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ACUTE RHEUMATIC

FEVER
Burhanuddin Iskandar
Pediatric Cardiology
Pediatric Department,Medical Faculty,
Hasanuddin University/ WS Hospital
Makassar

ETIOLOGY
1. Immunologic
Streptococcus Beta hemolytic group A
2. Predisposing factors
- Family history
- Socio economic status
- Age 5 -15 years ( peak 8 years)

PATHOLOGY
Inflammatory lesion : heart, brain, joints,
skin

Aschoff bodies (in atrial myocardium) :


characteristic ?
Central necrosis surrounded by lymphocy
tes, plasma cells, and large mononuclear
and giant multinucleate cell

Aschoof Body : the cells are large, multinucleotide

CLINICAL MANIFESTATIONS
History
Streptococcal pharyngitis, 1-5 wks (ave 3
wks) before onset; chorea 2-6 mos
Pallor, easy fatigability, epistaxis, abdo
minal pain
Positive family history

2. Carditis
50 % of cases, usu within first 3 wks
Diagnosis requires presence of 1 of 4:
- organic heart murmur
- pericarditis (friction rub, pericard effusion,
chest pain, ECG changes)
- cardiomegaly on chest X ray
- congestive heart failure

Jones criteria (updated


1992)
Mayor criteria
1. Arthritis

* Affects 70 % of cases
* Large joints : knee, ankle, elbow, wrist
* Often > 1 joints, simultaneously or
in succession, migratory
* Swelling, heat, redness, severe pain,
tenderness, motion <
* Dramatic response to salicylate

3. Erythema marginatum
- <10 % of cases
- Non pruritic annular erythematous
rashes, never on face
- Most prominent on trunk and inner
proximal portions
- Disappear on exposure to cold,
seldom detected on AC room

Erythema marginatum

4. Subcutaneous nodules
- 2-10 % of cases, esp in recurrences
- Hard, painless, non pruritic, freely
moveable, swelling 0.2-2 cm
- Usually symmetric on extensor
surfaces
of joints, scalp, along spine, has
significant association with carditis

Subcutaneous Nodule

5. Sydenhams chorea
- 15 % of patients, more often in prepubertal

girls.
- begin with emotional lability and personal
ity changes
- spontaneous, purposeless movement
followed by motor weakness, slurred speech
- Dysfunction of basal ganglia and cortical
neuronal components (antineuronal antibody)

Minor criteria
-

Arthralgia
Fever
Elevated acute phase reactants: CRP,
ESR
- ECG : PR interval > : not specific

Evidence of antecedent Group


A Streptococcal infection
Positive throat culture or rapid

streptococcal antigen tests for group A :


less reliable (recent and chronic infect)
Streptococcal antibody tests : most
reliable
- ASTO : 80%
- Anti-DNA se B
- Anti hyaluronidase

Diagnosis of rheumatic
fever
Based on
2 major criteria
or
1 major + 2 minor

ASTO

Exeptions
Chorea may occur as the only

manifestations of RF
Indolent carditis may be the only
manifestation
Occasionally patients with RF
recurrences
may not fulfill the Jones criteria

Differential diagnosis of RF
Juvenile rheumatoid arthritis
Collagen vascular diseases
Virus associated acute arthritis

Note
* Rheumatic fever is a clinical syndrome for
which no specific diagnostic test exist !
* No symptom, sign or lab test result is
pathognomonic, although several
combinations of them are diagnostic
* Only carditis can cause permanent cardiac
damage. Signs of mild carditis disappear
rapidly in weeks but severe carditis may last
for 2-6 months. Chorea and arthritis usually
subside without permanent damage.

Management of RF
Benzathin penicillin G 0.6 1.2 M units IM
for eradication and prophylaxis
Bed rest
Acetosal for mild cases
Prednison for severe cases
Antiinflammatory agents not needed for
isolated chorea

Recommended anti-inflammatory agents


_______________________________________________________________________________________

Arthritis
Mild
Moderate
alone
carditis
carditis
__________________________________________________
Prednisone
0
0
0
Aspirin
1-2 wk
3-4 wk#
6-8 wk
___________________________________________________

Severe
carditis
2-6 wk*
2-4 mo

* Prednisone should be tapered and aspirin started during the final


week
# Aspirin may be reduced to 60 mg/kg/day
Dosages
Prednisone : 2mg/kg/day, in 4 divided doses
Aspirin : 100 mg/kg/day, in 4-6 divided doses

Bed rest and indoor ambulation

____________________________________
Arthritis
Mild
Moderate
Severe
Alone
Carditis
Carditis
Carditis
__________________________________________________________
Bed rest
1-2 wk
3-4 wk
4-6 wk as long as HF +
Indoor ambulation
1-2 wk
3-4 wk
4-6 wk
2-3 mo
_________________________________________________________
ESR: important for duration of restriction of activities.
Full activity : ESR normal, except significant cardiac involvement _

Mild carditis : questionable

cardiomegaly
Moderate carditis : definite but mild
cardiomegaly
Severe carditis : marked
cardiomegaly or
HF (heart failure)

Prevention
- Ideally prophylaxis is indefinite
- Benzathin Penicillin (600,000-1,200,000

U) every 28 days, min till age 21-25 ys


- Sulfadiazine 0.5 g 1x daily (BW < 27 kg),
1 g 1X (BW >27 kg)
- Penicillin V 2 x 250 mg /day
- Erythromycin 2 X 250 mg /day

Thank
You
NO PAIN NO GAIN

RHEUMATIC HEART DISEASE


Affects
Mitral valve 75 %
Aortic valve 25 %
Tricuspid valve rare
Pulmonary valve never
Stenosis and regurgitation usually occur
together

Mitral stenosis
Prevalence
Most common valvular involvement in
adult
Requires 5-10 years from the initial
attack

Pathology

- Thickening of the leaflets and fusion of the


commisure
- Calcification results overtime
- Dilated and hypertrophied LA and right sided
heart
- Pulmonary venous hypertension pulmonary
congestion and edema and fibrosis of the
alveolar walls, hypertrophy of the pulmonary
arterioles, loss of lung compliance

Stenotic Mitral Valve

Commisures are fused and valve thickened

Clinical manifestations
Mild MS : asymptomatic
More severe : dyspnea with/out
exertion :
orthopnea, nocturnal
dyspnea or palpitation

Physical Examinations
Increased RV impulse along the LSB
Weak peripheral pulse with narrow
pulse pressure
Pulmonary hypertension : loud S1 at
apex and narrow split S2, accentuated
P2
Mid diastolic/presystolic murmur

ECG : RAD, LAH, RVH (due to PH)


CXR :
Enlarged LA and RV, MPA segment
prominent
Pulmonary venous congestion

Treatment of MS
Prophylactic antibiotic
Restriction of activity depends on
severity
Symptomatic patients (dyspnea on
exertion, pulmonary edema,
paroxysmal dyspnea) : baloon or
surgery

MITRAL REGURGITATION
Most common in RHD
Pathology
Mitral valve leaflets are shortened
because of fibrosis.
When degree of MR increases,
dilatation of LA and LV results, mitral
ring becomes dilated

Mitral Valve involvement

Echocardiography

Clinical manifestations
* Asymptomatic during childhood
* Rare : fatigue, palpitation

Physical examination
Heaving, hyperdynamic apical impulse
in severe MR
S1 normal or diminished. S2 may split
(shortening of LV ejection, early aortic
closure)
Pansystolic murmur at apex left
axilla

ECG
Normal in mild cases
LVH or LV dominance, with or without LAH
CXR
LA and LV enlarged
Pulmonary congestion pattern in CHF

Treatment
Prophylactic antibiotic
No restriction of activity in mild cases
Surgical : intractable CHF,
progressive
cardiomegaly, pulmonary
hypertension

AORTIC REGURGITATION
Less common than MR. Mostly
associated with mitral valve disease.
Pathology
* Semilunar cusps are deformed and
shortened.
* Valve ring is dilated
* Commisures usually are fused

Aortic Valvulitis

Clinical Manifestations

Mild regurgitation : asymptomatic


More severe : reduced exercise
tolerance test

Physical Examination
Precordium may be hyperdynamic. Diastolic thrill
at 3 LICS
S1 decreased, S2 may be normal or single
High pitched diastolic cresendo murmur at
3 LICS or 4 LICS
Systolic murmur at 2 RICS due to relative AS
Severe AS : middiastolic murmur at apex

ECG
Normal in mild cases
Severe : LVH, LAH
CXR
Cardiomegaly (LVH)
Dilated ascending aorta

Treatment
Prophylactic antibiotics
Mild cases : no restriction in activity
Surgical : in anginal pain or dyspnea
on exertion, significant cardiomegaly

Thank
You
NO PAIN NO GAIN