Rheumatic Fever in

Children
Edward Surjono, M.D.
Pediatric Department
Atma Jaya Faculty of Medicine
General Objective

To discuss about Rheumatic Fever in Children

Specific Objectives
To know the incidence and prevalence of Rheumatic
Fever
To understand the patophysiology of Rheumatic
Fever
To understand how to make a diagnosis of
Rheumatic Fever
To understand how Rheumatic Fever treatment and
prevented
Rheumatic Fever
Diffuse inflammation of connective tissues of
heart, joints, brain, blood vessels, and
subcutaneous tissues
Relationship with Group A beta-hemolytic
Streptococcus established
Rheumatic Fever: Tonsillitis
PREVALENCE RATE
1-10/1000 school children

INCIDENCE RATE
10-20/100,000 population with a high rate of recurrence attack and
severity

WHO estimates
12 M cases annually
400,000 deaths annually
Rheumatic Fever remains to
be the most common
Acquired Heart Disease
in developing countries
 Philippine Foundation for Prev & Control of RF/RHD
Distribution of Cases (1995-1999)
Age Distribution ( n = 2085 )

800 745
700 652
600 < 6 years
500 465
6 - 10 years
400 11 - 15 years
300 16 -18 years
200 > 18 years
98 125
100
0
 Philippine Foundation for Prev & Control of RF/RHD
Cardiac Involvement by Age Distribution at
National Capital Region (1999)

35.0%
30.9%
30.0%
25.0% < 6 years
20.6%
20.0% 6 - 10 years
11 - 15 years
15.0% 16 -18 years
10.2%
8.8% > 18 years
10.0% 8.4%
5.0%
0.0%
Pediatric Cardiac Physical Examination
Natural History of Rheumatic Fever and Points
at which Control Program can Act:
Group A Beta
Hemolytic Streptococcus
PRIMARY PREVENTION
PRIMARY
PREVENTION
SECONDARY
Susceptible Person PREVENTION
ACUTE RHEUMATIC
10-14 days
FEVER
Streptococcal Infection R
of the Respiratory Tract E
2-3 MOS
L
WITHOUT WITH A
CARDITIS CARDITIS P
S
E
CURE WITHOUT RHD RHD
PRIMARY PROPHYLAXIS
prompt Dx and Tx of Streptococcal sore
throat to prevent INITIAL ATTACK OF
ARF
SECONDARY PROPHYLAXIS
antibiotic Tx to prevent RECURRENCES
among patients previously diagnosed as
RF/RHD
 Jones Criteria UPDATE for Guidance in the
Diagnosis of Rheumatic Fever * 1992
Major Manifestation Minor Manifestation Supporting Evidence of
Streptococcal Infection
Clinical Laboratory

CARDITIS ^ UPDATE Acute phase UPDATE

reactants ; Positive Throat culture or
POLYARTHRITIS Arthralgia Erythrocyte Rapid Strep Antigen Test
Fever sedimentation Elevated or rising Strep
CHOREA ^ rate ; Antibody titer of at least two
C reactive fold from baseline
ERYTHEMA protein ;
MARGINATUM ^ prolonged PR
interval
SUBCUTANEOUS
NODULE ^

* 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 infection
PENICILLIN

Drug of Choice
GUYS . . .
Are you still with me ???
 PRIMARY PROPHYLAXIS
AGENT DOSE MODE DURATION EVIDENCE
Benzathine 600,000 U for px < 27 kg IM Once II A
Penicillin G ( 60 lbs.)
1,200,000 U for px >27 kg.
Penicillin V Children: 250 mg 2-3 x daily Oral 10 days IIA
(Phenoxymethyl Adolescents and adults :
Penicillin) 500mg 2-3 times daily

For individuals
allergic to Penicillin

Erythromycin 20-40 mg/kg/day Oral 10 days IIA

Estolate 2- 4 times daily ( max 1g /d)
Ethylsuccinate 40 mg/kg/d Oral 10 days IIA
2- 4 times daily ( max 1g /d)
Azithromycin 500 mg on first day Oral 5 days IIA
250 mg/d for the next 4 d
Secondary Prophylaxis of Rheumatic Fever
Agent Dose Mode Evidence
Benzathine 1,200,000 U every 4 Intramuscular IIA
Penicillin G wks (every 3 wks for
high-risk* pxs such
as those with
residual carditis)
Penicillin V 250 mg twice daily Oral IIA

For individuals
allergic
to penicillin and
sulfadiazine 250 mg twice daily Oral IIA
Erythromycin
 Suggested Schedules of Anti-inflammatory
Therapy
Clinical Severity Treatment

Arthritis; no carditis, or Aspirin at 90-100mg/kg/day

carditis without cardiomegaly, for 2 weeks; longer if necessary
but without failure at 60-70 mg/kg/day

Carditis with failure; with or Prednisone 1-2mg/kg/day, max

without joint manifestation 60 mg/day; after 2-3 weeks
taper to complete in 3 or more
weeks
Aspirin to be continued for 4
weeks after discontinuation of
Prednisone
Prophylaxis Duration
Category Duration
Rheumatic Fever without Carditis 5 year until 21 year of age, whichever is
longer
Rheumatic fever with carditis but without 10 year or well into adulthood, whichever is
residual heart disease (no valvular disease*) longer

Rheumatic fever with carditis and residual
heart disease (persistent valvular disease*)
* Clinical or echocardiographic
evidence
At least 10 year since last episode and at least
until 40 year of age; sometimes lifelong
prophylaxis
AAP, Redbook 2006
Class of Recommendation

Class I: Definitely recommended

Class II: Acceptable and useful
IIa: Good to very good evidence
IIb: Fair to good evidence
Class III: Not acceptable, may be harmful
Indeterminate: Preliminary evidence needs
confirmation; no harm
Class of recommendation reflects quality of
evidence and not clinical preference
carditis
Rheumatic Carditis
Chest radiograph of an 8 year old patient with acute carditis
before treatment
Rheumatic Carditis
Same patient after 4 weeks
Rheumatic Carditis
Aortic valve showing active valvulitis. The valve is slightly thickened
and displays small vegetations "verrucae
Rheumatic Carditis
Myocardial Aschoff body the cells are large, elongated, with large
nuclei; some are multinucleate
^
CHOREA ^
 Erythema
marginatum
Erythema Marginatum ^
Subcutaneous nodules
Rheumatic Fever:
Subcutaneous Nodules^
Small, firm, painless & transient
Extensor surface of elbows, knees, & wrists
Scalp & spinal areas
Often seen with carditis