Acute Rheumatic Fever

And

Rheumatic heart disease

by

Agus Priyatno

Etiologi :
1. Immunologic
Streptococcus beta hemolytic group A
2. Predispositing Factor:
- Family history
- Socio-economic status
- Age 5 15 years ( peak 8 years)

Pathology
Inflamatory lesion; heart,brain,joint,skin
Ashoff bodies ( in atrial myocardium):
characteristic ?
Central necrosis surrounded by lymphocytes,
plasma cell, and large mononuclear and giant
multinucleate cell

Clinical manifestations
History
Streptococcal pharyngitis, 1 5 weeks (ave 3) before
onset.
Chorea 2 6 mos.
Pallor, easy fatigability, epistaxis, abdominal pain
Positive family history.
Rheumatic fever
Strep throat
Strep skin

Acute glomerulonephritis

Histologic section of typical RF

Jones criteria ( update 1992)

o Minor criteria :

o Major criteria :
- arthritis
- carditis
- erythema marginatum
- subcutaneous nodules
- Sydenhams chorea.

- arthalgia
- fever
- elevated acute phase
reactan ( CRP, ESR)
- ECG : PR interval > :
not specific

plus
Supporting evident of antecedent Strep group A infec.
- culture (+) or rapid strept antigent test
- elevated or rising ASTO

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

Migratory arthritis

2. Carditis
- 50 % of cases
- Diagnosis requires presence of :
1. Tachycardia
2. Heart murmur of valvulitis ( MR and AR )
3. Pericarditis( friction rub, pericard
effusion,chest pain, ECG changes)
4. Cardiomegaly on chest X ray
5. Congestif heart failure ( severe carditis)

Involve of the heart;

pericarditis

Involve of the heart;

myocardium and
endocardium

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

Erythema
marginatum

4. Subcutaneous nodules
- 2 10% of cases, esp in recurrences
- Hard, painless, non pruritic,freely moveable,
swelling 0,2 2 cm
- Ussually symmetric on extensor surfaces
of joint, scalp, along spine, last for weeks

Subcutaneous
nodules

5. Sydenhams chorea.
- 15% of cases, more often in prepubertal girl
- Begin with emotional lability and personality
changes.
- Spontaneous, purposeless movement followed
by motor weakness, slurred speech
- Dysfunction of basal ganglia and cortical neural
components

Involuntary grimaces

Diagnosis of rheumatic fever

Based on
2 major criteria
or
1 minor criteria

+ ASTO

Exception to the Jones criteria

1. Chorea may occur as the only manifestation
of RF
2. Indolent carditis may be the only manifestation
3. Occasionally patients with RF recurrences may
not fulfill the Jones criteria

Differential diagnosis of RF

Juvenile rheumatoid arthritis

Collagen vascilar disease
Virus associated acute arthritis
Hematologic disorder: sicklemia and leukemia

Note
RF is a clinical syndrom for which no spesific
diagnostic test exist
No symptom, sign or lab test result is pathogno
monic, although several combinations of them
are diagnostic
Only carditis can cause permanent cardiac damage.
Sign of mild carditis disappear rapidly in weeks
but severe carditis may last for 2 6 month.
Chorea and arthritis usually subside without
permanent damage

Management of RF
Benzathin penicillin G 0,6 1,2 M units i.m
for eradication and prophylaxis.
Allergic to penicillin : erythromisin 40 mg/kg/day
in two to four doses for 10 days.
Bed rest
Acetosal for mild cases
Prednison for severe cases
Anti inflammatory agents not needed for isolated
chorea
Treatment of congestive heart failure

Recommended anti-inflammatory agents

Prednison
Aspirin

Arthritis
alone

Mild
carditis

1-2 wk

3-4 wk#

Moderate
carditis

0
6-8 wk

Severe
carditis

2-6 wk*
2-4mo

Prednison should be tapered and aspirin started during the

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

Bed rest and indoor ambulation

Arthritis
alone

Mild
carditis

Moderate
carditis

severe
carditis

Bed rest

1-2 wk

3-4 wk

4-6 wk

as long
as CHF

Indoor amb

1-2 wk

3-4 wk

4-6 wk

2-3 mo

2-4 wk

1-3 mo

2-3 mo

>6-10 wk

>3-6 mo

variable

Outdoor activ
2 wk
(school)
Full activity
>4-6 wk

Mild card: Questionable cardiomegaly, moderate: definite but mild,

Severe: marked cardiomegaly or CHF

ESR: important for duration of restriction of activities

Full activity: ESR normal, excep significant cardiac involvement

Prevention
Ideally prophylaxis is indefinite
Benzathin penicillin every 28 days, min till
age 21 25 ys
Sulfadiazine 0,5 g 1x daily ( bw < 27 kg)
1 g 1x daily (bw > 27 kg)
Peniccilin V 2 x 250 mg/day
Erythromycin 2 x 250 mg/day

RHEUMATIC HEART DISEASE

Affecs; - Mitral valve
75%
- Aortic valve
25%
- Tricuspid valve rare
- Pulmonary valve never
Stenosis and regurgitation usually occur
together.

MITRAL STENOSIS

prevalent
most common valvular involvement in adult
requires 5 10 ys from the initial attack
if RF is prevalent, MS occurs under age 15 ys

Pathology
Thickening of the leaflets and fusion of the
commisure
Calsification result overtime
LA and right-sides heart chambers become dilated
and hipertrophied
Pulmonary venous hypertension, pulmonary congestion
and edema and fibrosis of the alveolar walls,
hypertrophy of the pulmonary alveolar, loss of lung
compliance

Aortic and mitral valve

Clinical manifestations
Mild MS : asymptomatic
More severe: dyspnea with/out exertion, orthopneu,
nocturnal dyspnea and palpitation.

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

Cardiac finding of MS

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

- atrial fibrilation is rare in children
CXR : - enlarge LA and RV,
- MPA segment prominent
- pulmonary venous congestion
Echocardiography :
accurate noninvasive tool to detection of MS
M-mode; diminished E to F slope, thickened
mitral leaflets, large LA dimention
Two D : doming of thick mitral, a small mitral
orifice, dilated LA, MPA, RV and RA
Doppler: estimate of pressure gradient;Mitral
valve and pulmonary valve.

Treatment of MS
prophylactic antibiotic
restriction of activity depends on severity
symptomatic patient ( dyspnea on exertion,
pulmonary edema, paroxysmal dyspnea): balloon
or surgery

Typical appearances of advanced MS on mediastinal

organ and lung

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 result, mitral ring becomes dilated.

Clinical manifestation:
asymptomatic during chilhood
rare; fatigue, palpitation

Physical examination
heaving, hyperdinamic apical impuls in severe MR
S1 normal or diminished.
S2 may split ( shortening of LV ejection, early
aortic closure )
S3 commonly is present and loud
Pansystolic murmur at the apex, with transmision
to the left axilla

Cardiac finding of MR

ECG:
- normal in mild cases
-LVH or LV dominance, with or without LAH
-Atrial fibrilation is rare in children
CXR:
-LA and LV enlarged
-Pulmonary congestion pattern in CHF
Echocardiography:
-two D : dilated LA and LV
-color-flow mapping; regurgitant jet into the LA
-doppler: asses the severity of the regurgitation

Treatment:
Prophylactic antibiotic
No restriction of activity in mild cases
Surgical: intractable CHF,
progressive cardiomegaly,
pulmonary hypertension
If atrial fibrilation; digoxin
Afterload-reducing agent; maintaining the forward
stroke volume.

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

Clinical manifestation
mild regurgitation: asymptomatic
more severe; reduce exercise tolerance test
Physical examination
precordium may be hyperdinamic, distolic 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

Cardiac finding of AR

ECG : - normal in mild cases

- severe ; LVH, LAH
CXR : - cardiomegaly (LVH)
- dilated ascending aorta
Echocardiography :
the LV dimension is increased
color-flow and doppler to estimate the severe
of the regurgitation.

Treatment:
prophylactic antibiotics
mild case : no restriction in activity
surgical : in anginal pain or dyspnea on
exertion, significant cardiomegaly

Streptococcal infection
And reccurent
For three prophylactic:
1. Sulphadiazin
2. Penicillin
3. Benzatin penicillin

Decline in new cases of RF

admitted to hospital

Thank You