7/2/2014 Hailu B, MD 1

Acute rheumatic fever

Hailu Berta, MD
Pediatrician & Neonatologist
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Acute rheumatic fever (RF)

RF is a multi-system, non-suppurative, inflammatory
disease triggered by Group A β-hemolytic Streptococcus
infection of upper respiratory tract, tonsillopharyngitis, with
latency period about 3wks.
Generally classified as connective tissue disease or
collagen vascular disease.
Involve primarily heart, the joints and CNS.
Most common cause of acquired heart disease in children
world-wide.
Fibrosis of heart valves is ultimate finding.
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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.
Attack rate after GABHS tonsilopharyngitis is 3%
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Epidemiology
predisposing condition:
Poverty, overcrowding and conditions facilitating spread of gr.A
streptococcal pharyngitis.
Presence of Untreated Tonsilopharyngitis is main factor
Magnitude of the immune response to the antecedent
streptococcal pharyngitis.
Persistence of the organism during convalescence.
Rheumatogenicity of gr. A streptococcus strains.
Previous attack of RF (risk of re-attack is about 50%).
Familial predisposition.
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Epidemiology…
Pathogenesis:
The exact mechanism is yet to be
determined; different proposed theories:
Toxin theory : toxic effect of extra-cellular toxins
produced by strep on target organs such as
myocardium, valves, synovium & brain
Immunologic theory : most popular hypothesis;
immunologic damage on target organs as a
result of antigenic similarity b/n strep cellular
components & human tissue
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Pathogenesis
clues for supporting the immunological mechanism
includes
rheumatogenic strains (M types: 1,3,5,6,14,18,19,27 and
29). have distinct structural characteristics (Epitopes)
which is shared with human heart tissue; Antigenic
similarities
Have similar aa sequence with some human tissue which
can cross-react; genetic predisposition
the organism is heavily encapsulated, mucoid forming,
Resistant to phagocytosis.
The presence of latent period of 1-3weeks b/n URTI &
RF.
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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).
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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.
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Diagnosis
Modified Jones Criteria:
Major –
Carditis
Migratory polyarthritis.
Sydenham’s chorea
Erythema marginatum
Subcutaneous nodules
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Modified Jones…
Minor criteria
Clinical findings
 Arthralgia (In the absence of arthritis)
 Fever
Laboratory findings
 Elevated acute phase reactants (ESR, CRP).
 Prolonged PR interval.
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Diagnosis…
Supporting evidence for antecedent streptococcal
pharyngitis:
 Positive throat culture or rapid streptococcal antigen test.

 Elevated single measure of (320 TU) or rising

streptococcal antibody titer; ASO titer
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Diagnosis …
Diagnosis made with:
2 major criteria or 1 major and 2 minor
+
Supporting evidence for antecedent
streptococcal pharyngitis (mandatory)
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Diagnosis …
Exceptions (strict adherence to Jones criteria not
needed):
1. Sydenham’s Chorea: if other cause excluded
2. Indolent Carditis: no other explanation
3. Rheumatic Fever recurrence:
 in patients with documented rheumatic heart disease or prior RF,
 the presence of 1 major criteria OR of fever, arthralgia OR  acute
phase reactant suggests a presumptive Dx of recurrence (evidence
of strep infection is needed)
• N.B. 1 & 2 do not need evidence of strep infection
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Major manifestations
1. Carditis
in 50 – 60% of patients)
Pancarditis (myocardium, endocardium & 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.
Mitral regurgitation – hallmark of rheumatic carditis. PI
Involvement of the right side valves (TV & PV) – less
common.
present with MR, AI, Changing murmurs, Pericardial friction
rubs, Cardiomegaly, Arrhythmias (commonly 1st degree heart
block), CHF
Chronic & present with insufficiency, stenosis 2nd to scaring.
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Major …
2. Migratory polyarthritis
in about 75%
Most common major manifestation but least specific.
Almost always asymmetrical and migratory.
Involve Larger joints (knees, ankles, elbows, wrists).
Swelling, severe pain, redness, heat, limitation of
movement and tenderness.
May have effusion with Pleocytosis
No permanent joint deformity or chronicity.
Untreated – lasts 2 to 3weeks.
Dramatic response to salicylates - hallmark
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Major …
3. Chorea (Sydenham’s Chorea)
• Is sole neurologic manifestation of RF, involving cortex,
Basal ganglia & caudate nucleus,
• In about 20% of patients with RF, may be the only manifn.
• Is delayed manifestation – usually 3mo or longer.
• Characterized by purposeless and involuntary movements
(chorea), muscle incoordination, weakness (hypotonia) and
emotional liability.
 Chorea: Purposeless, involuntary & rapid movements –
prominent in the face, trunk & distal extremities & darts from
one muscle group to another
o Increased when stressed & disappears during sleep
o There may be deterioration in handwriting
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Chorea...
 Hypotonia: May involve the 4 extremities or may be unilateral
• When combined with severe chorea the child may be
incapable of feeding, dressing or walking
 Emotional Lability: Uncontrollable crying and extreme mood
swings are characteristic
Typical signs associated with S. Chorea are:
 “Milk-maid’s grip” – relaxing & tightening hand grip
 “Choreic hand” – spooning of extended hand by flexion at the
wrist & extension of fingers
 “Darting tongue” – tongue can’t be protruded for > few
seconds
 “Pronator sign” – arms & palms turn outward when held
above head.
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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.
Mostly seen in chronic carditis
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Erythema marginatum
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Erythema marginatum
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Major...
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.
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Differential diagnosis
Infective Endocarditis
 Juvenile rheumatic arthritis
Complications
 The major complication is rheumatic valvular
heart disease
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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.
Supportive treatment.
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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.
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.
Chorea: treat with chlorpromazine
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Prevention
1. Primary Prevention (prompt and proper
treatment of gr.A streptococcal pharyngitis).
 Benz. Penicillin
wt ≤ 27kg→ 600,000IU IM stat.
wt > 27kg→ 1,200,000IU IM stat.
 Penicillin V
Children 250mg po 2-3x/d for 10d.
Adolescents 500mg po 2-3x/d for 10d.
 Erythromycin (in penicillin allergy)
40mg/kg/24 divided into 2-4 doses po for 10d.
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Prevention …
 Azithromycin (fewer GI side effects)
500mg po on the first day, then 250mg po/d for 4 days.
 Oral cephalosporins (alternative).
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Prevention …
2. Secondary prevention (prevention of recurrence).
 Benz. Penicillin 1.2M IU IM every 3 – 4 weeks. or
 Penicillin V 250mg po bid or
 Sulfadiazine 500 – 1000mg po/d or
 Erythromycin 250mg po BID
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