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Rheumatic Fever Licks at the Joints, but Bites at the Heart

Rheumatic fever is the result of an abnormal autoimmune response to group A streptococcal pharyngeal infection in a susceptible host. However, in approximately one-third of cases a history of pharyngeal infection is not elucidated prior to the development of rheumatic fever. In certain individuals, which is not uncommon, streptococcal pharyngeal infection does not result in rheumatic fever. As a general rule, rheumatic fever affects young individuals (6–15 years old), but can occur in adulthood. The clinical manifestations of rheumatic fever include migratory arthritis, fever, erythema marginatum (nonpruritic rash with sharp erythematous borders), subcutaneous nodules, chorea, and carditis [1–3]. During the acute phase of the disease, typically the left sided cardiac valves appear edematous and inflamed. Tiny verrucae (1–2 mm in diameter), consisting of platelets and fibrin, are seen along the lines of valve closure. These findings can be detected with current imaging techniques, especially echocardiography. Inflammation involving the connective tissue of the valves can also be seen in the mitral annulus and the chordae tendineae. Mitral regurgitation is the most common clinical presentation of acute valvulitis, ranging from mild to severe [1]. In this issue of Cardiology, Narang et al. [4] report the clinical presentation of 126 children with acute rheumatic fever who were treated in a tertiary medical center (60 patients had a previous history of rheumatic fever). Joint symptoms were present in 29% and fever in only 25% of the patients; 2.4% of the patients had subcutaneous nodules and there were no patients with erythema marginatum or chorea. Tachycardia and heart failure were present in 53 and 21% of the patients, respectively. Echocardiogram demonstrated mitral leaflet thickening in 77%, small nodules on the tip of the mitral leaflets in 43%, thickening of the aortic valve in 27%, and nodules in the aortic cusps in 8% of the patients. Mitral regurgitation ranging from mild to severe was present in 95%, mitral stenosis in 16%, aortic regurgitation in 44%, and tricuspid regurgitation in 56% of the patients. Of interest, mitral valve prolapse (MVP) was seen in 29% and flail leaflet was seen in 5% of the patients. The most recent modified Jones criteria (2015) for the diagnosis of acute rheumatic fever were present in only 32% of the patients [5].