Sections View Full Chapter Figures Tables Videos Annotate Full Chapter Figures Tables Videos Supplementary Content + Download Section PDF Listen ++ For further information, see CMDT Part 10-48: Rheumatic Fever + Key Features Download Section PDF Listen +++ +++ Essentials of Diagnosis ++ More common (100 cases/100,000 population) in developing countries than in the United States (approximately 2 cases/100,000 population) Peak incidence ages 5–15 years Revision of Jones criteria in 2015 includes echocardiographic findings May involve mitral and other valves acutely, rarely leading to heart failure +++ General Considerations ++ Rheumatic fever A systemic immune process that is a sequela of a β-hemolytic streptococcal infection of the pharynx Pyodermic infections are not associated with rheumatic fever Rheumatic carditis and valvulitis May be self-limited May lead to slowly progressive valvular deformity A perivascular granulomatous reaction with valvulitis is characteristic lesion Chronic rheumatic heart disease Results from single or repeated attacks of rheumatic fever that produce Rigidity and deformity of valve cusps Fusion of the commissures Shortening and fusion of the chordae tendineae Valvular stenosis or regurgitation results and the two often coexist Affects mitral, aortic, and tricuspid valves, but the pulmonary valve only very rarely +++ Demographics ++ Rheumatic fever Rare before age 4 or after age 40 years Responsible for 250,000 deaths in young people worldwide each year Over 15 million have evidence for rheumatic heart disease Rheumatic heart disease While there has been progress against this disease, it remains a major cardiovascular problem in the poorest regions of the world In 2015, there were 33.4 million cases globally Responsible for 320,000 deaths in 2015 + Clinical Findings Download Section PDF Listen +++ +++ Symptoms and Signs ++ Signs of acute rheumatic fever Usually commence 2–3 weeks after infection May appear as early as 1 week or as late as 5 weeks +++ Major Criteria ++ Carditis Pericarditis Cardiomegaly Heart failure, with painful liver engorgement due to tricuspid regurgitation Mitral or aortic regurgitation murmurs Carey–Coombs short mid-diastolic mitral murmur may be present due to inflammation of the mitral valve Occurs most often in children and adolescents When any of the above definitive signs are absent, the diagnosis of carditis depends on the following less specific abnormalities: ECG changes, including changing contour of P waves or inversion of T waves Changing quality of heart sounds Sinus tachycardia, arrhythmia, or ectopic beats Erythema marginatum Begins as rapidly enlarging macules that assume the shape of rings or crescents with clear centers May be raised, confluent, and either transient or persistent Subcutaneous nodules Small (≤ 2 cm in diameter), firm, and nontender Persist for days or weeks and are recurrent Attached to fascia or tendon sheaths over bony prominences Indistinguishable from rheumatoid nodules Uncommon except in children Sydenham chorea Involuntary choreoathetoid movements primarily of the face, tongue, and upper extremities May be the sole manifestation of rheumatic fever; only half of cases have other overt signs Girls are more frequently affected; occurrence in adults is rare Least common (3% of cases) but most diagnostic manifestation of acute rheumatic fever Polyarthritis Involves the large joints sequentially The arthritis lasts 1–5 weeks and subsides without residual deformity In adults, only a single joint may be affected +++ Minor Criteria ++ Fever Polyarthralgias Reversible prolongation of the PR interval Elevated erythrocyte sedimentation rate (ESR) or C-reactive protein (CRP) Positive throat culture or rapid streptococcal antigen test and elevated or rising streptococcal antibody titer + Diagnosis Download Section PDF Listen +++ ++ Rapid ESR is nonspecific evidence of inflammatory disease High or increasing titers of antistreptococcal antibodies (antistreptolysin O and anti-DNase B) + Treatment Download Section PDF Listen +++ +++ General Measures ++ Bed rest until the temperature, resting pulse rate, ESR and ECG have returned to normal +++ Medications ++ Salicylates Adults may require large doses of aspirin, 0.6–0.9 g every 4 hours; children, lower doses Markedly reduce fever, relieve joint pain and swelling, but have no effect on the natural course of the disease Penicillin Benzathine penicillin, 1.2 million units intramuscularly once, or procaine penicillin, 600,000 units intramuscularly daily for 10 days) to eradicate streptococcal infection Erythromycin may be substituted (40 mg/kg/day) Corticosteroids Prednisone, 40–60 mg orally daily, with tapering over 2 weeks Usually causes rapid improvement of the joint symptoms Indicated when response to salicylates has been inadequate + Outcome Download Section PDF Listen +++ +++ Complications ++ Rheumatic valvular disease affects The mitral valve most commonly The aortic valve second most commonly The tricuspid valve third most commonly Heart failure Arrhythmias, pericarditis with effusion, and rheumatic pneumonitis +++ Prevention ++ Initial episode of rheumatic fever can usually be prevented by early treatment of streptococcal pharyngitis with penicillin Prevention of recurrent episodes is critical Preferred: Benzathine penicillin G, 1.2 million units intramuscularly every 4 weeks Less reliable: Oral penicillin (200,000–250,000 units twice daily) Penicillin allergy: Sulfadiazine (or sulfisoxazole), 1 g daily; or erythromycin, 250 mg orally twice daily +++ Prognosis ++ Initial episodes may last months in children and weeks in adults The immediate mortality rate is 1–2% Persistent rheumatic carditis with cardiomegaly, heart failure, and pericarditis implies a poor prognosis + References Download Section PDF Listen +++ + +Gewitz MH et al. Revision of the Jones Criteria for the diagnosis of acute rheumatic fever in the era of Doppler echocardiography. A scientific statement from the American Heart Association. Circulation. 2015;131:1806–18. [PubMed: 25908771] + +Roberts K et al. Screening for rheumatic heart disease: current approaches and controversies. Nat Rev Cardiol. 2013 Jan;10(1):49–58. [PubMed: 23149830] + +Watkins DA et al. Global, regional, and national burden of rheumatic heart disease, 1990-2015. N Engl J Med. 2017 Aug 24;377(8):713–22. [PubMed: 28834488] + +Yacoub M et al. Eliminating acute rheumatic fever and rheumatic heart disease. Lancet. 2017 Jul 15;390(10091):212–3. [PubMed: 28721865]