Pediatrics

2. 0 Acute rheumatic fever is a systemic disease of child-hood,
often recurrent that follows group A beta hemolytic
streptococcal infection. Autoimmune consequence of
infection (pharyngeal infection not the skin infection)
0 It is characterized by inflammatory lesions of connective
and endothelial tissue
0 Generalized inflammatory response affecting brain,
joints, skin, subcutaneous tissues, blood vessels & the
heart.

3. 0 The etiology of rheumatic fever is not clear, but there
is strong association with Beta hemolytic streptococci
sore throat.

4. ACUTE RHEUMATIC FEVER
Redness & swelling
of throat & tonsils;
Beefy, swollen, red
uvula; Soft palate
petechiae
(“doughnut
lesions”)
Tonsillopharyngeal
erythema &
exudatesSore throat: fever,
white draining
patches on the
throat & swollen or
tender lymph glands
in the neck

5. Epidemiology/Predisposing
factors
Family history of rheumatic fever
Low socioeconomic status (poverty, poor hygiene, medical
deprivation, poor dietary intake)
Age: 5-15 years
0 Rare <3 yrs
0 Girls>boys
0 Common in 3rd world countries
 At a rate of 5/ 1000 incidence
Incidence more during fall ,winter & early spring

6. 6
0Delayed immune response to infection with- Group A
beta hemolytic streptococci.
0It is characterized by inflammatory lesions of
connective tissue and endothelial tissue, primarily
affecting the joints and heart
0After a latent period of 1-5 weeks( Average 3 weeks),
antibody induced immunological damage occur to
heart valves, joints, subcutaneous tissue & basal
ganglia of brain

7. Contd..
0 Autoantibodies attack the myocardium, pericardium and
cardiac valves
ASCHOFF’S BODIES( fibrin deposits) develop on valves,
possibly leading to permanent valve dysfunction, especially of
the mitral and aortic valves.
Severe myocarditis may cause dilation of the heart and CHF
0 Inflammation of large joints causes a painful arthritis that
may last 6-8 weeks
0 Involvement of nervous system causes chorea(sudden
involuntary movement

8. 8
Diagrammatic structure of the group A beta hemolytic
streptococcus
Capsule
Cell wall
Protein antigens
Group carbohydrate
Peptidoglycan
Cyto.membrane
Cytoplasm
…………………………………………………...
Antigen of outer
protein cell wall
of GABHS
induces antibody
response in
victim which
result in
autoimmune
damage to heart
valves,
subcutaneous
tissue, tendons,
joints & basal
ganglia of brain

9. “Rheumatism licks the joints
but bites the whole heart” -William
Boyd

10. 10
0Fibrinoid degeneration of connective tissue,
inflammatory edema, inflammatory cell infiltration &
proliferation of specific cells resulting in formation
of Ashcoff nodules, resulting in-
- in the heart
in the joints
-Ashcoff nodules in the subcutaneous tissue
- Basal ganglia lesions resulting in chorea (
involuntary movements)

11. 11
Rheumatic heart
disease.
Abnormal mitral
valve. Thick,
fused chordae

12. 12
Another view of
thick and fused
mitral valves in
Rheumatic
heart disease

13. 13
(Contd)
0 Occur in 5-10% of cases
0 Mainly in girls of 1-15 yrs age
0 May appear even 6/12 after the attack of rheumatic fever
0 Clinically manifests as-clumsiness, deterioration of hand-
writing, emotional lability or grimacing of face
0 Clinical signs- pronator sign, milking sign of hands

15. 15
(Contd)
0 Occur in <5%.
0 Unique, transient, serpiginous-looking lesions of 1-2
inches in size
0 Pale center with red irregular margin
0 More on trunks & limbs & non-itchy
0 Worsens with application of heat
0 Often associated with chronic carditis

17. 17
(Contd)
0 Occur in 10%
0 Painless, pea-sized, palpable nodules
0 Mainly over extensor surfaces of joints, spine, scapulae
& scalp
0 Associated with strong sero-positivity
0 Always associated with severe carditis

19. 05/05/1999 Dr.Said Alavi 19
(Contd)
0 Fever-(upto 101 degree F)
0 Arthralgia
0 Previous attacks of rheumatic fever or RHD
0 ECG- Prolonged P-R Interval
0 Elevated ESR or C- reactive protein

20. Clinical Features contd
0Elevated ASO titre-Antistreptolysin O –titre
indicates previous streptococcal infection(
normal IU/ml)
0Positive throat swab culture may show
streptococcal infection.

21. Other Manifestations
0 Precordial pain
0 Abdominal pain
0 Headache
0 Easy fatigability
0 General weakness
0 Tachycardia
0 Malaise
0 Sweating
0 Vomiting
0 Skin rash
0 Erythema nodosum
0 Epistaxis
0 Anemia
0 Pleuritis
0 Weight loss

23. 0 2 major criteria or 1 major & 2 minor criteria &
the absolute requirement
0 High ESR
0 Anemia, leuco-cytosis
0 Elevated C-reactive protein
0 ASO titre >200 IU.(Peak value attained at 3 weeks, then
comes down to normal by 6 weeks)
0 Throat culture-GABH streptococci
0 CXR- shows cardiomegaly and heart-failure

24. 24
0 ECG- prolonged PR interval, 2nd or 3rd degree blocks,
ST depression, T inversion
0 2D Echo cardiography- valve edema, mitral
regurgitation, LA & LV dilatation, pericardial effusion,
decreased contractility

25. 25
0 Rheumatic fever is mainly a clinical diagnosis
0 No single diagnostic sign or specific laboratory test
available for diagnosis
0 Diagnosis based on MODIFIED JONES
CRITERIA

26. 05/05/1999 Dr.Said Alavi 26
Jones Criteria (Revised) for Guidance in the
Diagnosis of Rheumatic Fever*
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
*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

27. 27
0 Step I - primary prevention (eradication of streptococci)
0 Step II - anti inflammatory treatment (aspirin, steroids)
0 Step III- supportive management & management of
complications
0 Step IV- secondary prevention (prevention of recurrent attacks)

28. 05/05/1999 Dr.Said Alavi 28
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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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
Step II: Anti inflammatory treatment
Clinical condition Drugs

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0 Bed rest
0 Treatment of congestive cardiac failure: -digitalis,
diuretics
0 Treatment of chorea -diazepam or haloperidol
0 Rest to joints & supportive splinting
3.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
Recommendations of American Heart Association

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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

33. , penicillin is administered after
skin test to eradicate streptococcal infection.
0 Initially procaine penicillin 4 lakh units Deep IM twice a
day is given for 10-14 days.
0 Long acting Benzathine penicillin 1.2 mega units every 21
days or 6 mega unit every 15 days to be given.
0 Oral penicillin 4lakh units(250mg), q4-6hours for 10-14
days can be also given
0 Erythromycin can be used in penicillin sensitive patients.

34. is administered as suppressive therapy to
control pain and inflammation of joints.
0 The dose of aspirin is 90-120mg/kg/day in 4 divided
dose for 12 weeks.
0 Antacid to be given just prior to or with the aspirin.

35. 0 Steroid – prednisolone therapy is given as suppressive
therapy along with aspririn.
0 The initial dose is 40-60mg/kg/day in 4 divided
doses, for 7-10 days
0 Then the dose is reduced, for 7-10days
0 Then the dose is reduced to 1mg/kg/day
0 It must be tapered off gradually over 12weeks period
and used for patients having carditis with or without
CCF

36. 0 Management of chorea can be done with diazepam or
phenobarbitone
0 Symptomatic care
0 Emotional support to the child and parents.

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