1. Acute rheumatic fever is a systemic disease that follows group A streptococcal infection and causes inflammation of connective tissues, especially the heart. 2. It most commonly affects children ages 5-15 and is more prevalent in developing countries. The symptoms include migratory arthritis, carditis, chorea, and subcutaneous nodules. 3. Diagnosis is based on the modified Jones criteria of either two major symptoms or one major and two minor symptoms along with evidence of a prior streptococcal infection. Treatment involves antibiotics to treat strep, anti-inflammatories, and long-term antibiotic prophylaxis to prevent recurrence.

1. Acute
Rheumatic Fever
Yani Dewi Suryani

2. 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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3. 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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4. Pathogenesis
 Hypersensitivity 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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5. Pathogenesis
 During strep infection, antigen presenting cells
present bacterial antigen to helper T cells.
 These helper T cells then activate B cells to
induce production of antibodies against the
Streptococcal cell wall.
 These antibodies can also interact with other cells
in the body (for example, myocardium or joints,
etc) producing the symptoms responsible with
acute rheumatic fever
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6. Group A Beta Hemolytic Streptococcus
 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 glomerulonephritis only. It will not
result in Rh.Fever or carditis
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7. Clinical Features
 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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1. Arthritis

8. 8

9. Clinical Features (Contd)
 Manifest as pancarditis (endocarditis, myocarditis
and pericarditis), occur in 40-50% of cases
 Cardiomegaly, congestive heart failure
 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
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2. Carditis

10. Clinical Features (Contd)
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 Occur in 5-10% of cases
 Mainly in girls of 1-15 yrs age
 May appear even 6 months after the attack of
rheumatic fever
 Clinically manifest as clumsiness, deterioration
of handwriting, emotional lability or grimacing
of face
3. Sydenham Chorea

11. 11

12. Clinical Features (Contd)
 Occur in <5%
 Unique, transient 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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4. Erythema Marginatum

13. 13

14. Clinical Features (Contd)
 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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5. Subcutaneous nodules

15. 15

16. 16

17. Clinical Features (Contd)
 Fever – Low grade
 Arthralgia
 Pallor
 Anorexia
 Loss of weight
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Other features (Minor features)

18. Laboratory Findings
 High ESR
 Anemia, leucocytosis
 Elevated C-reactive protien
 ASO titre >200
(Peak value attained at 3 weeks, then comes down
to normal by 6 weeks)
 Anti-DNAse B test
 Throat culture-GABH streptococci
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19. Laboratory Findings (Contd)
ECG- prolonged PR interval
Echo - valve edema,mitral regurgitation, LA & LV
dilatation, pericardial effusion, decreased
contractility
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20. 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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21. DIAGNOSIS
JONES CRITERIA
◦ Need 2 major criteria or 1 major and 2 minor
criteria in the presence of a prior strep infection to
make the diagnosis
◦ Evidence of prior strep infection with positive
throat culture or antigen test, elevated
streptococcal antibody titer, or history of
rheumatic fever/heart disease

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Major
Manifestation
Minor
Manifestations
Supporting Evidence
of Streptococal
Infection
Carditis
Polyarthritis
Chorea
Erythema
Marginatum
Subcutaneous
Nodules
Clinical Laboratory
Increased Titer of
Anti-Streptococcal
Antibodies ASO
(anti-streptolysin O),
others
Positive Throat
Culture for Group A
Streptococcus
Recent Scarlet Fever
Previous
rheumatic
fever or
rheumatic
heart disease
Arthralgia
Fever
Acute phase
reactants >>
Erythrocyte
sedimentation
rate >>
C-reactive
protein >>
Leukocytosis
Prolonged P-R
interval
Jones Criteria (Revised) for Guidance in the
Diagnosis of Rheumatic Fever*

23. Diagnostic criteria
 The presence of two major criteria
or
 one of major and two minor criteria, indicates a
high probability of acute rheumatic fever,
 and supported by evidence of Group A
streptococcal infection
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24. 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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25. 25
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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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 off 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

27.  Bed rest
 Treatment of congestive cardiac failure:
- digitalis, diuretics
 Treatment of chorea:
- diazepam or haloperidol
 Rest to joints & supportive splinting
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Step III: Supportive management &
management of complications

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STEP IV : Secondary Prevention of Rheumatic Fever
(Prevention of Recurrent Attacks)
Agent Dose Mode
Benzathine penicillin G 1 200 000 U every 4 weeks* Intramuscular
or
Penicillin V 250 mg twice daily Oral
or
Sulfadiazine 0.5 g once daily for patients 27 kg (60 lb Oral
1.0 g once daily for patients >27 kg (60 lb)
For individuals allergic to penicillin and sulfadiazine
Erythromycin 250 mg twice daily Oral
*In high-risk situations, administration every 3 weeks is justified and
recommended

29. PROPHYLAXIS
Prophylaxis needed to prevent recurrence of Acute
Rheumatic Fever
Start prophylaxis after acute episode resolves
Can use:
 Penicillin V 250mg BID or,
 Sulfadiazine 1000mg daily, or
 Penicillin G 1.2 million units IM q4weeks
 For PCN allergic patients: erythromycin 250mg PO
BID
Recurrence of disease generally occurs in the first
couple years

30. PROPHYLAXIS
WHO GUIDELINES
◦ At least 5 years of prophylaxis or if child until
age 18 if not cardiac involvement
◦ 10 years prophylaxis or if child until age 25 if has
mild mitral regurgitation
◦ Lifelong prophylaxis if has severe valve disease

31. Complications
Mitral valve
stenosis

32. Complications

33. Complications

34. Prognosis
 Rheumatic fever can recure 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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