Dr.

Said Alavi
MD,DCH,DNB,FCPS
MD,DCH,DNB,FCPS
Dept. of Pediatrics and Neonatology
Saqr Hospital,Ras Al Khaimah
UNITED ARAB EMIRATES

E-mail: drsaid@emirates.net.ae
 Objectives
Objectives

Etiology

Epidemiology

Pathogenesis

Pathologic lesions

Clinical manifestations & Laboratory
findings

Diagnosis & Differential diagnosis

Treatment & Prevention

Prognosis

References
05/05/1999 2 Dr.Said Alavi
 Etiology
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
05/05/1999 3 Dr.Said Alavi
 Epidemiology
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

05/05/1999 4 Dr.Said Alavi

 Pathogenesis
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

05/05/1999 5 Dr.Said Alavi

 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
05/05/1999 6 Dr.Said Alavi
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
Cyto.membrane
valves,
sub cutaneous
tissue,tendons,
Cytoplasm joints & basal
ganglia of brain
……………………………………………
……...
05/05/1999 7 Dr.Said Alavi
 Pathologic
Pathologic Lesions
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
05/05/1999 8 Dr.Said Alavi
 Rheumatic
Rheumatic Carditis
Carditis Histology
Histology (40X)
(40X)

05/05/1999 9 Dr.Said Alavi

 Histology
Histologyof
ofMyocardium
Myocardium in inRheumatic
RheumaticCarditis
Carditis
((200X)
200X)

05/05/1999 10 Dr.Said Alavi

 Clinical
Clinical Features
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
05/05/1999 11 Dr.Said Alavi
 Clinical
Clinical Features
Features (Contd)
(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)
05/05/1999 12 Dr.Said Alavi
 Rheumatic
heart
disease.
Abnormal
mitral
valve.
Thick,
fused
chordae

05/05/1999 13 Dr.Said Alavi

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

05/05/1999 14 Dr.Said Alavi

 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
05/05/1999 15 Dr.Said Alavi
 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

05/05/1999 16 Dr.Said Alavi

 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

05/05/1999 17 Dr.Said Alavi

 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
Laboratory Findings
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

05/05/1999 19 Dr.Said Alavi

 Laboratory
Laboratory Findings
Findings (Contd)
(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

05/05/1999 20 Dr.Said Alavi

 Diagnosis
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

05/05/1999 21 Dr.Said Alavi

 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

05/05/1999 22 Dr.Said Alavi
 Exceptions
Exceptions to
to Jones
Jones Criteria
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

05/05/1999 23 Dr.Said Alavi

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

05/05/1999 24 Dr.Said Alavi

 Treatment
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)
05/05/1999 25 Dr.Said Alavi
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
Recommendations 1 g/d) Heart Association
of American
05/05/1999 26 Dr.Said Alavi
 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

05/05/1999 27 Dr.Said Alavi

 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

05/05/1999 28 Dr.Said Alavi

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
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
05/05/1999 30 Dr.Said Alavi
 Prognosis
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

05/05/1999 31 Dr.Said Alavi

 References
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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