2. DIAGNOSTIC CRITERIA
• JONES CRITERIA
• MODIFIED JONES CRITERIA (1992)
• 2002-2003 WHO CRITERIA FOR DIAGNOSIS OF
RHEUMATIC FEVER AND RHEUMATIC HEART DISEASE
3. JONES CRITERIA:
Because there is no definitive test, the diagnosis of ARF relies on the presence of a
combination of typical clinical features together with evidence of the precipitating
group A streptococcal infection, and the exclusion of other diagnoses. This uncertainty
led Dr. T. Duckett Jones in 1944 to develop a set of criteria (subsequently known as the
Jones criteria) to aid in the diagnosis. The current guidelines are an update of these
criteria
4. MODIFIED JONES CRITERIA (1992):
Diagnostic categories Diagnostic criteria
Major criteria • Carditis
• Polyarthritis
• Chorea
• Erythema marginatum
• Subcutaneous nodules
Minor criteria • Fever
• Arthralgia
• Previous rheumatic fever or rheumatic heart disease
• Prolonged P-R interval on electrocardiogram (ECG)
Evidence of preceding streptococcal infection • Elevated C-Reactive Protein
• Positive throat culture for Group A beta-hem,olytic
streptococci
• Increased antistreptolysin O or other streptococcal
antibodies.
5. 1992 Revised Jones criteria do not include elevated leukocyte count
as a laboratory minor manifestation (but do include elevated C-
reactive protein), and do not include recent scarlet fever as
supporting evidence of a recent streptococcal infection
6. AN EXPERT PANEL CONVENED BY THE WORLD HEALTH ORGANIZATION (WHO) CLARIFIED THE
USE OF THE JONES CRITERIA IN ARF RECURRENCES . BECAUSE EACH REVISION OF THE JONES
CRITERIA SINCE 1944 HAS REDUCED SENSITIVITY AND INCREASED SPECIFICITY, IN RESPONSE TO
THE DECLINE IN INCIDENCE OF ARF IN HIGH-INCOME COUNTRIES, THERE IS NOW CONCERN
THAT THEY MAY BE TOO INSENSITIVE FOR COUNTRIES WHERE ARF INCIDENCE REMAINS HIGH.
AS A RESULT, SOME COUNTRIES (E.G., AUSTRALIA AND NEW ZEALAND) HAVE DEVELOPED THEIR
OWN, MORE SENSITIVE, DIAGNOSTIC CRITERIA FOR ARF IN THEIR POPULATIONS.
2002-2003 WHO CRITERIA FOR DIAGNOSIS OF
RHEUMATIC FEVER AND RHEUMATIC HEART DISEASE
7. 2002-2003 WHO CRITERIA FOR DIAGNOSIS
OF RHEUMATIC FEVER AND RHEUMATIC
HEART DISEASE
Diagnostic categories Diagnostic criteria
Major manifestations
• Carditis
• Polyarthritis
• Chorea
• Erythema marginatum
• Subcutaneous nodules
Minor manifestations
• Clinical: Fever, polyarthralgia
• Lab. finding: Elevated E.S.R. or leucocyte count
• ECG: Prolonged P-R interval
8. 2002-2003 WHO CRITERIA FOR DIAGNOSIS
OF RHEUMATIC FEVER AND RHEUMATIC
HEART DISEASE
Diagnostic categories Diagnostic criteria
Supporting evidence of preceding
streptococcal infection within the last
45 days
• Elevated or rising Anti-Streptolysin
O or other streptococcal antibody or,
• A positive throat culture or,
• Rapid antigen test for group A
streptococcus or,
• Recent scarlet fever
9. 2002-2003 WHO CRITERIA FOR DIAGNOSIS
OF RHEUMATIC FEVER AND RHEUMATIC
HEART DISEASE
Diagnostic categories Diagnostic criteria
Primary episode of
rheumatic fever
Two major
or,
(one major and two minor
manifestations + evidence of preceding
group A streptococcal infection)
10. Patients may present with polyarthritis (or with only
polyarthralgia or mono arthritis) and with several (three or
more) other minor manifestations, together with evidence of
recent group A streptococcal infection. Some of these cases
may later turn out to be rheumatic fever. It is prudent to
consider them as cases of "probable rheumatic fever" (once
other diagnoses are excluded) and advise regular secondary
prophylaxis. Such patients require close follow-up and regular
examination of the heart. This cautious approach is
particularly suitable for patients in vulnerable age groups in
high incidence settings
11. 2002-2003 WHO CRITERIA FOR DIAGNOSIS
OF RHEUMATIC FEVER AND RHEUMATIC
HEART DISEASE
Diagnostic categories Diagnostic criteria
Recurrent attack of
rheumatic fever in a
patient without
established rheumatic
heart disease
Two major
or,
(one major and two minor
manifestations + evidence of preceding
group A streptococcal infection)
12. 2002-2003 WHO CRITERIA FOR DIAGNOSIS
OF RHEUMATIC FEVER AND RHEUMATIC
HEART DISEASE
Diagnostic categories Diagnostic criteria
Recurrent attack of
rheumatic fever in a
patient with established
rheumatic heart disease
Two minor manifestations + evidence
of preceding group A streptococcal
infection)
13. 2002-2003 WHO CRITERIA FOR DIAGNOSIS
OF RHEUMATIC FEVER AND RHEUMATIC
HEART DISEASE
Diagnostic categories Diagnostic criteria
Rheumatic chorea Insidious onset
rheumatic Carditis
Other major manifestations or evidence of
group A streptococcal infection NOT
REQUIRED
14. In case of Rheumatic chorea insidious onset rheumatic
Carditis and Recurrent attack of rheumatic fever in a patient
with established rheumatic heart disease, infective
endocarditis should be excluded.
15. 2002-2003 WHO CRITERIA FOR DIAGNOSIS
OF RHEUMATIC FEVER AND RHEUMATIC
HEART DISEASE
Diagnostic categories Diagnostic criteria
Chronic valve lesions of rheumatic
heart disease (patients presenting for
the first time with pure mitral
stenosis or mixed mitral valve
disease and/or aortic valve disease)
Do not require any other criteria to be
diagnosed as having rheumatic heart
disease
16. In case of Chronic valve lesions of rheumatic heart disease ,
congenital heart disease should be excluded.
17. RECOMMENDED TESTS IN
CASES OF POSSIBLE ACUTE
RHEUMATIC FEVER
• RECOMMENDED FOR ALL CASES
• TESTS FOR ALTERNATIVE DIAGNOSIS
DEPENDED UPON CLINICAL FEATURES
18. RECOMMENDED TESTS FOR ALL PROBABLE
CASES OF ACUTE RHEUMATIC FEVER
• White blood cell count
• Erythrocyte sedimentation rate
• C-reactive protein
• Blood cultures if febrile
• Electrocardiogram (repeat in 2 weeks and 2 months if prolonged P-R interval or other rhythm
abnormality)
• Chest x-ray if clinical or echocardiographic evidence of Carditis
• Echocardiogram (consider repeating after 1 month if negative)
• Throat swab (preferably before giving antibiotics)–culture for group A streptococcus
• Anti-streptococcal serology: both anti-streptolysin O and anti-DNase B titres, if available (repeat 10–
14 days later if 1st test not confirmatory)
19. TESTS FOR ALTERNATIVE DIAGNOSIS DEPENDED
UPON CLINICAL FEATURES
• Repeated blood cultures if possible endocarditis
• Joint aspirate (microscopy and culture) for possible
septic arthritis
• Copper, ceruloplasmin, anti-nuclear antibody, drug
screen for choreiform movements
• Serology and auto-immune markers for arboviral, auto-
immune or reactive arthritis
21. TREATMENT:
BED REST & SUPPORTIVE THERAPY:
• Bed rest is important, as it lessens joint pain and reduces cardiac workload.
• Patients can then return to normal physical activity but strenuous exercise should
be avoided in those who have had carditis.
• Cardiac failure should be treated as necessary. Some patients, particularly those in
early adolescence, develop a fulminant form of the disease with severe mitral
regurgitation and sometimes concomitant aortic regurgitation. If heart failure in
these cases does not respond to medical treatment, valve replacement may be
necessary and is often associated with a dramatic decline in rheumatic activity.
• AV block is seldom progressive and pacemaker insertion rarely needed.
22. TREATMENT:
• There is no treatment for ARF that has been proven to alter the likelihood of
developing, or the severity of, RHD. With the exception of treatment of heart failure,
which may be life-saving in cases of severe Carditis, the treatment of ARF is
symptomatic.
• Antibiotics:
Penicillin is the drug of choice and can be given orally [as phenoxymethyl penicillin,
500 mg (250 mg for children 27 kg) twice daily, or amoxicillin 50 mg/kg (max 1 g)
daily, for 10 days] or as a single dose of 1.2 million units (600,000 units for children 27
kg) IM benzathine penicillin G.
23. TREATMENT:
Salicylates and NSAIDs :
• These may be used for the treatment of arthritis, arthralgia, and fever, once the
diagnosis is confirmed.
• Aspirin is the drug of choice. An initial dose of 80–100 mg/kg per day in children (4–
8 g/d in adults) in 4–5 divided doses is often needed for the first few days up to 2
weeks. A lower dose should be used if symptoms of salicylate toxicity emerge, such
as nausea, vomiting, or tinnitus. When the acute symptoms are substantially resolved,
the dose can be reduced to 60–70 mg/kg per day for a further 2–4 weeks.
• Although less well studied, naproxen at a dose of 10–20 mg/kg per day has been
reported to lead to good symptomatic response