Acute rheumatic fever | What are the new Jones criteria?

Table of Contents

This guide covers the 2015 revision of Jones's criteria for diagnosis of Acute Rheumatic Fever. It also covers clinical features and diagnostic approaches to patients with echocardiographic carditis (also called as subclinical), arthritis, Sydenham chorea, and Erythema margiantum with respect to acute rheumatic fever.

It is the first important revision to the Jones Criteria by the American Heart Association since 1992.

It is focused on epidemiological differences in high-risk and low-risk populations and led by technology (ECHO and doppler)

Based on epidemiological data, the new Jones criteria for the diagnosis of acute rheumatic fever divides the cases into two main groups - Low risk and high-risk population.

Why do new jones criteria 2015 consider high-risk and low-risk categories?

Epidemiological Background

In the current era, the incidence and prevalence of ARF and RHD have reduced substantially in developed nations. In developing countries though, the incidence continues to remain high with uneven global distribution of ARF/RHD.

What are the low and high risk categories?

Low-risk population

ARF incidence ≤2 per 100 000 school-aged children or all-age RHD prevalence of ≤1 per 1000 population year

High-risk population

Children not clearly from a low-risk population

Reason

By setting variations of criteria in both categories, the new Jones criteria ensure high sensitivity among those at greatest risk and high specificity for those at lower risk.

Current Jones Criteria for diagnosis of rheumatic fever

First episode of acute rheumatic fever

Evidence of recent streptococcal infection + 2 Major or 1 major + 2 minor          

Diagnosis of recurrent ARF

Although patients with a history of ARF are at high risk for recurrences, specific diagnostic criteria were missing in1992 revision.

2015 revision provides direct guidance as

Reliable past history of ARF/RHD + Evidence of recent streptococcal infection + 2 Major or 1 major + 2 minor or 3 minor criteria.

What are the Major criteria?

For all patient populations with evidence of preceding GAS infection

Individual criteria are explained in the approach to diagnosis section

What is subclinical carditis?

Subclinical carditis is a situation where classic auscultatory findings of valvar dysfunction either are not present or are not recognized by the diagnosing clinician but Doppler studies reveal mitral or aortic valvulitis.

Pathological mitral regurgitation (all 4 criteria met)

1. Seen in at least 2 views
2. Jet length ≥2 cm in at least 1 view
3. Peak velocity >3 m/s
4. Pansystolic jet in at least 1 envelope

Pathological aortic regurgitation (all 4 criteria met)

1. Seen in at least 2 views
2. Jet length ≥1 cm in at least 1 view
3. Peak velocity >3 m/s
4. Pan diastolic jet in at least 1 envelope

What are the Minor criteria?

For all patient populations with evidence of preceding GAS infection

How to prove evidence of Preceding Streptococcal Infection?

Note that the diagnosis of acute rheumatic fever is doubtful when evidence of preceding Group A streptococcal infection is not available.

Exceptions to this rule are

1. Chorea (Maybe the only manifestation of rheumatic fever at the time of its presentation)
2. Chronic, indolent rheumatic carditis with insidious onset and slow progression. Such patients don't have an identifiable history of ARF and had subclinical carditis that was not detected previously.

Evidence of recent Group A strep infection - Any 1 of the following (As per AHA)

1. Increased or rising anti-streptolysin O titer or other streptococcal antibodies (anti-DNASE) Note that A rise in titer is better evidence than a single titer result.
2. Positive throat culture for group A β-hemolytic streptococci.
3. Positive rapid group A streptococcal carbohydrate antigen test in a child whose clinical presentation suggests a high pretest probability of streptococcal pharyngitis.

What are the Difficulties in the Interpretation of streptococcal serology?

Note that Interpretation of streptococcal serology results can be difficult in populations with endemic skin or upper respiratory group A streptococcal infections.

In these settings, a negative streptococcal antibody test helps to exclude a recent infection, but a positive test does not necessarily indicate an infection in the past few months.

Clinical Manifestations of ARF

Generally, the clinical profile of ARF in low- and middle-income countries closely resembles that of high-income countries.

The most common major manifestations during the first episode of ARF in descending order are

Diagnostic approach to acute rheumatic fever

A. Carditis in acute rheumatic fever

Previous AHA Jones criteria (1992) mention carditis as a clinical diagnosis based on the auscultation of typical murmurs that indicate MR or AR, at either or both valves.

Note that Valvulitis is by far the most consistent feature of ARF, and isolated pericarditis or myocarditis is rarely rheumatic in origin.

With declining auscultatory skills and widespread use of ECHO, the concept of subclinical carditis is introduced as a valid rheumatic fever major manifestation.

Flowchart for approaching case of carditis in acute rheumatic fever

Subclinical carditis - Refer above for the definition and criteria

Recommendations for ECHO in AHA 2015 revision

1. Echocardiography with Doppler should be performed in all cases of confirmed and suspected ARF.
2. Reasonable to consider performing serial echocardiography/Doppler studies in any patient with diagnosed or suspected ARF even if documented carditis is not present on the diagnosis.
3. Echocardiography/Doppler testing should be performed to assess whether carditis is present in the absence of auscultatory findings, particularly in moderate- to high-risk populations and when ARF is considered likely.
4. Echocardiography/Doppler findings not consistent with carditis should exclude that diagnosis in patients with a heart murmur otherwise thought to indicate rheumatic carditis.

B. Arthritis in acute rheumatic fever

Flowchart for approaching case of arthritis in acute rheumatic fever

Migratory polyarthritis IN ARF

1. Most frequently involved are larger joints such as knees, ankles, elbows, and wrists. Involvement of small joints of the hands and feet and the spine is much less common in ARF than in other arthritic illnesses.
2. Rapid improvement with salicylates or nonsteroidal anti-inflammatory drugs is characteristic.
3. Self-limited course, even without therapy, lasting ≈4 weeks.
4. No long-term joint deformity.

Aseptic Monoarthritis

Studies have indicated that aseptic monoarthritis may be an important clinical manifestation. This led to the inclusion of monoarthritis as a major criterion in high-risk populations.

Polyarthralgia

Polyarthralgia is a very common but nonspecific manifestation of many rheumatic disorders.

But children with polyarthralgia are more likely to have ARF if they come from a high-incidence population. Therefore, it is included as a minor criterion for low-risk and a major for the high-risk group in newly revised jones criteria.         

C. Sydenham Chorea in acute rheumatic fever

Chorea associated with acute rheumatic fever is characterized by purposeless, involuntary and non-stereotypical movements of the trunk or extremities. It is commonly associated with muscle weakness and emotional lability.

What is a stereotypical movement?

Repetitive, fixed, predictable movement like hand banging.

Flowchart for approaching case of Chorea in acute rheumatic fever

D. Erythema marginatum in acute rheumatic fever

Erythema marginatum is a unique, evanescent, pink rash with pale centers and rounded margins. Classically seen on the trunk and proximal extremities and is not facial.

Heat can induce its appearance, and it blanches with pressure. It is harder to detect in dark-skinned individuals.

E. Subcutaneous nodules in acute rheumatic fever

Firm, painless protuberances are found on extensor surfaces at specific joints, such as knees, elbows, wrists, occiput, and along the spinous processes vertebrae.

They are commonly seen in those who also have carditis.

Similar to erythema marginatum, subcutaneous nodules almost never occur as the sole major manifestation of ARF.

Flowchart for approaching a patient with erythema marginatum and subcutaneous nodule in acute rheumatic fever

Rheumatic Fever Recurrences

1992 AHA guidelines mention that children with a history of acute rheumatic fever or established RHD are at high risk for recurrence if reinfected with group A streptococci.

Read the criteria for diagnosing Rheumatic Fever Recurrences above.

Possible Rheumatic Fever

Sometimes the required criteria may not be fulfilled for the diagnosis of acute rheumatic fever.

Therefore in a high incidence population, the clinician must use clinical acumen to make the likely diagnosis and start management rather than refuting the diagnosis based on the fulfillment of all the criteria. This is specifically important in areas where rheumatic fever and rheumatic heart disease are highly prevalent.

AHA recommendations for possible rheumatic fever are

1. 12 months of secondary prophylaxis followed by reevaluation with history and physical examination with repeat echo.
2. For a patient who is strictly adherent to prophylaxis recommendations but still has recurrent symptoms and also lacks serological evidence of group A streptococcal infection and ECHO evidence of valvulitis, it is reasonable to rule out rheumatic fever and discontinue antibiotic prophylaxis.

Video by Michael H. Gewitz, the lead author of the statement, about brief explaination on revision.

Author

DR Faraz Ahmed Khan | DNB Pediatrics