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Revision of the Jones Criteria for the Revision of the Jones Criteria for the Diagnosis of Acute Diagnosis of Acute Rheumatic Fever 2015 in the Era of Rheumatic Fever 2015 in the Era of Doppler Echocardiography Doppler Echocardiography Salem Deraz, MD Salem Deraz, MD Associate Professor of Pediatrics and Pediatric Cardiology Associate Professor of Pediatrics and Pediatric Cardiology European Board Certification of Congenital Echocardiography European Board Certification of Congenital Echocardiography (2014-2019) (2014-2019)

Modified Jones Criteria in the era of Echo

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Page 1: Modified Jones Criteria in the era of Echo

Revision of the Jones Criteria for the Revision of the Jones Criteria for the Diagnosis of AcuteDiagnosis of Acute

Rheumatic Fever 2015 in the Era of Rheumatic Fever 2015 in the Era of Doppler EchocardiographyDoppler Echocardiography

Salem Deraz, MD Salem Deraz, MD Associate Professor of Pediatrics and Pediatric CardiologyAssociate Professor of Pediatrics and Pediatric CardiologyEuropean Board Certification of Congenital Echocardiography European Board Certification of Congenital Echocardiography

(2014-2019)(2014-2019)

Page 2: Modified Jones Criteria in the era of Echo

Modified Jones's criteria(1992)

A. Major Criteria:

1.Carditis- Pericarditis: Stitching pain -Pericardial rub

- Myocarditis: Tachycardia out of proportion of fever - Gallop rhythm - Muffled heart sounds.

- Endocarditis: Edema of the Mitral valve⇒ MS⇒ transient mid-diastolic murmur (Carey combs murmur) - Destruction & deformity → Ml ± AI - On previous rheumatic heart⇒ change of character of old murmur or appearance of new murmurs

Where is the role of Echocardiography ?!!!!

Page 3: Modified Jones Criteria in the era of Echo

Modified Jones's criteria(1992)

A. Major Criteria:

2. Arthritis

•- Usually large Joints e.g. knees, ankle hip, shoulder, elbow or wrest.

•-Involved joints become ⇒ red, hot, swollen, tender + loss of function.

•-Fleeting polyarthritis (migratory) .

•-Leaving no residual damage or deformity .

•- Dramatic response to salicylates.

Page 4: Modified Jones Criteria in the era of Echo

Modified Jones's criteria(1992)

A. Major Criteria:

3. Chorea

4. Subcutaneous nodule

5. Erythema marginatum

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Modified Jones's criteria(1992)

B. Minor Criteria:

Clinical:

1. Fever

2. Arthralgia

Laboratory:

3. +ve Acute phase reactants (ESR, CRP, Lecocytosis)

4. Prolonged P-R interval in ECG

Page 6: Modified Jones Criteria in the era of Echo

Modified Jones's criteria(1992)

C. Evidence of streptococci infection:

1. Recent scarlet Fever

2. + ve throat cultures

3. ↑↑ ASOT (> 250 Todd's' unit ) & other streptococcal antibodies

Page 7: Modified Jones Criteria in the era of Echo

Modified Jones's criteria(1992)

Rheumatic fever can be diagnosed if there are

2 major criteria + evidence of streptococcal infection

Or

1 major + 2 minor + evidence of streptococcal infection

Page 8: Modified Jones Criteria in the era of Echo

Modified Jones's criteria(1992)

Exceptions to Johns' criteria:

1. Rheumatic Chorea

As rheumatic chorea can be the only manifestation of rheumatic fever, diagnosis can be made in absence of other major or minor manifestations provided that other causes of chorea have been excluded.

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2. Insidious or late onset carditis

Diagnosis can be made in absence of other major or minor manifestations provided that other causes of carditis have been excluded.

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3. Rheumatic recurrences

In patients with old documented chronic rheumatic heart disease, the diagnosis of rheumatic recurrences can be made in absence of major manifestations. Minor manifestations (fever or arthralgia) plus evidence of streptococcal infections is considered a presumptive diagnosis of recurrence.

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Why it was necessary to revise Jone’s Criteria 1992?

1. Subclinical carditis

Echocardiography had limited role in the diagnosis of carditis in the Jones criteria revision of 1992.

Numerous studies suggested to use of echocardiography as a way to diagnose carditis even in the absence of overt clinical findings (“subclinical carditis”).

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Why it was necessary to revise Jone’s Criteria 1992?

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Why it was necessary to revise Jone’s Criteria 1992?

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Why it was necessary to revise Jone’s Criteria 1992?

2. Monoarticular Arthritis:

Many publications suggested that in selective high-risk populations, monoarticular arthritis may be an indicator of the major manifestation of ARF.

3. Levels of Evidence categories:

Previous AHA ARF guidelines did not categorize recommendations using the current Recommendations and Levels of Evidence categories.

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Levels of Evidence categories

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Epidemiological Background: AHA Recommendations

• 1. It is reasonable to consider individuals to be at low risk for ARF if they come from a setting or population known to experience low rates of ARF or RHD (Class IIa; Level of Evidence C).

• 2. It is reasonable that where reliable epidemiological data are available, low risk should be defined as having an ARF incidence <2 per 100 000 school-aged children per year or the prevalence of RHD of ≤1 per 1000 population per year (Class IIa; Level of Evidence C).

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Epidemiological Background: AHA Recommendations

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

(Class I; Level of Evidence C).

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Epidemiological Background

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Epidemiological Background

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Epidemiological Background

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Epidemiological Background

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Epidemiological Background

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Epidemiological Background

So

EGYPTIAN CHILDREN

are at high risk or at least moderate risk depending on their reference population

(Class I; Level of Evidence C).

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Clinical Manifestations of ARF1. CarditisSubclinical carditis

When classic auscultatory findings of valvar dysfunction are not present but echocardiography/Doppler studies reveal mitral or aortic valvulitis.

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Clinical Manifestations of ARF1. Carditis

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Clinical Manifestations of ARF1. Carditis

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Clinical Manifestations of ARF1. Carditis

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Clinical Manifestations of ARF1. Carditis

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Clinical Manifestations of ARF1. Carditis

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Clinical Manifestations of ARF1. Carditis

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Clinical Manifestations of ARF1.Carditis: AHA Recommendations• 1. Echocardiography with Doppler should be

performed in all cases of confirmed and suspected ARF (Class I; Level of Evidence B).

• 2. It is 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 (Class IIa; Level of Evidence C).

Page 33: Modified Jones Criteria in the era of Echo

Clinical Manifestations of ARF 2. Arthritis - Reactive Arthritis

• Some pediatric patients with poststreptococcal reactive arthritis have later developed episodes of ARF or RHD, which indicates that the initial diagnosis should probably have been ARF.

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Clinical Manifestations of ARF 2. Arthritis - Aseptic Monoarthritis

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Clinical Manifestations of ARF Minor Manifestations

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Clinical Manifestations of ARF Minor Manifestations

• In the 1965 revision of the Jones criteria, the authors commented that during an episode of ARF, temperature usually exceeds 38°C, and in the 1992 revision, that was revised to 39°C.

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Clinical Manifestations of ARF Minor Manifestations

• In Australian high risk population, the definition of fever as a temperature >38°C has resulted in improved sensitivity, with 75% of individuals with ARF.

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Rheumatic fever recurrence:AHA Recommendations

1. With a reliable past history of ARF or established RHD, and in the face of documented group A streptococcal infection, 2 major or 1 major and 2 minor or 3 minor manifestations may be sufficient for a presumptive diagnosis (Class IIb; Level of Evidence C).

Page 39: Modified Jones Criteria in the era of Echo

Rheumatic fever recurrence:AHA Recommendations

2. When minor manifestations alone are present, the exclusion of other more likely causes of the clinical presentation is recommended before a diagnosis of an ARF recurrence is made (Class I; Level of Evidence C).

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Revised Jones Criteria 2015

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“Possible” Rheumatic Fever: AHA Recommendations

1. Where there is doubt, it is reasonable to consider offering 12 months of secondary prophylaxis followed by reevaluation and repeat echocardiogram (Class IIa; Level of Evidence C).

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“Possible” Rheumatic FeverAHA Recommendations

2. In patients with recurrent symptoms (particularly involving the joints) who are on prophylaxis but lacks serological evidence of group A streptococcal infection and echocardiographic evidence, it is reasonable to conclude that the recurrent symptoms are not likely related to ARF, and discontinuation of antibiotic prophylaxis (Class IIa; Level of Evidence C).

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