1. Dr.Said Alavi
MD,DCH,DNB,FCPS
Dept. of Pediatrics and Neonatology
Saqr Hospital,Ras Al Khaimah
UNITED ARAB EMIRATES
E-mail: drsaid@emirates.net.ae

2. Objectives
q Etiology
q Epidemiology
q Pathogenesis
q Pathologic lesions
q Clinical manifestations & Laboratory
findings
q Diagnosis & Differential diagnosis
q Treatment & Prevention
q Prognosis
q References
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3. Etiology
q Acute rheumatic fever is a systemic
disease of childhood,often recurrent that
follows group A beta hemolytic
streptococcal infection
q It is a delayed non-suppurative sequelae
to URTI with GABH streptococci.
q It is a diffuse inflammatory disease of
connective tissue,primarily involving
heart,blood vessels,joints, subcut.tissue
and CNS
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4. Epidemiology
q Ages 5-15 yrs are most susceptible
q Rare <3 yrs
q Girls>boys
q Common in 3rd world countries
q
Environmental factors-- over crowding,
poor sanitation, poverty,
q Incidence more during fall ,winter &
early spring
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5. Pathogenesis
q Delayed immune response to infection
with group.A beta hemolytic
streptococci.
q 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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6. Group A Beta Hemolytic Streptococcus
q Strains that produces rheumatic fever -
M types l, 3, 5, 6,18 & 24
q Pharyngitis- produced by GABHS can
lead to- acute rheumatic fever ,
rheumatic heart disease &
post strept. Glomerulonepritis
q 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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7. Diagrammatic structure of the group A
beta hemolytic streptococcus
Capsule Antigen of outer
protein cell wall
Cell wall of GABHS
induces antibody
Protein antigens response in
victim which
Group carbohydrate result in
autoimmune
Peptidoglycan damage to heart
valves,
Cyto.membrane
sub cutaneous
tissue,tendons,
Cytoplasm joints & basal
ganglia of brain
……………………………………………
……...
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8. Pathologic Lesions
q 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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9. Rheumatic Carditis Histology (40X)
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10. Histology of Myocardium in Rheumatic Carditis
(200X)
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11. Clinical Features
1.Arthritis
q Flitting & fleeting migratory polyarthritis,
involving major joints
q Commonly involved joints-
knee,ankle,elbow & wrist
q Occur in 80%,involved joints are
exquisitely tender
q In children below 5 yrs arthritis usually
mild but carditis more prominent
q Arthritis do not progress to chronic
disease
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12. Clinical Features (Contd)
2.Carditis
q
Manifest as pancarditis(endocarditis,
myocarditis and pericarditis),occur in
40-50% of cases
q Carditis is the only manifestation of
rheumatic fever that leaves a sequelae &
permanent damage to the organ
q Valvulitis occur in acute phase
q Chronic phase- fibrosis,calcification &
stenosis of heart valves(fishmouth
valves)
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13. Rheumatic
heart
disease.
Abnormal
mitral
valve.
Thick,
fused
chordae
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14. Another view of
thick and fused
mitral valves in
Rheumatic
heart disease
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15. Clinical Features (Contd)
3.Sydenham Chorea
q Occur in 5-10% of cases
q Mainly in girls of 1-15 yrs age
q May appear even 6/12 after the attack of
rheumatic fever
q Clinically manifest as-clumsiness,
deterioration of handwriting,emotional
lability or grimacing of face
q Clinical signs- pronator sign, jack in the
box sign , milking sign of hands
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16. Clinical Features (Contd)
4.Erythema Marginatum
q Occur in <5%.
q Unique,transient,serpiginous-looking
lesions of 1-2 inches in size
q Pale center with red irregular margin
q More on trunks & limbs & non-itchy
q Worsens with application of heat
q Often associated with chronic carditis
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17. Clinical Features (Contd)
5.Subcutaneous nodules
q Occur in 10%
q Painless,pea-sized,palpable nodules
q Mainly over extensor surfaces of
joints,spine,scapulae & scalp
q Associated with strong seropositivity
q Always associated with severe carditis
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18. Clinical Features (Contd)
Other features (Minor features)
q Fever-(upto 101 degree F)
q Arthralgia
q Pallor
q Anorexia
q Loss of weight
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19. Laboratory Findings
q High ESR
q Anemia, leucocytosis
q Elevated C-reactive protien
q ASO titre >200 Todd units.
(Peak value attained at 3 weeks,then
comes down to normal by 6 weeks)
q Anti-DNAse B test
q Throat culture-GABHstreptococci
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20. Laboratory Findings (Contd)
q ECG- prolonged PR interval, 2nd or 3rd
degree blocks,ST depression,
T inversion
q 2D Echo cardiography- valve
edema,mitral regurgitation, LA & LV
dilatation,pericardial effusion,decreased
contractility
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21. Diagnosis
q Rheumatic fever is mainly a clinical
diagnosis
q No single diagnostic sign or specific
laboratory test available for diagnosis
q
Diagnosis based on MODIFIED
JONES CRITERIA
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22. Jones Criteria (Revised) for Guidance in the
Diagnosis of Rheumatic Fever*
Major Manifestation Minor Supporting Evidence
Manifestations of Streptococal Infection
Carditis Clinical Laboratory
Polyarthritis Previous Acute phase
Chorea rheumatic reactants: Increased Titer of Anti-
Erythema Marginatum fever or Erythrocyte Streptococcal Antibodies ASO
Subcutaneous Nodules rheumatic sedimentation (anti-streptolysin O),
heart disease rate, others
Arthralgia C-reactive Positive Throat Culture
Fever protein, for Group A Streptococcus
leukocytosis Recent Scarlet Fever
Prolonged P-
R interval
*The presence of two major criteria, or of one major and two minor criteria,
indicates a high probability of acute rheumatic fever, if supported by evidence of
Group A streptococcal nfection.
Recommendations of the American Heart Association
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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
q Juvenile rheumatiod arthritis
q Septic arthritis
q Sickle-cell arthropathy
q Kawasaki disease
q Myocarditis
q Scarlet fever
q Leukemia
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25. Treatment
q Step I - primary prevention
(eradication of streptococci)
q Step II - anti inflammatory treatment
(aspirin,steroids)
q Step III- supportive management &
management of complications
q Step IV- secondary prevention
(prevention of recurrent attacks)
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26. 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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27. 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
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28. 3.Step III: Supportive management &
management of complications
q Bed rest
q Treatment of congestive cardiac failure:
-digitalis,diuretics
q Treatment of chorea:
-diazepam or haloperidol
q Rest to joints & supportive splinting
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29. 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
05/05/1999
Recommendations of 29
American Heart Association Dr.Said Alavi

30. Duration of Secondary Rheumatic Fever
Prophylaxis
Category Duration
Rheumatic fever with carditis and At least 10 y since last
residual heart disease episode and at least
until (persistent valvar disease*) age 40 y, sometimes lifelong
prophylaxis
Rheumatic fever with carditis 10 y or well into adulthood,
but no residual heart disease whichever is longer
(no valvar disease*)
Rheumatic fever without carditis 5 y or until age 21 y,
whichever is longer
*Clinical or echocardiographic evidence.
Recommendations of American Heart Association
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31. Prognosis
q Rheumatic fever can recur whenever
the individual experience new GABH
streptococcal infection,if not on
prophylactic medicines
q Good prognosis for older age group & if
no carditis during the initial attack
q Bad prognosis for younger children &
those with carditis with valvar lesions
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32. References
Hoffman JIE: Rheumatic Fever . Rudolph's Pediatrics; 20th Ed:
1518 - 1521,1996.
Stollerman GH: Rheumatic Fever . Harrison's Principles Of Internal
Medicine; 13th Ed: 1046 - 1052,1995.
Special Writing Group of the Committee on Rheumatic
Fever,endocarditis & Kawasaki Disease of the Council on
Cardiovascular Disease in the Young of the American Heart
Association: Guidelines for the Diagnosis of Rheumatic Fever. In
Jones Criteria, 1992 Update JAMA 268:2029,1992
Todd J: Rheumatic Fever . Nelson's Textbook Of Pediatrics; 15th
Ed: 754 - 760, 1996.
Warren R, Perez M, Wilking A: Pediatric Rheumatic Diseases .
Pediatric Clinics of North America; 41: 783 - 818,1994.
WorldHealth Organization Study Group: Rheumatic Fever &
Rheumatic Heart Disease,technical Report Series No.
764.Geneva,world Health Organization, 1988
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