Jones Criteria for Acute Rheumatic Fever Diagnosis

Jones Criteria Calculator
Carditis
Polyarthritis
Chorea
Erythema Marginatum
Subcutaneous Nodules
Fever
Arthralgia
Elevated ESR or CRP
Prolonged PR Interval
Prior Strep Infection
Jones Criteria: 0 major / 0 minor
Evidence of Prior Strep Required
Jones Criteria require evidence of preceding streptococcal infection (positive throat culture, rapid strep test, or elevated/rising streptococcal antibody titer).
Diagnoses acute rheumatic fever based on major and minor criteria.

Why Use

To establish a diagnosis of initial or recurrent acute rheumatic fever. Failing to meet the criteria suggests that another diagnosis is more likely. Adherence to the Jones Criteria will help prevent over-diagnosis and the long-term sequelae of over-diagnosis such as prophylactic antibiotic use, patient/family stress, and insurance concerns.

When to Use

Use in the diagnosis of suspected acute rheumatic fever (ARF). Diagnosis of ARF is a clinical diagnosis based on these standard diagnostic criteria. Do not use to measure rheumatic activity, establish the diagnosis of inactive or chronic rheumatic heart disease, or to predict the course or severity of the disease.

Formula

Revised Jones Criteria ( Gewitz 2015 ): Diagnosis for initial ARF is positive if evidence of preceding group A streptococcal (GAS) infection is present AND two major criteria (or one major and two minor criteria) from the table below are present. Diagnosis for recurrent ARF is positive if evidence of preceding GAS infection is present, there is a previous history of ARF or established rheumatic heart disease, AND two major criteria (or one major and two minor OR three minor criteria) from the table below are present. Criteria Evidence of preceding GAS infection (≥1 of the following) Increased or rising anti-streptolysin O titer or other streptococcal antibodies (anti-DNASE B) A positive rapid group A streptococcal carbohydrate antigen test in a child whose clinical presentation suggests a high pretest probability of streptococcal pharyngitis A positive throat culture for group A β-hemolytic streptococci Major criteria Low-risk populations* Moderate- and high-risk populations Carditis (clinical and/or subclinical**) Carditis (clinical and/or subclinical**) Arthritis (polyarthritis only) Arthritis (monoarthritis or polyarthritis; polyarthralgia) Chorea Chorea Erythema marginatum Erythema marginatum Subcutaneous nodules Subcutaneous nodules Minor criteria Low-risk populations* Moderate- and high-risk populations Prolonged PR interval, after accounting for age variability (unless carditis is a major criterion) Prolonged PR interval, after accounting for age variability (unless carditis is a major criterion) Polyarthralgia Monoarthralgia Fever (≥38.5°C or 101.3°F) Fever (≥38°C or 100.4°F) ESR ≥60 mm in the first hour and/or CRP ≥3.0 mg/dL*** ESR ≥30 mm/hr and/or CRP ≥3.0 mg/dL*** *Low-risk populations are those with ARF incidence ≤2 per 100,000 school-aged children or all-age rheumatic heart disease prevalence of ≤1 per 1000 population per year. **Subclinical carditis indicates echocardiographic valvulitis. ***CRP value must be greater than the upper limit of normal for laboratory; use peak ESR values as ESR may evolve during the course of ARF.

Pearls / Pitfalls

An initial presentation of possible ARF should not be diagnosed based on minor criteria alone. Other illnesses may mimic ARF. Laboratory evidence of an antecedent group A streptococcal (GAS) infection (i.e., streptococcal antibody titers, positive throat culture or rapid Strep test) is mandatory to establish the diagnosis. Only GAS infections of the upper respiratory tract lead to ARF; GAS skin infections do not lead to ARF. A 2015 revision made modifications to the criteria, most notably providing two separate diagnostic pathways for those at low risk and those at moderate/high risk, and adding subclinical carditis as a major criterion. However, the 1992 (original) version remains popular in clinical use. Children less than 3 years of age are unlikely to develop an autoimmune response that leads to ARF. Even with laboratory evidence of GAS infection, consider other diagnoses first. Expert consultation with pediatric infectious disease, cardiology, and/or rheumatology may be helpful. Myocarditis without valvulitis (without new murmur or echo evidence suggestive of RHD) is unlikely to be rheumatic in origin.

Management

Echocardiogram is key in the evaluation of ARF, and should be performed in all cases of confirmed or suspected ARF. If the diagnosis of ARF has been made without evidence of structural heart disease, an echocardiogram and cardiology consult are still recommended.

Critical Actions

Household members living with a patient with confirmed ARF should be screened and treated for GAS infection/colonization when appropriate. A penicillin antibiotic is typically the first line treatment for ARF (oral penicillin V or intramuscular penicillin G); amoxicillin can also be considered.

Advice

Cases of isolated chorea, indolent carditis, or recurrent episodes of rheumatic fever are more suggestive of rheumatic fever regardless of the presence of other Jones Criteria. In these scenarios, ARF is the presumptive diagnosis until proven otherwise. Remember that arthralgia (joint pain) is distinct from arthritis (objective findings related to inflammation such as warmth, erythema, or swelling).

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