Acute rheumatic fever
Evidence of Preceding Streptococcal Infection
Any 1 of the following can serve as evidence of preceding infection, per a recent AHA statement:
1. Increased or rising anti-streptolysin O titer or other streptococcal antibodies (anti-DNASE B) (Class I; Level of Evidence B). A rise in titer is better evidence than a single titer result.
2. A positive throat culture for group A β-hemolytic streptococci (Class I; Level of Evidence B).
3. A positive rapid group A streptococcal carbohydrate antigen test in a child whose clinical presentation suggests a high pretest probability of streptococcal pharyngitis (Class I; Level of Evidence B).
Diagnosis: Initial ARF 2 Major manifestations or 1 major plus 2 minor manifestations.
Diagnosis: Recurrent ARF 2 Major or 1 major and 2 minor or 3 minor
Major criteria
Carditis • Clinical and/or subclinical
Arthritis • Monoarthritis or polyarthritis
• Polyarthralgia
Chorea
Erythema marginatum
Subcutaneous nodules
Minor criteria
Monoarthralgia
Fever (≥38°C)
ESR ≥30 mm/h and/or CRP ≥3.0 mg/dL
Prolonged PR interval, after accounting for age variability (unless carditis is a major criterion)
Subclinical carditis
Doppler Findings in Rheumatic Valvulitis
Pathological mitral regurgitation (all 4 criteria met)
-
Seen in at least 2 views
-
Jet length ≥2 cm in at least 1 view
-
Peak velocity >3 m/s
-
Pansystolic jet in at least 1 envelope
Pathological aortic regurgitation (all 4 criteria met)
-
Seen in at least 2 views
-
Jet length ≥1 cm in at least 1 view
-
Peak velocity >3 m/s
-
Pan diastolic jet in at least 1 envelope
Morphological Findings on Echocardiogram in Rheumatic Valvulitis
Acute mitral valve changes
Annular dilation
Chordal elongation
Chordal rupture resulting in flail leaflet with severe mitral regurgitation
Anterior (or less commonly posterior) leaflet tip prolapse
Beading/nodularity of leaflet tips
Chronic mitral valve changes: not seen in acute carditis
Leaflet thickening
Chordal thickening and fusion
Restricted leaflet motion
Calcification
Aortic valve changes in either acute or chronic carditis
Irregular or focal leaflet thickening
Coaptation defect
Restricted leaflet motion
Leaflet prolapse
Primary prophylaxis for rheumatic fever
Antibiotic
Dose
Duration
Penicillin V
Amoxicillin
Benzathine penicillin G
Allergic to penicillin
Narrow-spectrum cephalosporin
Clindamycin
Azithromycin
Clarithromycin
Children: 250 mg oral three times daily
Adolescents and adults: 500 mg oral two or three times daily
50 mg/kg oral once daily (maximum 1 g)
600,000 IU IM ( ≤27 kg ) or
1,200,000 IU IM ( >27 kg )
variable
20 mg/kg per day oral divided in 3 doses oral (maximum 1.8 g/d)
12 mg/kg oral once daily (maximum 500 mg)
15 mg/kg per day oral divided into 2 doses (maximum 250 mg twice daily)
10 days
10 days
Once
10 days
10 days
5 days
10 days
Secondary prophylaxis for rheumatic fever
Category of patient
Duration after last attack
Rheumatic fever without carditis
Rheumatic fever with carditis but no residual valvular disease
Rheumatic fever with carditis and residual valvular disease
5 years or until age 21 years
(whichever is longer)
10 years or until age 21 years
(whichever is longer)
10 years or until age 40 years
(whichever is longer)
lifetime prophylaxis may be need
Antibiotic
Dose
Benzathine penicillin G
Penicillin V
Sulfadiazine
Macrolide
600,000 units IM ( ≤27 kg ) or
1,200,000 units IM ( >27 kg )
Every 4 weeks (3 weeks in high-risk areas)
250 mg oral twice daily
0.5 g oral daily ( ≤27 kg ) or
1 g oral daily ( >27 kg )
Drug-dependent
References
Gewitz MH, Baltimore RS, Tani LY, Sable CA, Shulman ST, Carapetis J, et al. Revision of the Jones Criteria for the Diagnosis of Acute Rheumatic Fever in the Era of Doppler Echocardiography. Circulation. 2015 May 19;131(20):1806–18.
Zuhlke L, Karthikeyan G. Primary Prevention for Rheumatic Fever : Progress, Obstacles, and Opportunities. Global Heart. 2013 Sep 1;8:221–6.
Gerber MA, Baltimore RS, Eaton CB, Gewitz M, Rowley AH, Shulman ST, Taubert KA. Prevention of rheumatic fever and diagnosis and treatment of acute Streptococcal pharyngitis: a scientific statement from the American Heart Association Rheumatic Fever, Endocarditis, and Kawasaki Disease Committee of the Council on Cardiovascular Disease in the Young, the Interdisciplinary Council on Functional Genomics and Translational Biology, and the Interdisciplinary Council on Quality of Care and Outcomes Research. Circulation. 2009;119:1541–1551.