This document discusses acute rheumatic fever in children. It begins by noting that rheumatic heart disease is the most common acquired heart disease in children worldwide, especially in developing countries. It then describes the pathogenesis of acute rheumatic fever and rheumatic heart disease, caused by an autoimmune response to group A streptococcal infection. The document outlines the revised Jones criteria for diagnosing acute rheumatic fever and reviews treatment recommendations, including antibiotic therapy, anti-inflammatory drugs, and secondary prophylaxis to prevent recurrent episodes of rheumatic fever and progression of cardiac damage.

1. ACUTE RHEUMATIC FEVER
IN CHILDREN
Dr.S.Srinivasan
Professor of Paediatrics
MBBS – Medical Undergraduate Theory
Lecture for 8th and 9th Semester Students
ARF -Dated: 11th February 2016

2. World Heart Federation
“ Rheumatic heart disease (RHD) is the most
common acquired heart disease in children in
many countries of the world, especially in
developing countries.
The global burden of disease caused by rheumatic
fever currently falls disproportionately on
children living in the developing
world, especially where poverty is widespread ”

3. Immuno-pathogenesis of
Acute Rheumatic Fever & RHD
• Group A Beta Hemolytic Streptococcal infection
• Autoantibody response against the heart
• (Anti-myosin antibodies)
• Antibody deposition on heart valve endothelium and in
the myocardial vessels and tissues.
• Infiltration of myocardium and valvular region with
macrophages and autoreactive T lymphocytes.
• Destruction of myofibers
• (Cardiomyocytes in myocardium)
• Valvulitis, Scarring, collagen deposition
• (Heart murmur)
ARF -Dated: 11th February 2016

4. ARF -Dated: 11th Feb 2016

5. Acute Rheumatic Fever
Arthritis Carditis Chorea
Rheumatoid arthritis
Systemic lupus
erythematosis,
Serum sickness
Sickle cell disease
Post infective reactive
arthritis
(Shigella, Salmonella,
Yersinia)
Leukemia
Kawasaki
disease
Viral
myocarditis,
Viral
pericarditis
Congenital
heart disease
Innocent
murmur
TICS,
Hyperactvity
Huntington chorea
Wilson disease
SLE
ARF -Dated: 11th February 2016

6. Revised Jones criteria of 1992
(WHO adoption of 2004)
Clinical and
Laboratory
criteria
Major Polyarthritis
Carditis
Chorea
Subcutaneous nodules
Erythema marginatum
Minor Fever
Polyarthralgia
ESR, CRP. leukocytosis
ECG: Prolonged PR interval
Supportive
evidence of
preceding
streptococcal
infection
Anti streptolysin O, Anti-deoxyribonuclease
H/ of (within previous 45 days) streptococcal sore throat
Scarlet fever
Positive throat culture
Positive rapid streptococcal antigen detection test
ARF -Dated: 11th February 2016

7. Management of Children with
Acute Rheumatic Fever
ANTIBIOTIC THERAPY
A single deep IM injection: Benzathine Penicillin regardless
of the throat culture results to eradicate GABS from the
upper respiratory tract.
or 10 days of orally administered Penicillin or Erythromycin
Anti-inflammatory Therapy
Aspirin (Arthritis) & Prednisolone + Aspirin (Carditis)
General Measures -
Rest and ambulation
Ambulate as soon as the signs of acute inflammation have
subsided with longer periods of bed rest for carditis children

8. Treatment of Acute Rheumatic Fever
Clinical condition Anti-inflammatory Drug
Arthralgia Any analgesic can be used
Arthritis Aspirin 100mg/kg/day in divided doses for two
weeks followed by 75mg/kg/day in divided doses
for 4-6 weeks.
Carditis with
cardiomegaly or
CHF
Prednisolone 2mg/kg/day in divided doses x 2
weeks
Tapering dose to be stopped in another two
weeks.
While tapering Steroids, Start
Aspirin 75mg/kg/day in divided doses for 4-6
weeks to prevent re-bound phenomena.
CHF Antifailure measures – Bed Rest , Diuretics,
Digoxin, Salt Restricted Diet.

9. Duration of Acute Rheumatic Fever
• Average : About 12 weeks
• Rarely prolonged even up to 6 months
especially with carditis or chorea
• “ Chronic Rheumatic Fever ”:
Rare (active for more than six months )

10. COURSE AND PROGNOSIS of ARF
“Rheumatic Fever licks the joints and bites the heart”
(1884, Lasegue)
PROGNOSIS
Arthritis Good prognosis
Chorea
Carditis 25% ( 1in 4 ) of RF patients : Residual heart disease (
MR,MS,AR,AS ) If carditis is limited to systolic murmur
ARF with congestive cardiac failure develops chronic
valvular heart disease over a period of 5-10 years
Death
during
acute ARF
Rare in the past 30 years
( better awareness and early Health seeking behaviour
and treatment facilities )

11. Primary Prophylaxis for Rheumatic Fever
Treatment of streptococcal tonsillo pharyngitis
(Adopted from WHO Technical Report Series 2004 )
Antibiotic Mode Dose Duration
Benzathine
Penicillin
Intramuscular <27 kg: 6 lac units
>27 kg: 12 lac units
Single dose.
Phenoxymethyl
penicillin
(penicillin-V)
Orally 2-4 times/day Children:
250-500 mg
Adults:
250 mg or 500mg
10 days.
Amoxicillin Orally 2-3 times/day 25-50 mg/kg/day
Adult dose: 750-1,500
mg/day.
10 days
First generation
Cephalosporin
Orally 2-3 times/day Varies with agent 10 days
Erythromycin
ethyl succinate
Orally 4 times/day 40 mg/kg/day 10 days

12. Secondary Penicillin Prophylaxis
CONDITION DURATION OF PENICILLIN
PROPHYLAXIS
No carditis 5 years or until 21 years old
Carditis without
residual cardiac
defect-
10 years or until 21 years
old
Cardiac defect until 40 years old or later

13. Drugs for Secondary Rheumatic
Prophylaxis
Drugs Dose Route Frequency
Benzathine penicillin
(benzathine penicillin G)
600,000 units for patients
<27kg (60 lb)
im 3 to 4
weeks
1,200,000 units for
patients ≥27kg (60 lb)
OR
Phenoxymethyl penicillin
(penicillin V)
250 mg Oral bd
Patients allergic to penicillin:
Sulfadiazine 0.5g for patients <27kg
(60 lb)
Oral od
1.0g for patients ≥27kg (60
lb)
Patients allergic to penicillin and Sulfadiazine:
Erythromycin 250 mg Oral bd