rural area

Causes and risk factors

• In all instances, occurs as a delayed sequela of group A streptococcal pharyngitis, including pharyngitis associated with scarlet fever
• Never results from group A streptococcal skin infections

Contributory or predisposing factors:

• Repeated upper respiratory infections
• Family history of rheumatic fever
• Crowded living or working conditions, such as military bases

Screening

Summary approach

Not applicable.

Primary prevention

Summary approach

Examination of a sore throat and culture of a specimen obtained from it is required to determine whether preventive treatment for rheumatic fever is necessary.

Preventive measures

Penicillin or erythromycin (in persons who are allergic to penicillin) is recommended for prevention in persons with group A β-hemolytic streptococcal infection.

Evidence

• Recommendations from the American Heart Association are that penicillin is the treatment of choice in the prevention of rheumatic fever in patients with group A β-hemolytic streptococcal pharyngitis. Penicillin may be administered intramuscularly or orally, depending on the physician's assessment of the patient's likelihood of adherence to an oral regimen and the risks of rheumatic fever in a particular population. Oral erythromycin is acceptable to prevent rheumatic fever in patients with group A β-hemolytic streptococcal pharyngitis who are allergic to penicillin.[1],[2]Level of evidence: 3

References