Technology Enhances Historical Criteria: The Revision of the Jones Criteria for Acute Rheumatic Fever

Acute rheumatic fever (ARF) remains one of the major causes of cardiovascular disease burden throughout the world particularly among the young.¹ ARF is especially problematic in countries with limited economic resources. The American Heart Association (AHA) has historically been a leader in guiding diagnosis of ARF and in helping to formulate appropriate management plans. In addition, the AHA, in partnership with a number of other health organizations throughout the world, has focused tremendous time, energy, and resources to prevent and combat ARF and its cardiovascular consequences.

The diagnostic accuracy for ARF was advanced tremendously with the pioneering work of Dr. T. Duckett Jones.² In the 1940s, Dr. Jones, through careful analysis of published literature and his own clinical experience, helped to formulate diagnostic criteria to allow the appropriate identification and treatment of those with ARF. Since that time, the AHA has been a partner in publicizing and revising the “Jones Criteria.” These are among the most oft-cited diagnostic criteria in our field. In keeping with Dr. Jones’ belief that the criteria should have a high specificity in order to allow the fewest number of missed cases, the AHA has periodically re-evaluated the diagnostic criteria. We are fortunate therefore to be able to share the most recent revision of the Jones Criteria.³ This work was led by the RFEKD Committee of the Council on Lifelong Congenital Heart Disease and Heart Health in the Young (Young Hearts) and is endorsed by the World Heart Federation.

The newest revisions have a number of key changes that will improve the diagnostic accuracy of the Jones Criteria. The expert panel of authors recognizes that the yield of screening test depends largely upon the likelihood that the disease is present in the population. Based on some prior work from epidemiologists in Australia, it was found to be helpful to stratify populations into those that are at higher risk or lower risk for ARF.⁴ Low risk is considered those cases in which the ARF incidence is <2:100,000 school-aged children per year. Therefore, the revised Jones Criteria guidelines are stratified by either those that are high-risk populations or low-risk populations. This is important in that some findings, such as polyarthralgia or mono-arthritis, should be considered major diagnostic criteria in the high-risk population. This would not be true in the low-risk population. Similarly, the writing group has carefully reviewed evidence on some of the minor criteria used to make the diagnosis of ARF. Studies from examining the indigenous population in Australia have examined the contribution of fever to the diagnostic accuracy of the Jones Criteria.⁵ In the previous revision of the Jones Criteria, a fever needed to be greater than 39°C to be considered as a risk factor. However, recent data have suggested that lower grade fevers can be helpful in assessing for the presence of ARF. Therefore, in low-risk populations, fever > 38.5°C would be considered a minor criterion. Whereas in the high-risk population, fevers > 38°C would be consistent with ARF. Many other diagnostic criteria from the Jones Criteria remain unchanged, as they have stood the test of time quite well.

The most important revision in the Jones Criteria relates to the use of echocardiography. Echocardiography is now recognized as an essential clinical compliment to the physical exam, electrocardiogram, and serum diagnostic studies. Up until this point echocardiographic findings were not considered essential and had always been considered secondary to other clinical findings of carditis. However, numerous publications from throughout the world have demonstrated how echocardiography can improve our detection of ARF. Carditis represents inflammation of the endocardium, myocardium, and pericardium. Valvulitis is the most common feature of carditis. For many decades carditis was identified by ausculatation. However, with the widespread use of echocardiography, it is now recognized that carditis may often occur in the absence of auscultatory findings such as a murmur. For the purpose of the revisions of the diagnostic criteria, the authors use the term subclinical carditis. This is meant to describe valvular dysfunction without the presence of any auscultatory findings.

Importantly, newer evidence suggests that by adding subclinical carditis, identified by echocardiography, an additional 15% to 20% of patients with ARF can be identified who otherwise would have been missed.^6,7 As such, in the revised guidelines, echocardiographic finding of valvulitis would now meet criteria for a major criteria for ARF. The definition of valvulitis is provided in a table within the statement. The expert panel recommends that echocardiography with Doppler be performed in all cases of confirmed or suspected ARF. Echocardiography may have been considered a challenge in many developing nations in previous decades, owing to the technical necessity and costs related to echocardiography. However, echocardiography has become more widely available and smaller portable units make this practical even in developing nations. A number of studies have shown that by incorporating echocardiography in developing nations, the diagnostic accuracy for ARF can be improved substantially.

The Jones Criteria remain one of the remarkable advances in cardiac health in the last 100 years. They were designed out of a determination of Dr. Jones and other leaders to enhance detection of ARF through a simple and evidence-based approach. The AHA has been a leader in publicizing and monitoring these criteria over time. The most recent revisions strengthen the Jones Criteria and follow both the underlying premise of the initial publication as well as the latest approaches to evidence-based medicine. These latest revisions will be welcomed by healthcare providers worldwide. Undoubtedly, they will result in improved detection of ARF and the best use of limited resources throughout the world. As is the case with so many comprehensive statements and guidelines, the AHA is dedicated to the expert writing committee for hundreds of hours of work devoted to this endeavor. Dr. T. Duckett Jones will always remain one of the most esteemed members of the AHA and his work is proudly continued through the RFEKD committee of Young Hearts.