The following are 10 points to remember about the revision of the Jones Criteria for the Diagnosis of Acute Rheumatic Fever in the Era of Doppler Echocardiography:

1. Rationale: Although the incidence of acute rheumatic fever (ARF) has declined in Europe and North America over the past 4-6 decades, the disease remains one of the most important causes of cardiovascular morbidity and mortality among socially and economically disadvantaged populations all over the world, especially in developing countries that are home to the majority of the world’s population. The Jones criteria, which have been the clinical standard for establishing the diagnosis of ARF since 1944, were last modified by the American Heart Association in 1992. Because echocardiographic techniques have evolved worldwide during the past 2 decades, and because echocardiography has become a cornerstone in worldwide screening programs to evaluate the prevalence of rheumatic heart disease (RHD), the limited diagnostic role for echocardiography in the diagnosis of carditis may no longer be appropriate.
2. Epidemiology: The global distribution of ARF and RHD is heterogeneous; certain geographic regions and specific ethnic and socioeconomic groups experience very high rates of ARF, whereas the disease has virtually disappeared in other regions. Because the clinical utility of a diagnostic test is influenced by pretest probability and background disease prevalence, a single set of diagnostic criteria may no longer be sufficient for all population groups and in all geographic regions.
3. Epidemiologic implications:
   • It is reasonable to consider individuals at low risk of ARF if they come from a setting or population with known low rates of ARF and RHD (Class IIa, Level of Evidence C).
   • Where reliable epidemiological data are available, it is reasonable that low risk is defined as an ARF incidence <2 per 100,000 school-aged children (5-14 years old) per year, or an all-age prevalence of RHD of ≤1 per 1,000 population per year (Class IIa, Level of Evidence C).
   • Children not clearly from a low-risk population are at moderate to high risk depending on their reference population (Class I, Level of Evidence C).
4. Clinical manifestations of ARF: The major clinical manifestations of ARF are carditis and arthritis, followed in descending frequency by chorea (with a female predominance), subcutaneous nodules, and erhythema marginatum (uncommon but specific of ARF).
5. Carditis diagnosis in an era of widely available echocardiography: Numerous studies over the past 20 years have addressed the role of echocardiography (compared with purely clinical assessment) in the diagnosis of ARF. More than 25 studies have reported echocardiography/Doppler evidence of mitral or aortic valve regurgitation in patients with ARF despite the absence of classic auscultatory findings.
• Echocardiography with Doppler should be performed in all cases of confirmed and suspected ARF (Class I, Level of Evidence B).
• It is reasonable to consider performing serial echocardiography/Doppler studies in any patient with diagnosed or suspected ARF, even if documented carditis is not present on diagnosis (Class IIa, Level of Evidence C).
• Echocardiography/Doppler testing should be performed to assess whether carditis is present in the absence of auscultatory findings, particularly in moderate- to high-risk populations and when ARF is considered likely (Class I, Level of Evidence B).
• Echocardiography/Doppler findings not consistent with carditis should exclude that diagnosis in patients with a heart murmur otherwise thought to indicate rheumatic carditis (Class I, Level of Evidence B).
• Specific criteria exist for Doppler findings in rheumatic valvulitis: 1) Mitral regurgitation (all four): seen in ≥2 views, jet length ≥2 cm, peak velocity >3 m/s, pansystolic; and 2) Aortic regurgitation (all four): seen in ≥2 views, jet length ≥1 cm, peak velocity >3 m/s, pandiastolic.
6. Evidence of preceding Streptococcal infection: Because other illnesses may closely resemble ARF, laboratory evidence of antecedent group A streptococcal infection is needed whenever possible, and the diagnosis is in doubt when such evidence is not available. Any one of the following can serve as evidence of preceding infection:
   • 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.
   • A positive throat culture for group A β-hemolytic streptococci (Class I, Level of Evidence B).
   • 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).
7. Revised Jones criteria, low-risk populations: Major and minor criteria are as follows:
   • Major criteria: carditis (clinical and/or subclinical), arthritis (polyarthritis), chorea, Erythema marginatum, and subcutaneous nodules
   • Minor criteria: olyarthralgia, fever (≥38.5° F), sedimentation rate ≥60 mm and/or C-reactive protein (CRP) ≥3.0 mg/dl, and prolonged PR interval (unless carditis is a major criterion)
8. Revised Jones criteria, moderate- and high-risk populations: Major and minor criteria are as follows:
   • Major criteria: carditis (clinical and/or subclinical), arthritis (monopolyarthritis or polyarthritis, or polyarthralgia), chorea, Erythema marginatum, and subcutaneous nodules
   • Minor criteria: fever (≥38.5° F), sedimentation rate ≥30 mm and/or CRP ≥3.0 mg/dl, and prolonged PR interval (unless carditis is a major criterion)
9. ARF diagnosis (initial episode): The diagnosis of an initial episode of ARF requires two major criteria, or one major plus two minor criteria.
10. ARF diagnosis (subsequent episode): Patients with a history of ARF or RHD are at high risk for recurrent attacks if re-infected with group A streptococci.
    • With a reliable past history of ARF or established RHD, and in the face of documented group A streptococcal infection, two major, one major and two minor, or three minor manifestations may be sufficient for a presumptive diagnosis (Class IIb, Level of Evidence C).
    • When minor manifestations alone are present, the exclusion of other more likely causes of the clinical presentation is recommended before a diagnosis of an ARF recurrence is made (Class I, Level of Evidence C).

Keywords: Aortic Valve Insufficiency, Aortic Valve, Arthralgia, Bacterial Proteins, C-Reactive Protein, Chorea, Developing Countries, Diagnostic Tests, Routine, Echocardiography, Echocardiography, Doppler, Epidemiology, Endocarditis, Erythema, Heart Defects, Congenital, Heart Murmurs, Incidence, Mitral Valve Insufficiency, Myocarditis, Pharyngitis, Polysaccharides, Bacterial, Pharynx, Prevalence, Rheumatic Fever, Rheumatic Heart Disease, Streptococcal Infections, Streptococcus pyogenes, Streptolysins, Vulnerable Populations

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