Acute rheumatic fever remains a serious healthcare concern for the majority of the world’s population despite its decline in incidence in Europe and North America. This statement reviews the historic Jones criteria used to diagnose acute rheumatic fever in the context of the current epidemiology of the disease and updates those criteria to also taking into account the use of Doppler echocardiography in the diagnosis of carditis as a major manifestation of acute rheumatic fever.
1. It is reasonable to consider individuals to be at low risk for ARF if they come from a setting or population known to experience low rates of ARF or RHD (Class IIa; Level of Evidence C).
2. It is reasonable that where reliable epidemiological data are available, low risk should be defined as having an ARF incidence <2 per 100 000 school-aged children (usually 5–14 years old) per year or an allage prevalence of RHD of ≤1 per 1000 population per year (Class IIa; Level of Evidence C).
3. 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).
2. Clinical Manifestations of ARF:
Generally, the clinical profile of ARF in low- and middle-income countries closely resembles that of high-income countries. Universally, the most common major manifestations during the first episode of ARF (the “major criteria” for diagnosis) remain
carditis (50%–70%) and arthritis (35%–66%). These are followed in frequency by chorea (10%–30%), which has been demonstrated to have a female predominance, and then
subcutaneous nodules (0%–10%) and erythema marginatum (<6%), which remain much less common but highly specific manifestations of ARF.
3.Carditis: Diagnosis in the Era of Widely Available Echocardiography:
Classically, as discussed in the 1992 AHA revised Jones criteria statement, carditis as a major manifestation of ARF has been a clinical diagnosis based on the auscultation of typical murmurs that indicate mitral or aortic valve regurgitation, at either valve or both valves. 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. This writing group concludes the following:
1. Echocardiography with Doppler should be performed in all cases of confirmed and suspected ARF (Class I; Level of Evidence B).
2. 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).
3. 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).
4. 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).
4.Specific doppler criteria for diagnosis of rheumatic valvulitis
Pathological mitral regurgitation (all 4 criteria met)
1.Seen in at least 2 views
2.Jet length ≥2 cm in at least 1 view
3.Peak velocity >3 m/s
4.Pansystolic jet in at least 1 envelope
Pathological aortic regurgitation (all 4 criteria met)
1.Seen in at least 2 views
2.Jet length ≥1 cm in at least 1 view
3.Peak velocity >3 m/s
4.Pan diastolic jet in at least 1 envelope
Morphological Findings on Echocardiogram in Rheumatic Valvulitis
Acute mitral valve changes
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
Chordal thickening and fusion
Restricted leaflet motion
Aortic valve changes in either acute or chronic carditis
Irregular or focal leaflet thickening
Restricted leaflet motion
5.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).
6. Diagnosis of Acute rheumatic fever:
For all patient populations with evidence of preceding GAS infection
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
Flow charts for diagnosis of rheumatic fever
7.Rheumatic Fever Recurrences
As stated in the 1992 guidelines, patients who have a history of ARF or RHD are at high risk for “recurrent” attacks if reinfected with group A streptococci. Such an attack is considered
a new episode of ARF, but one in which the complete set of Jones criteria, even as revised, may not be completely fulfilled.
The guideline recommendations for diagnosing rheumatic fever recurrences are:
1. With a reliable past history of ARF or established RHD, and in the face of documented group A streptococcal infection, 2 major or 1 major and 2 minor or 3 minor manifestations may be sufficient for a presumptive diagnosis (Class IIb; Level of Evidence C).
2. 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).
8.“Possible” Rheumatic Fever
In some circumstances, a given clinical presentation may not fulfill these updated Jones criteria, but the clinician may still have good reason to suspect that ARF is the diagnosis.
This may occur in high-incidence settings. In such situations the clinicians should use their discretion and clinical acumen to make the diagnosis that they consider most likely and manage the patient accordingly.
1. Where there is genuine uncertainty, it is reasonable to consider offering 12 months of secondary prophylaxis followed by reevaluation to include a careful history and physical examination in addition to a repeat echocardiogram (Class IIa; Level of Evidence C).
2. In a patient with recurrent symptoms (particularly involving the joints) who has been adherent to prophylaxis recommendations but lacks serological evidence of group A streptococcal infection and lacks echocardiographic evidence of valvulitis, it is reasonable
to conclude that the recurrent symptoms are not likely related to ARF, and discontinuation of antibiotic prophylaxis may be appropriate (Class IIa; Level of Evidence C).
Jones criteria needed revision to meet current technological advances and clinical needs. Strict application of echocardiography/Doppler findings may be used to fulfill the major criterion of carditis, even in the absence of classic auscultatory findings, providing that ambient loading conditions are taken into consideration. In addition, monoarthritis or polyarthralgia could be accepted as fulfilling the major criterion of arthritis, but only in moderate- to high-risk populations. For low-risk populations, monoarthritis is not included, and polyarthralgia remains a minor criterion. Similarly, the requirement for the presence of fever can be fulfilled with oral, tympanic, or rectal temperature documented at 38°C in moderate- to high-risk populations, but only at ≥38.5°C in others.