The manifestations of acute rheumatic fever have evolved over the last 300 years. Initially it was believed to be a transient arthritis associated with chorea, skin lesions, and pericarditis. The presence of two major criteria—or one major and two minor criteria—establishes the diagnosis. While India, New Zealand, and Australia have all published revised guidelines since 2001, the 2015 recommendations have revised the Jones criteria (Table 10–17) in a scientific statement from the AHA where subclinical carditis is now recognized with the advent of echocardiography. In the review, subclinical carditis was evident in 16.8% of the 10 studies used by the World Health Organization. The revised criteria also recognize that a lower threshold should be used to diagnosis acute rheumatic fever in high-risk populations.
Table 10–17.The 2015 revised Jones criteria.1 ||Download (.pdf) Table 10–17. The 2015 revised Jones criteria.1
|Population ||Criteria |
| ||Major ||Minor |
|Low risk ||Carditis (clinical or subclinical) ||Polyarthralgia |
| ||Arthritis (polyarthritis only) ||Fever (≥ 38.5°C) |
| ||Chorea ||ESR ≥ 60 mm/h or CRP ≥ 3.0 mg/dL (or both) |
| ||Erythema marginatum ||Prolonged PR interval (unless carditis is major criterion) |
| ||Subcutaneous nodules || |
|Moderate and high risk ||Carditis (clinical or subclinical) ||Monoarthralgia |
| ||Arthritis (monoarthritis, polyarthritis, polyarthalgia) ||Fever (≥ 38°C) |
| ||Chorea ||ESR ≥ 30 mm/h or CRP ≥ 3.0 mg/dL (or both) |
| ||Erythema marginatum ||Prolonged PR interval (unless carditis is a major criterion) |
| ||Subcutaneous nodules || |
Carditis is most likely to be evident in children and adolescents. Any of the following suggests the presence of carditis: (1) pericarditis; (2) cardiomegaly, detected by physical signs, radiography, or echocardiography; (3) heart failure, right- or left-sided—the former perhaps more prominent in children, with painful liver engorgement due to tricuspid regurgitation; and (4) mitral or aortic regurgitation murmurs, indicative of dilation of a valve ring with or without associated valvulitis or morphologic findings on echocardiography of rheumatic valvulitis. The Carey–Coombs short mid-diastolic mitral murmur may be present due to inflammation of the mitral valve. It is a class I (LOE B) indication to perform echocardiography/Doppler studies on all cases of suspected or confirmed acute rheumatic fever.
2. Erythema marginatum and subcutaneous nodules
Erythema marginatum begins as rapidly enlarging macules that assume the shape of rings or crescents with clear centers. They may be raised, confluent, and either transient or persistent and usually on the trunk or proximal extremities. Subcutaneous nodules are uncommon except in children. They are small (2 cm or less in diameter), firm, and nontender and are attached to fascia or tendon sheaths over bony prominences. They persist for days or weeks, are recurrent, and are indistinguishable from rheumatoid nodules. Neither the rash nor nodules ever occur as the sole manifestation of acute rheumatic fever.
This is the most definitive manifestation of acute rheumatic fever. Defined as involuntary choreoathetoid movements primarily of the face, tongue, and upper extremities, Sydenham chorea may be the sole manifestation of rheumatic fever. Girls are more frequently affected than boys, and occurrence in adults is rare.
This is a migratory polyarthritis that involves the large joints sequentially. In adults and in certain moderate- to high-risk populations, only a single joint may be affected. The arthritis lasts 1–5 weeks and subsides without residual deformity. Prompt response of arthritis to therapeutic doses of salicylates or nonsteroidal agents is characteristic.
These include fever, polyarthralgia, reversible prolongation of the PR interval, and an elevated erythrocyte sedimentation rate or CRP. A lower threshold is set for patients at high risk (Table 10–17). The 2015 guidelines stipulate that evidence for a preceding streptococcal infection can be defined by an increase or rising anti-streptolysin O titer or streptococcal antibodies (anti-DNAase B), a positive throat culture for group A beta-hemolytic streptococcal or a positive rapid group A streptococcal carbohydrate antigen test in a child with a high pretest probability of streptococcal pharyngitis.