Criteria

Jones criteria[2]

The Jones criteria were established in 1944 to help clinicians make the diagnosis of acute rheumatic fever (ARF), as there is no single gold standard diagnostic test.[57] The criteria have been revised several times by the American Heart Association in response to falling rates of ARF in the US. Early revisions increased the specificity but reduced the sensitivity of the criteria.[83][84][85][86] The 1992 update specified for the first time that the criteria apply only to the initial diagnosis of ARF.

The 2015 revision of the criteria provide two different sets of criteria: one for low-risk populations (i.e., those with a rheumatic fever incidence ≤2 in 100,000 school-aged children or all-age rheumatic heart disease prevalence ≤1 in 1000 population per year) and one for moderate- to high-risk populations where increased sensitivity is important.[2] Patients not clearly from a low-risk population are deemed to be at moderate-to-high risk depending on their reference population.

Low-risk populations

  • Five manifestations are considered major manifestations of ARF:

    • Carditis (clinical and/or subclinical)

    • Polyarthritis

    • Chorea

    • Erythema marginatum

    • Subcutaneous nodules.

  • Four manifestations are considered minor manifestations of ARF:

    • Fever (≥101.3°F [≥38.5°C ])

    • Polyarthralgia

    • Elevated inflammatory markers (erythrocyte sedimentation rate [ESR] ≥60mm/hour and/or C-reactive protein [CRP] ≥3.0 mg/dL)

    • Prolonged PR interval on electrocardiogram.

Moderate- to high-risk populations

  • Five manifestations are considered major manifestations of ARF:

    • Carditis (clinical and/or subclinical)

    • Arthritis (monoarthritis or polyarthritis; polyarthralgia can be considered as a major manifestation if other causes are ruled out)

    • Chorea

    • Erythema marginatum

    • Subcutaneous nodules.

  • Four manifestations are considered minor manifestations of ARF:

    • Fever (100.4°F [≥38.0°C])

    • Monoarthralgia

    • Elevated inflammatory markers (ESR ≥30 mm/hour and/or CRP ≥3.0 mg/dL)

    • Prolonged PR interval on electrocardiogram.

Chorea does not require evidence of a preceding group A streptococcal infection. In a patient in whom arthritis is considered a major manifestation, arthralgia cannot be counted as a minor manifestation. In a patient in whom carditis is considered as a major manifestation, prolonged PR interval cannot be counted as a minor manifestation.

Doppler echocardiogram findings in rheumatic valvulitis

  • Pathological mitral regurgitation (all 4 criteria met):

    • Seen in at least two views

    • Jet length ≥2 cm in at least one view

    • Peak velocity >3 m/s

    • Pansystolic jet in at least one envelope.

  • Pathological aortic regurgitation (all 4 criteria met):

    • Seen in at least two views

    • Jet length ≥1 cm in at least one view

    • Peak velocity >3 m/s

    • Pan diastolic jet in at least one 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.

Recurrent ARF

  • Patients who have had a single episode of rheumatic fever are at high risk of subsequent episodes of recurrent rheumatic fever. The Jones criteria allow diagnosis of recurrent rheumatic fever if there are:

    • 2 major manifestations, or

    • 1 major and 2 minor manifestations, or

    • 3 minor manifestations.

All other likely diagnoses should be excluded before a diagnosis of recurrent rheumatic fever, especially in patients presenting with 3 minor manifestations.

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