Acute rheumatic fever is diagnosed using the clinical and laboratory findings of the revised Jones criteria (Table 146-1).The presence of either two major criteria or one major and two minor criteria, along with evidence of an antecedent streptococcal infection, confirm a diagnosis of acute rheumatic fever. The infection often precedes the presentation of rheumatic fever by 2 to 6 weeks. Streptococcal antibody tests, such as the antistreptolysin O titer, are the most reliable laboratory evidence of prior infection.
Table 146-1 Major Jones Criteria for Diagnosis of Acute Rheumatic Fever*,†
Polyarthritis Common; swelling, limited motion, tender, erythema Migratory; involves large joints but rarely small or unusual joints, such as vertebrae
Carditis Common; pancarditis, valves, pericardium, myocardium
Tachycardia greater than explained by fever; new murmur of mitral or aortic insufficiency; Carey-Coombs mid-diastolic murmur; heart failure marginatum proximal extremities, evolving to serpiginous border with central clearing; evanescent, elicited by application of local heat; nonpruritic
Subcutaneous Uncommon; associated with repeated nodules episodes and severe carditis; located over extensor surface of elbows, knees, knuckles, and ankles or scalp and spine; firm, nontender *Minor criteria include fever (temperatures of 101°–102°F [38.2°–38.9°C]), arthralgias, previous rheumatic fever, leukocytosis, elevated erythrocyte sedimentation rate/C-reactive protein, and prolonged PR interval.
†One major and two minor, or two major, criteria with evidence of recent group A streptococcal disease (e.g., scarlet fever, positive throat culture, or elevated antistreptolysin O or other antistreptococcal antibodies) strongly suggest the diagnosis of acute rheumatic fever.
Arthritis is the most common major manifestation. It usually involves the large joints and is migratory. Arthralgia cannot be used as a minor manifestation if arthritis is used as a major manifestation. Carditis occurs in about 50% of patients. Tachycardia, a new murmur (mitral or aortic regurgitation), pericarditis, cardiomegaly, and signs of heart failure are evidence of carditis. Erythema marginatum, a serpiginous, nonpruritic, and evanescent rash, is uncommon, occurs on the trunk, and is brought out by warmth. Subcutaneous nodules are seen predominantly with chronic or recurrent disease. They are firm, painless, nonpruritic, mobile nodules found on the extensor surfaces of the large and small joints, the scalp, and the spine. Chorea (Sydenham chorea or St. Vitus dance) consists of neurologic and psychiatric signs. It also is uncommon and often presents long after the infection.
Management of acute rheumatic fever consists of benzathine penicillin to eradicate the beta-hemolytic streptococcus, anti-inflammatory therapy with salicylates, and bed rest. Additional supportive therapy for heart failure or chorea may be necessary. Longterm penicillin prophylaxis, preferably with intramuscular benzathine penicillin G, 1.2 million U every 28 days, is required. Oral regimens for prophylaxis generally are not as effective. The prognosis of acute rheumatic fever depends on the degree of permanent cardiac damage. Cardiac involvement may resolve completely, especially if it is the first episode and the prophylactic regimen is followed. The severity of cardiac involvement worsens with each recurrence of rheumatic fever.