Revised Jones Criteria 2015: Summarised

by Dr Faraz Ahmed Khan
dr jonesThe famous Jones criteria for diagnosis of Acure Rhemaric Fever are recently revised in 2015 by AHA with emphasis on doppler echocardiogarphy for involvement of heart.

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

It is technology driven (ECHO) and focuses on epidemiological differences in high-risk and low-risk populations.

This is a effort to summarize important points for Pediatric post graduates exams.

link to article : Here
(For any discrepancy refer original Article)

Epidemiological Background

1. During the 20th century, the incidence of ARF and the prevalence of RHD declined substantially in Europe, North America, and developed nations.

2. Attributed to improved hygiene, improved access to antibiotic drugs and medical care, reduced household crowding, and other social and economic changes.

3. However, in developing countries the incidence remains high.
In summary, the global distribution of ARF/RHD is clearly disproportionate. Surprised smile

As per epidemiological data, cases are divided into:

Low risk should be defined as having an ARF incidence <2 per 100 000 school-aged children (usually 5–14 years old) per year.

Or

an allege prevalence of RHD of ≤1 per 1000 population per year.

Children not clearly from a low-risk population are at moderate to high risk depending on their reference population.


Revised Jones Criteria
A. Diagnosis
For all patient populations with evidence of preceding GAS infection
          initial ARF - 2 Major or 1 major plus 2 minor
          recurrent ARF - 2 Major or 1 major and 2 minor or 3 minor

B. What are Major criteria ? 

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C. What are the Minor criteria

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Evidence of Preceding Streptococcal Infection

“ Laboratory evidence of antecedent group A streptococcal infection is needed whenever possible, and the diagnosis is in doubt when such evidence is not available  ”

Exceptions include chorea, chronic, indolent rheumatic carditis with insidious onset and slow progression. This latter problem refers to patients without an identifiable history of ARF who have had subclinical carditis that was not detected previously.

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.

Any 1 of the following can serve as evidence of preceding infection, per a recent AHA statement :

1. Increased or rising anti-streptolysin O titer or other streptococcal antibodies (anti-DNASE B). A rise in titer is better evidence than a single titer result.

2. A positive throat culture for group A β-hemolytic streptococci.

3. A positive rapid group A streptococcal carbohydrate antigen test in a child whose clinical presentation suggests a high pretest probability of streptococcal pharyngItis.

 Clinical Manifestations of ARF

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

2. Most common major manifestations during the first episode of ARF remain carditis (50%–70%) and arthritis (35%–66%).

3. These are followed in frequency by chorea (10%–30%), which has been demonstrated to have a female predominance, and then subcutaneous nodules (0%–10%) and erythema marginatum (<6%), which remain much less common but highly specific manifestations of ARF. Pointing up

However in very high-risk populations, such as the indigenous Australian population, variability in typical Jones criteria manifestations has been described. As discussed below, these include presentations with

        1. aseptic monoarthritis
        2. polyarthralgia and
        3. low-grade (as opposed to traditionally considered high-grade) fevers.

                             1. Carditis

1. As per 1992 AHA revised Jones criteria statement, carditis as a major manifestation of ARF was mostly a clinical diagnosis based on the auscultation of typical murmurs that indicate MR or AR, at either or both valves.

(Valvulitis is by far the most consistent feature of ARF, and isolated pericarditis or myocarditis should rarely, if ever, be considered rheumatic in origin.)

2. With declining auscultatory skills and widespread use of ECHO, the concept of subclinical carditis has become incorporated into other guidelines and consensus statements as a valid rheumatic fever major manifestation.

3. Subclinical carditis refers exclusively to the circumstance in which 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.

4. Specific Doppler Findings in Rheumatic Valvullitis and Morphological Findings on Echocardiogram in Rheumatic Valvulitis (both acute and chronic) are distinctly defined in the guidelines. 

Additional recommendations include:

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 diagnosis.

3. Echocardiography/Doppler testing should be performed (strictly fulfilling the findings defined) 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.
                     
               2. Arthritis  

1. Migratory polyarthritis

2. Most frequently involved are larger ones, including 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.

3. Rapid improvement with salicylates or nonsteroidal anti-inflammatory drugs is characteristic.

4. Self-limited course, even without therapy, lasting ≈4 weeks.

5. No long-term joint deformity.

Aseptic Monoarthritis

Studies from India, Australia, and Fiji have indicated that aseptic monoarthritis may be important as a clinical manifestation of ARF in selected high-risk populations.

Polyarthralgia

Polyarthralgia is a very common, highly nonspecific manifestation of a number of rheumatologic disorders.
No compelling evidence to amend this conclusion in low risk.
                                   But
Children with polyarthralgia are more likely to have ARF if they come from a population with a high incidence population.

Therefore, it is minor criteria for low risk, major for high risk!
      
      Chorea (Sydenham Chorea)
1. Purposeless, involuntary, nonstereotypical movements of the trunk or extremities.
2. Associated with muscle weakness and emotional lability.
                 
                          Skin Findings

1.Erythema marginatum.

a. Unique, evanescent, pink rash seen with pale centers and rounded or serpiginous margins.
b. Usually is present on the trunk and proximal extremities and is not facial.

Heat can induce its appearance, and it blanches with pressure.

Harder to detect in dark-skinned individuals.

2. Subcutaneous nodules

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

Common in those who also have carditis, and as with erythema marginatum, subcutaneous nodules almost never occur as the sole major manifestation of ARF.

Rheumatic Fever Recurrences

As stated in the 1992 guidelines, patients who have a history of ARF or RHD are at high risk for “recurrent” attacks if reinfected with group A streptococci.  

                “Possible” Rheumatic Fever

A given clinical presentation may not fulfill these updated Jones criteria, but the clinician may still have good reason to suspect that ARF is the diagnosis. This may occur in high-incidence settings.

In such situations, clinicians should use their discretion and clinical acumen to make the diagnosis that they consider most likely and manage the patient accordingly.

AHA recommendations for management of ''possible'' rheumatic fever are:

1. In genuine uncertainty, 12 months of secondary prophylaxis followed by reevaluation including careful history and physical examination with repeat echo.

2. In recurrent symptoms (particularly involving the joints) who has been adherent to prophylaxis recommendations but lacks serological evidence of group A streptococcal infection and lacks echo evidence of valvulitis, it is reasonable to conclude that the recurrent symptoms are not likely related to ARF, and discontinuation of antibiotic prophylaxis may be appropriate.

                 Want to read more ?

                               Source: http://my.americanheart.org/
                           Here is the link for above article

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

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