The document discusses rheumatic fever, a disease that can occur after a streptococcal throat infection and causes inflammation in connective tissues, especially the heart. It causes symptoms like arthritis, heart issues, involuntary movements, and skin rashes. The document covers the causes, symptoms, diagnosis, treatment, and prevention of rheumatic fever through antibiotics and lifestyle changes.

1. Rheumatic Fever
Dr.B.BALAGOBI

2. Objectives
• Introduction
• Etiology
• Epidemiology
• Pathogenesis
• Pathologic lesions
• Clinical manifestations & Laboratory
findings
• Diagnosis & Differential diagnosis
• Treatment & Prevention
• Prognosis
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3. Acute Rheumatic Fever...
• A connective tissue disease
• Acquired heart disease
• Mainly in Developing countries
• Significant morbidity and mortality
• Association with pharyngitis - group A
haemolytic streptococci
• High risk of recurrence –So prophylaxis is needed
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4. Etiology
• Acute rheumatic fever is a systemic disease of
childhood,often recurrent that follows group
A beta hemolytic streptococcal infection
• It is a delayed non-suppurative sequelae to
URTI with GABH streptococci.
• It is a diffuse inflammatory disease of
connective tissue,primarily involving
heart,blood vessels,joints, subcut.tissue and
CNS
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5. Epidemiology
• Ages 5-15 yrs are most susceptible
• Rare <3 yrs
• Girls>boys
• Common in 3rd world countries
• Environmental factors-- over crowding, poor
sanitation, poverty,
• Incidence more during fall ,winter & early
spring
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6. Pathogenesis
• Delayed immune response to infection with
group.A beta hemolytic streptococci.
• After a latent period of 1-3 weeks, antibody
induced immunological damage occur to
heart valves,joints, subcutaneous tissue
& basal ganglia of brain
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7. Group A streptococcal pharyngitis
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8. Group A Beta Hemolytic Streptococcus
• Strains that produces rheumatic fever - M
types l, 3, 5, 6,18 & 24
• Pharyngitis- produced by GABHS can lead to-
acute rheumatic fever ,
rheumatic heart disease &
post strept. Glomerulonepritis
• Skin infection- produced by GABHS leads to
post streptococcal glomerulo nephritis only. It
will not result in Rh.Fever or carditis as skin
lipid cholesterol inhibit antigenicity
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9. Pathologic Lesions
• Fibrinoid degeneration of connective
tissue,inflammatory edema, inflammatory cell
infiltration & proliferation of specific cells resulting
in formation of Ashcoff nodules, resulting in-
-Pancarditis in the heart
-Arthritis in the joints
-Ashcoff nodules in the subcutaneous
tissue
-Basal gangliar lesions resulting in chorea
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10. Clinical Features
1.Arthritis
• Flitting & fleeting migratory polyarthritis,
involving major joints
• Commonly involved joints-knee,ankle,elbow &
wrist
• Occur in 80%,involved joints are exquisitely
tender
• In children below 5 yrs arthritis usually mild
but carditis more prominent
• Arthritis do not progress to chronic disease
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11. Clinical Features (Contd)
2.Carditis
• Manifest as pancarditis(endocarditis,
myocarditis and pericarditis),occur in 40-50%
of cases
• Carditis is the only manifestation of
rheumatic fever that leaves a sequelae &
permanent damage to the organ
• Valvulitis occur in acute phase
• Chronic phase- fibrosis,calcification &
stenosis of heart valves(fishmouth valves)
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12. Rheumatic
heart disease.
Abnormal
mitral valve.
Thick, fused
chordae
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13. Clinical Features (Contd)
3.Sydenham Chorea
• Occur in 5-10% of cases
• Mainly in girls of 1-15 yrs age
• May appear even 6/12 after the attack of
rheumatic fever
• Clinically manifest as-clumsiness,
deterioration of handwriting,emotional
lability or grimacing of face
• Clinical signs- pronator sign, jack in the box
sign , milking sign of hands
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14. Clinical Features (Contd)
4.Erythema Marginatum
• Occur in <5%.
• Unique,transient,serpiginous-looking
lesions of 1-2 inches in size
• Pale center with red irregular margin
• More on trunks & limbs & non-itchy
• Worsens with application of heat
• Often associated with chronic carditis
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15. Clinical Features (Contd)
5.Subcutaneous nodules
• Occur in 10%
• Painless,pea-sized,palpable nodules
• Mainly over extensor surfaces of
joints,spine,scapulae & scalp
• Associated with strong seropositivity
• Always associated with severe carditis
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16. Clinical Features (Contd)
Other features (Minor features)
• Fever-(upto 101 degree F)
• Arthralgia
• Pallor
• Anorexia
• Loss of weight
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17. Laboratory Findings
• High ESR
• Anemia, leucocytosis
• Elevated C-reactive protien
• ASO titre >200 Todd units.
(Peak value attained at 3 weeks,then
comes down to normal by 6 weeks)
• Anti-DNAse B test
• Throat culture-GABHstreptococci
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18. Laboratory Findings (Contd)
• ECG-
– prolonged PR interval, 2nd or 3rd degree
blocks,ST depression, T inversion
• 2D Echo cardiography
– valve edema,mitral regurgitation, LA & LV
dilatation,pericardial effusion,decreased
contractility
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19. Diagnosis
• Rheumatic fever is mainly a clinical diagnosis
• No single diagnostic sign or specific laboratory
test available for diagnosis
• Diagnosis based on MODIFIED JONES
CRITERIA
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20. Guidelines for diagnosis of the initial attack of
rheumatic fever. Duckett Jones criteria, 1992 update -
American Heart Association
• 2 major manifestations
or
• 1 major and 2 minor manifestations
• supported by
– Evidence of antecedent streptococcal
infection
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21. Major manifestations...
• Polyarthritis
• Carditis
• Chorea
• Subcutaneous nodules
• Erythema marginatum
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22. Minor manifestations...
• Clinical
– Arthralgia
– Fever
• Laboratory
– Elevated acute-phasereactants (ESR,CRP)
– Prolonged PR interval
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23. Exceptions to Jones Criteria
 Chorea alone, if other causes have been
excluded
 Insidious or late-onset carditis with no other
explanation
 Patients with documented RHD or prior
rheumatic fever,one major criterion,or of
fever,arthralgia or high CRP suggests
recurrence
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24. Differential Diagnosis
• Juvenile rheumatiod arthritis
• SLE
• Septic arthritis
• Sickle-cell arthropathy
• Kawasaki disease
• Myocarditis
• Scarlet fever
• Leukemia
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25. Management...
Average course of 6-8 weeks
• Admit - confirmation, education, drugs
• Investigations
• Bed rest - CCF - strict bed rest
• Antibiotics - oral penicillin for 10 days or IM
Benzathine penicillin
• Anti rheumatic drugs - aspirin / steroids
• Aspirin - dose/administration/side effects
• Duration: RF: ~ 6 weeks and tail off over ~ 2wks
RC: 8 -10 weeks and tail off over ~ 2 wks
• Steroids - no effect on long term prognosis
CCF / impending heart failure
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26. Treatment
• Step I - primary prevention
(eradication of streptococci)
• Step II - anti inflammatory treatment
(aspirin,steroids)
• Step III- supportive management &
management of complications
• Step IV- secondary prevention
(prevention of recurrent attacks)
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27. STEP I: Primary Prevention of Rheumatic Fever (Treatment
of Streptococcal Tonsillopharyngitis)
Agent Dose Mode Duration
Benzathine penicillin G 600 000 U for patients Intramuscular Once
27 kg (60 lb)
1 200 000 U for patients >27 kg
or
Penicillin V Children: 250 mg 2-3 times daily Oral 10 d
(phenoxymethyl penicillin) Adolescents and adults:
500 mg 2-3 times daily
For individuals allergic to penicillin
Erythromycin: 20-40 mg/kg/d 2-4 times daily Oral 10 d
Estolate (maximum 1 g/d)
or
Ethylsuccinate 40 mg/kg/d 2-4 times daily Oral 10 d
(maximum 1 g/d)
Recommendations of American Heart Association
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28. Step II: Anti inflammatory treatment
Clinical condition Drugs
Arthritis only Aspirin 75-100
mg/kg/day,give as 4
divided doses for 6
weeks
(Attain a blood level 20-
30 mg/dl)
Carditis Prednisolone 2-2.5
mg/kg/day, give as two
divided doses for 2
weeks
Taper over 2 weeks &
while tapering add
Aspirin 75 mg/kg/day
for 2 weeks.
Continue aspirin alone
100 mg/kg/day for
another 4 weeks
28
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29. 3.Step III: Supportive management &
management of complications
• Bed rest
• Treatment of congestive cardiac failure:
-digitalis,diuretics
• Treatment of chorea:
-diazepam or haloperidol
• Rest to joints & supportive splinting
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30. Why prophylaxis..?
• To prevent streptococcal infections which
precipitate recurrences of rheumatic fever
• Prevent development of chronic rheumatic heart
disease
• If recurrences are prevented, 70% of patients with
carditis in the initial attack will eventually have
normal hearts
• No documented evidence of resistance of group A
streptococci to penicillin
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31. Prophylaxis...
• Primary -
Adequate treatment of streptococcal sore
throats - oral penicillin for 10 days
Clinical differentiation of viral/bacterial
sore throats is difficult
Throat swab for culture and ABST
Erythromycin
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32. Prophylaxis ctd...
• Secondary -
• Benzathine penicillin 1.2 mega units IM ( ARF - 4
weekly/RC - 3 weekly )
• Duration - ARF - 18 / 21yrs or 5yrs after last attack
• Carditis - (extent of damage) ~ 25
• Chronic valvular heart disease - life long
• Infective endocarditis prophylaxis - life long
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33. Prognosis
• Rheumatic fever can recur whenever the
individual experience new GABH streptococcal
infection,if not on prophylactic medicines
• Good prognosis for older age group & if no
carditis during the initial attack
• Bad prognosis for younger children & those
with carditis with valvar lesions
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34. T/F In Rheumatic fever?
A. is causing deformity in joints
B. small joints of the hands are commonly
affected
C. Anti streptolysin O is elevated
D. Aspirin treatment prevents the cardiac
involvement
E. Sleeping pulse rate is elevated in Carditis
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35. T/F Features of rhematic carditis?
A. Pericardial rub
B. Congestive heart failure
C. Coronary artery aneurysm
D. Mid diastolic murmur
E. tachycardia
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36. T/F which of the following are the
minor criteria of Rheumatic fever?
A. sub cutaneous nodule
B. Arthritis
C. Elevated ASOT
D. Raised ESR
E. Fever
F. Prolonged PR interval in ECG
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37. T/F regarding Rheumatic fever?
A. Chorea is associated with subcutaneous
nodule
B. Prolong PR interval in ECG indicates the
underlying carditis
C. Erythema nodosum is a major criteria
D. IM Benzathine penicillin given 3 weekly if
carditis is present
E. Mitral stenosis is common at the acute stage
of the disease
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38. T/F regarding Rheumatic fever?
A. Steroids are superior to salicylates in prevention
of carditis
B. Subcutaneous nodules are associated with bad
prognosis
C. History of sore throat is essential for the
diagnosis
D. Can Cause early diastolic murmur at left lower
sternal edge
E. Can cause cardiomegaly
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39. T/F regarding Rheumatic fever?
A. In patient with Rheumatic valvular heart disease
antibiotic prophylaxis monthly given to up to 21
years of age
B. In patient with Rheumatic valvular heart disease
antibiotic prophylaxis monthly given to prevent
infective endocarditis
C. Emotional lability is a feature of Chorea
D. Aortic valve involvement is commoner than
mitral valve involvement.
E. New onset Pansystolic murmur is a feature.
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40. T/F Rheumatic fever?
A. Low dose aspirin is used in the treatment
B. Common in children than adults
C. Cause erosive arthritis
D. Seen in 15% of children with phayrngitis
E. There are no recurrence
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41. T/F Rheumatic fever?
A. Associated with β haemolytic streptococci
B. Can not be diagnosed if normal ASOT
C. Chorea is a late feature
D. Commonly affects the endocardium of the
heart
E. Chorea is common in boys
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42. T/F regarding Acute Rheumatic fever?
• Salicylates or steroids should not be started until
diagnosis is confirmed
• Antibiotic therapy during acute infection can alter
the severity of cardiac involvement
• Compared to salicylates ;steroids use
significantly reduce rheumatic valvular disease
• Prophylaxis with Oral penicillin /IM benzathine
penicillin are equally effective
• Effective serum concentration of drug detected
up to 4 wks after IM Benzathine penicillin
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