Acute Rheumatic Fever Clinical Presentation

Updated: Sep 22, 2022
  • Author: Robert J Meador, Jr, MD; Chief Editor: Herbert S Diamond, MD  more...
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Usually, a latent period of approximately 18 days occurs between the onset of streptococcal pharyngitis and the onset of acute rheumatic fever (ARF). This latent period is rarely shorter than 1 week or longer than 5 weeks.

Approximately 70% of older children and young adults recollect the pharyngitis. However, only approximately 20% of young children recollect pharyngitis. Therefore, younger children who present with signs or symptoms consistent with ARF merit a higher index of suspicion. [22]

Typically, the first manifestation of ARF is a very painful migratory polyarthritis. Large joints such as knees, ankles, elbows, or shoulders are typically affected. Often, associated fever and constitutional toxicity develop. Sydenham chorea (ie, rapid, irregular, aimless involuntary movements of the arms and legs, trunk, and facial muscles [23] ) was once a common late-onset clinical manifestation but is now rare. [4] Acute attacks usually resolve within 12 weeks.


Physical Examination

Physical findings can be nonspecific and misleading. Therefore, a high index of suspicion is required for diagnosis.

Suspicious signs for carditis include new or changing valvular murmurs, cardiomegaly, congestive heart failure, and/or pericarditis. Nearly 60% of patients with carditis develop isolated mitral valve involvement, followed in prevalence by combined mitral and aortic valve involvement.

When present, Sydenham chorea is seldom evident at the time of initial presentation. Erythema marginatum and subcutaneous nodules are rare (< 10% of patients). See the image below.

Erythema marginatum, the characteristic rash of ac Erythema marginatum, the characteristic rash of acute rheumatic fever.

Arthritis, which occurs in 80% of patients, usually involves multiple large joints, particularly the knees, ankles, elbows, and wrists. Hips and smaller joints of hands and feet are less commonly involved.

Migratory polyarthritis is usually associated with a febrile illness. It involves a series of painful joints, followed by another series of painful joints. This form of arthritis rarely causes permanent joint deformity.

Unusual presentations, such as indolent carditis and isolated chorea, may also occur. Even rarer manifestations include epistaxis and abdominal pain due to serositis.