Jones Criteria for Acute Rheumatic Fever Diagnosis
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
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).