Modified Jones criteria

• Used to diagnose acute rheumatic fever

Criteria

Low-risk populations	Moderate- and high-risk populations
Preceding infection
Increased/rising ASOT or anti-DNASE B
Positive throat culture
Positive rapid antigen test in a classic case
Major criteria
Carditis (clinical or subclinical)	Carditis (clinical or subclinical)
Arthritis (polyarthritis)	Arthritis (monoarthritis, polyarthritis, or polyarthralgia)
Chorea	Chorea
Erythema marginatum	Erythema marginatum
Subcutaneous nodules	Subcutaneous nodules
Minor criteria
Polyarthralgia (without arthritis)	Monoarthralgia (without arthritis)
Fever ≥38.5ºC	Fever ≥38ºC
ESR ≥60 mm/h or CRP ≥3 mg/dL	ESR ≥30 mm/h or CRP ≥3 mg/dL
Prolonged PR interval (without carditis)	Prolonged PR interval (without carditis)

• Low-risk = ≤2 cases of acute rheumatic fever per 100,000 school-aged children, or ≤1 case of rheumatic heart disease per 1000 people of any age

Preceding Streptococcus pyogenes Infection

• The diagnosis of acute rheumatic fever requires evidence of preceding streptococcal infection, as indicated by one of the following:
  • Increased or rising anti-streptolysin O titre, or other streptococcal antibody such as anti-DNASE B; a rising titre is considered stronger evidence
  • A positive throat culture for Streptococcus pyogenes
  • A positive rapid streptococcal antigen test in a child whose clinical presentation suggests a high pretest probability of streptococcal pharyngitis

Major Criteria

• Polyarthritis: A temporary migrating inflammation of the large joints, usually starting in the legs and migrating upwards.
• Carditis: Inflammation of the heart muscle (myocarditis) which can manifest as congestive heart failure with shortness of breath, pericarditis with a rub, or a new heart murmur. This includes subclinical carditis suggested by valvulitis on echo.
• Subcutaneous nodules: Painless, firm collections of collagen fibers over bones or tendons. They commonly appear on the back of the wrist, the outside elbow, and the front of the knees.
• Erythema marginatum: A long-lasting reddish rash that begins on the trunk or arms as macules, which spread outward and clear in the middle to form rings, which continue to spread and coalesce with other rings, ultimately taking on a snake-like appearance. This rash typically spares the face and is made worse with heat.
• Sydenham's chorea (St. Vitus' dance): A characteristic series of involuntary rapid movements of the face and arms. This can occur very late in the disease for at least three months from onset of infection.

Minor Criteria

• Fever
• Arthralgia: joint pain without swelling (cannot be included if arthritis is present as a major symptom)
• Evidence of inflammation: raised erythrocyte sedimentation rate or C reactive protein
• Heart block: ECG showing features of heart block, such as a prolonged PR interval (cannot be included if carditis is present as a major symptom)

Interpretation

• Initial acute rheumatic fever
  • Evidence of preceding Streptococcus pyogenes infection, plus either:
    • 2 major criteria, or
    • 1 major criterion and 1 minor criteria
• Recurrent acute rheumatic fever
  • Evidence of preceding Streptococcus pyogenes infection, plus one of:
    • 2 major criteria, or
    • 1 major criterion and 2 minor criteria, or
    • 3 minor criteria
• Possible acute rheumatic fever
  • Not meeting above criteria but high clinical suspicion

Further Reading

• Revision of the Jones Criteria for the Diagnosis of Acute Rheumatic Fever in the Era of Doppler Echocardiography. Circulation. 2015;131:1806-1818. doi: 10.1161/CIR.0000000000000205