Dr.

Dr. wangdiao
wangdiao
Dept. of Pediatrics and Neonatology

The third affiliated Hospital,

zhengzhou university
E-mail: deai315@163.com
 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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 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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 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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 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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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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 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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 Rheumatic Carditis Histology (40X)

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 Histology of Myocardium in Rheumatic
Carditis (200X)

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

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

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 Clinical
Clinical Features
Features (Contd)
(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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 Clinical
Clinical Features
Features
(Contd)
(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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 Clinical
Clinical Features
Features
(Contd)
(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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 Clinical
Clinical Features
Features
(Contd)
(Contd)
Other features (Minor features)
 Fever-(upto 101 degree F)
 Arthralgia
 Pallor
 Anorexia
 Loss of weight

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 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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 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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 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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 Jones C riteria (R evised) for G uidance in th
D iagnosis of R heum atic Fever*
M ajo r M an ifestatio n M ino r S u p p o rtin g E vid ence
M an ifestatio ns o f S trep toco cal Infection
C arditis C lin ical L abo ratory
Polyarth ritis Previo u s A cu te p hase
C h orea rh eu m atic reactants: In creased Titer of An ti-
Erythem aM arg inatum fever or Erythro cyte Strep to coccal Antib od ies A SO
Sub cutan eous N od ulesrh eu m atic sedim entation (anti-
strepto lysin O ),
h eart d isease rate, o th ers
A rthralgia C -reactive Po sitive T hroat Cu ltu re
F ever p ro tein, for G ro up A Strepto coccus
leu kocytosis R ecent Scarlet F ever
Pro lon ged P-
R in terval

*The presence of two m ajor criteria, or of one m ajor and tw o m inor criteria,
indicates a high probability of acute rheum atic fever, if supported by eviden
nfection.
G roup A streptococcal

Recommendations of the American Heart Association

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 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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 Differential Diagnosis
 Juvenile rheumatiod arthritis
 Septic arthritis
 Sickle-cell arthropathy
 Kawasaki disease
 Myocarditis
 Scarlet fever
 Leukemia

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 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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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
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(maximum
Recommendations 1 g/d) Heart Association
of American 25
 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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 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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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
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Recommendations of American Heart Association
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.
2007-5-15 Recommendations of American Heart Association 29
 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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