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 + ASTO
1 major + 2 minor


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 Severe
alone carditis carditis carditis
__________________________________________________
Prednisone 0 0 0 2-6 wk*

Aspirin 1-2 wk 3-4 wk# 6-8 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








DEMAM REMATIK
KARDITIS (+)
SEMBUH
KARDITIS (-)
PENYAKIT JANTUNG
REMATIK
REAKTIVASI

SEMBUH
REAKTIVASI
3 6 bulan
Bising masih
ada
Bising -


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