Acute rheumatic fever is a connective tissue disease that primarily affects the heart, joints, and central nervous system. It follows a streptococcal throat infection after a latency period of about 3 weeks. The most common manifestations are migratory polyarthritis and carditis, which can lead to valvular fibrosis and heart disease. Treatment involves bed rest, antibiotics to treat the initial strep infection, and anti-inflammatory drugs. Long-term antibiotic prophylaxis is also used to prevent recurrence.

1. Acute rheumatic fever

2. Acute rheumatic fever
Generally classified as connective tissue
disease or collagen vascular disease.
Primarily heart, the joints and CNS.
Follow gr.A streptococcal
tonsillopharyngitis with a latency period of
about 3 weeks.
Most common cause of acquired heart
disease in children world-wide.
Fibrosis of heart valves.

3. Acute rheumatic fever
Peak age, 5 – 15yrs (rare before 5yrs of
age).
No sex predilection.
Untreated gr.A streptococcal pharynigitis
precipitates rheumatic fever.
Proper treatment of streptococcal
pharynigitis virtually eliminates the risk for
rheumatic fever.

4. Epidemiology
Poverty, overcrowding and conditions
facilitating spread of gr.A streptococcal
pharyngitis.
Magnitude of the immune response to the
antecedent streptococcal pharyngitis.
Persistence of the organism during
convalescence.
Rheumatogenicity of gr.A strept strains.

5. Epidemiology…
Previous attack of RF (risk of re-attack is about
50%).
? Familial predisposition.
Pathogenesis:
Toxin theory ???
Immunologic theory? – rheumatogenic strains
(M types 1,3,5,6,14,18,19,27 and 29).

6. Pathogenesis
Distinct structural characteristics of M
proteins (Epitopes shared with human
heart tissue).
Heavily encapsulated forming mucoid
colonies.
Resistance to phagocytosis.
Susceptible host (genetic predisposition).

7. Pathology
Exudative & proliferative inflammatory rxns
involving connective or collagen tissue →
fibrinoid degeneration → edematous and
eosinophilic interstitial connective tissue →
mononuclear cell infilteration (including
large modified fibrohistiocytic cells
(Aschoff cells) → Aschoff Giant cells
(Aschoff nodules).

8. Pathology…
Aschoff nodules are Pathognomonic of
rheumatic carditis and are found only in
the heart.
Inflammation of valvular tissue is the
most common manifestation of rheumatic
carditis.
Valvular insufficiency is the initial feature
while stenosis is due to fibrosis and
calcification.

9. Diagnosis
Modified Jones Criteria:
Major –
Carditis
Migratory polyarthritis.
Sydenham’s chorea
Erythema marginatum
Subcutaneous nodules

10. Modified Jones…
Minor criteria
Clinical findings
 Arthralgia
 Fever
Laboratory findings
 Elevated acute phase reactants (ESR,
CRP).
 Prolonged PR interval.

11. Diagnosis…
Supporting evidence for antecedent
streptococcal pharyngitis:
 Positive throat culture or rapid
streptococcal antigen test.
 Elevated or rising streptococcal antibody
titer.

12. Diagnosis …
Diagnosis made with:
2 major criteria or 1 major and 2 minor
+
Supporting evidence for antecedent
streptococcal pharyngitis (mandatory)

13. Diagnosis …
Exceptions (strict adherence to Jones
criteria not needed):
1. Sydenham’s Chorea
2. Indolent Carditis
3. Rheumatic Fever recurrence

14. Major manifestations
1. Carditis (in 50 – 60% of patients)
Pancarditis (myocardium, endocardium and
pericardium).
The most specific manifestation of rheumatic
fever.
Cardiac murmur – most important
manifestation.
Mitral and Aortic valvulitis and involvement of
the chordae of the mitral valve – most
characteristic.

15. Major …
Mitral regurgitation – hallmark of
rheumatic carditis.
Involvement of the right side valves (TV
& PV) – less common.
2. Migratory polyarthritis (in about 75%):
Most common major manifestation but
least specific.
Almost always asymmetrical and
migratory.

16. Major …
Larger joints (knees, ankles, elbows,
wrists).
Swelling, severe pain, redness, heat,
limitation and tenderness.
No permanent joint deformity.
Untreated – lasts 2 to 3weeks.
Dramatic response to salicylates -
hallmark

17. Major …
3. Chorea (involvement of Basal ganglia &
caudate nucleus)
 In about 20% of patients with RF.
 Delayed manifestation – usually 3mo or
longer.
 Purposeless and involuntary movements,
muscle incoordination, weakness and
emotional liability.
 May disappear with sleep.

18. Major …
4. Erythema marginatum:
In < 5% of cases.
Evanescent, erythematous, macular
nonpruritic rash with pale centers and
rounded or serpinginous margins.
Mostly trunk and proximal extremities.
May be induced by application of heat.

19. Erythema marginatum

20. Erythema marginatum

21. 5. Subcutaneous nodules
In less than 3% of patients with RF.
Firm, painless, freely movable nodules
(0.5 – 2cm in size).
Most often seen in patients with carditis.
Usually located over the extensor
surfaces of the joints (elbows, knees and
wrists), in the occipital portion of the
scalp, or over the spinous processes.

22. Treatment
General
Place on bed rest and monitor closely for
evidence of carditis.
Antibiotic treatment for 10 days with oral
penicillin or erythromycin or a single IM
dose of Benz. Penicillin.
Long-term antibiotic prophylaxis.

23. Treatment …
Anti – rheumatic therapy:
Withheld anti-inflammatory treatment till
full blown picture of RF appears.
Pain relief – achieved by acetaminophen.
Migratory polyarthritis and carditis with
out Cardiomegaly or CHF → ASA
100mg/kg/24hr divided into 4 doses po for
3 – 5 days, then 75mg/kg/24hr for
4weeks.

24. Treatment …
Carditis with cardiomegaly or CHF →
Prednisone 2mg/kg/24hr divided into 4
doses po for 2 – 3weeks. While tapering
prednisone start ASA 75mg/kg/24hr in 4
divided doses for 6weeks.
Supportive treatment.

25. Prevention
I. Primary Prevention (prompt and proper
treatment of gr.A streptococcal pharyngitis).
A. Benz. Penicillin
wt ≤ 27kg→ 600,000IU IM stat.
wt > 27kg→ 1,200,000IU IM stat.
B. Penicillin V
Children 250mg po 2-3x/d for 10d.
Adolescents 500mg po 2-3x/d for 10d.

26. Prevention …
C. Erythromycin (in penicillin allergy)
40mg/kg/24 divided into 2-4 doses po
for 10d.
D. Azithromycin (fewer GI side effects)
500mg po on the first day, then 250mg
po/d for 4 days.
E. Oral cephalosporins (alternative).

27. Prevention …
II. Secondary prevention (prevention of
recurrence).
A. Benz. Penicillin 1.2M IU IM every 3 – 4
weeks.
B. Penicillin V 250mg po bid
C. Sulfadiazine 500 – 1000mg po/d
D. Erythromycin 250mg po BID

28. References
1. Braunwald’s Heart Disease: A textbook of
Cardiovascular Medicine. 7th Edition, 2005.
2. Nelson Textbook of Pediatrics. 17th edition,
2004.
3. Heart Disease in infants, children and
adolescents including the fetus and young
adult. 6th edition, 2001.
4. www.eMedicine.com
5. www.medscape.com