ACUTE RHEUMATIC FEVER

Dr Nadjib.A.SpA(k)
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
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
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
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
3. Erythema marginatum
- <10 % of cases
- Non pruritic erythematous rashes,
never on face
- Most prominent on trunk and inner
proximal portions
- Disappear on exposure to cold,
seldom detected on AC hospitals
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, lasts for
weeks
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
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
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
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
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
Clinical manifestations
* Asymptomatic during childhood
* Rare : fatigue
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
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

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