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Clinical Summary

Acute rheumatic fever is a postinfectious complication of group A Streptococcus tonsillopharyngitis. Clinical signs can include migratory polyarthritis, carditis, subcutaneous nodules, erythema marginatum, and Sydenham chorea (the five major diagnostic criteria). Arthritis is the most common manifestation, which can be distinguished from other rheumatologic or infectious conditions by its tendency to affect large joints. The migratory pattern is also characteristic and less commonly seen in other conditions. Carditis is the next most common manifestation, often causing endocarditis and possible valvular damage. Subcutaneous nodules (located on extensor surfaces) and erythema marginatum (macular erythematous rash with serpiginous border, as pictured) are less commonly seen. Sydenham chorea presents with jerky, involuntary movements of the face and extremities. While the other major manifestations are typically seen 2 to 3 weeks after streptococcal pharyngitis, chorea can develop several months later. Diagnosis of rheumatic fever also requires evidence of preceding streptococcal infection, either by positive rapid strep test or evidence of elevated streptococcal antibody tiers (anti-streptolysin O [ASO] or anti-DNAse B). Diagnosis is made based on the presence of two major criteria (carditis, arthritis, chorea, erythema marginatum, subcutaneous nodules) or one major and two minor criteria (fever, arthralgia [in absence of arthritis], elevated acute phase reactants, prolonged PR interval).

Management and Disposition

Patients with migratory polyarthritis or other features concerning for rheumatic fever, as described earlier, require careful auscultation for murmur, echocardiogram, and electrocardiogram for evaluation for carditis. ASO and anti-DNAse B antibodies should be drawn to confirm recent streptococcal infection. Inflammatory markers are often elevated as well. Consult rheumatology and cardiology for assistance with diagnosis. Treatment includes treating the streptococcal infection, as well as long-term antibiotic prophylaxis to prevent recurrent episodes of streptococcal infection, which can lead to further cardiac damage.

FIGURE 26.30

Acute Rheumatic Fever—Erythema Marginatum. An evanescent, erythematous, macular eruption with a pale center on the arm of a patient with acute rheumatic fever. (Image appears with permission from VisualDx [www.visualdx.com].)

Pearls

  1. Streptococcal antibodies are helpful in determining preceding infection, as many patients were unaware of or do not recall symptoms of streptococcal pharyngitis.

  2. Patients with migratory arthritis following streptococcal infection who do not otherwise meet criteria for rheumatic fever are diagnosed with poststreptococcal reactive arthritis. These patients are given prophylactic antibiotics as well, but for a shorter time course.

  3. Obtaining family history of acute rheumatic fever or valvular heart disease is important, as there appears to be a genetic susceptibility.

FIGURE 26.31

Acute Rheumatic Fever—Erythema Marginatum. An evanescent, erythematous, macular eruption with a pale center on the arm of a patient with acute rheumatic fever. (Image appears with permission from VisualDx [www.visualdx.com].)

FIGURE 26.32

Acute Rheumatic Fever. Bilateral knee arthritis in a patient ...

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