A systemic connective tissue disease which follows a streptococcal upper respiratory tract infection. It may involve the heart, joints, skin, subcutaneous tissue, and CNS.
The first attack usually occurs between ages of 3–15 years.
Causes
- Hypersensitivity reaction to group A streptococcal throat infection
Clinical features
- Arthritis (migrating asymmetric polyarthritis)
- Acute rheumatic carditis, signs of cardiac failure, murmurs and pericarditis
- Subcutaneous nodules
- Chorea (involuntary movements of limbs)
- Skin rash
- Other minor signs/symptoms: fever, arthralgia, laboratory findings
Differential diagnosis
- Any form of arthralgia/arthritis including sickle cell disease, haemophilia
- Pyrexia with cardiac failure
Investigations
- Blood: Haemogram (raised ESR)
- Chest X-ray
- ECG
- Echocardiography
- Antistreptolysin O titre (ASOT)
Diagnostic criteria (revised Jones criteria)
- Evidence of recent streptococcal infection
- Elevated ASO-titer or other streptococcal Ab titres or positive throat swab for group A beta-hemolyticus streptococcus
PLUS - Two major manifestations or one major and two minor manifestations
- Elevated ASO-titer or other streptococcal Ab titres or positive throat swab for group A beta-hemolyticus streptococcus
MAJOR MANIFESTATIONS | MINOR MANIFESTATIONS |
---|---|
|
|
Management
- Bed rest
To eradicate any streptococci:
- Phenoxymethylpenicillin (Pen V) 250 mg every 6 hours for 10 days
Child: 125 mg per dose - Or Benzathine benzylpenicillin dose 1.2 MU IM
stat
Child < 30 kg: 0.6 MU
Child > 30 kg: 1.2 MU
To treat the inflammation
- Acetylsalicylic acid 4-8 g/day untill signs of inflammation subside (usually 4-8 weeks)
Child: 80-100 mg/kg/day in 3 doses - Plus magnesium trisilicate compound 2-4 tablets every 8 hours
Taken 30 minutes after the acetylsalicylic acid tablets
If allergic to aspirin
- Low dose steroid
If carditis/heart failure symptoms
- Treat as per heart failure guidelines
- Consider high dose steroids (specialist only)
If chorea:
- Valproate 10-20 mg/kg/day
Prophylaxis
To prevent further episodes
- Pen V 500 mg 12 hourly
Child: 125-250 mg 12 hourly - Or Benzathine benzylpenicillin 1.2 MU IM every 4 weeks
Child <30 kg: 0.6 MU
If allergic to penicillin:
- Erythromycin 250 mg 12 hourly
- Child: 10 mg/kg twice a day
Duration of prophylaxis depends on severity of disease:
- Rheumatic fever without carditis: for 5 years or until age 18 or 21 years old
- Carditis but no residual heart disease: for 10 years or until age 25 years old
- Carditis with residual heart disease: untill age 40- 45 years or for life
Prevention
- Early diagnosis and treatment of group A Streptococcus throat infection
- Avoid overcrowding, good housing
- Good nutrition