Acute rheumatic fever is an inflammatory disease that occurs 2-3 weeks following a group A streptococcal infection, due to antigenic similarities between the bacteria and heart tissues. It is most common in children ages 5-15. The infection triggers an autoimmune response that causes inflammation in the heart, joints, skin and brain. Long-term complications include rheumatic heart disease. Diagnosis is based on modified Jones criteria of both clinical features and evidence of a preceding streptococcal infection. Treatment involves antibiotics for the initial infection along with medications for symptoms, and long-term antibiotic prophylaxis to prevent recurrences.

1. Acute rheumatic fever
Inflammatory disease following group A
β-hemolytic streptococcal infection,
due to cross-reactivity of antigens

2. Epidemiology
 Onset- ~2-3 weeks after streptococcal
infection
 Rate of development after untreated infection-
~3%
 Recurrence with a subsequent untreated
infection- 5-50%, more in patients with RHD
 Common in children- age 5-15,
only 20% first attacks in adults

3. Pathogenesis
 Possible autoimmune disease
 Follows pharyngitis due to encapsulated GABH streptococci
 A complex interplay of genetically determined host
susceptibility, pathogenic GABH streptococcal infection,
in a susceptible environment
 Best defined virulence factor- M protein
 Present on surface of bacteria
 Promotes bacterial adherence & resist phagocytosis
 Shares homology with cardiac myosin, tropomyosin, keratin, laminin
 Types 1,3,5,6,14,18,19,24 associated with ARF
 Other-
 T cell activation by streptococcal superantigens,
leading to granuloma formation

4. Pathology
 Myocardial Aschoff body- a submiliary
granuloma, later forms scar
 Endocardial verrucous valvulitis, heals
with fibrous thickening & adhesions,
causing stenosis or regurgitation
 Serofibrinous pericarditis
 Joint- exudative arthritis
 Subcutaneous nodules- granuloma

5. Modified Jones’ criteria
 Used for diagnosis
 2 major or 1 major+2 minor or 2
minor (in patient with RHD), with
evidence of streptococcal infection
 Evidence of s’coccal infection
within last 45 days-
 Elevated/rising ASO/anti-DNase B titres
 Positive throat culture
 Rapid Ag test for GABH s’cocci
 Recent scarlet fever

6. Jones’ criteria
 Major
 Migratory polyarthritis,
involves large joints
 Carditis- pancarditis
 Subcutaneous nodules-
painless, extensor
 Erythema marginatum-
over trunk/arms
 Sydenham’s chorea
 Minor
 Fever
 Arthralgia
 Raised ESR/CRP
 ECG- heart-block-
prolonged PR interval
 Previous e/o rheumatic
fever

7. Acute carditis
 New murmur or change in pre-existing murmur
 Apical pansystolic murmur- MR ± Carey Coomb murmur-apical MDM
 Basal early diastolic murmur- AR
 Tachycardia
 Soft heart sounds
 New onset CHF- gallop rhythm- S3
 Pericardial rub or effusion
 Cardiomegaly
Within 6 months of acute streptococcal infection

8. Course
90% attacks subside within 12 weeks
<5% persist >6 months

9. Recurrence
With new M-type streptococcal infection
Most common within first 5 years
Frequency decreases with time

10. GABH streptococcal pharyngitis
Age <15 years, high-grade fever,
tonsillar swelling/exudate,
tender anterior Cxal LNE,
absence of cough

11. Treatment
 Acute streptococcal infection-
1.2 million units of benzathine penicillin G
or amoxycillin/1st
gen. oral cephalosporin x 10 days
 Erythromycin, 500 BD x 10 days,
if penicillin sensitive/allergic
 Acute arthritis- ASA/NSAIDs
 Acute carditis- prednisolone, rest, diuretics, ACEI
 Chorea- diazepam, haloperidol, carbamazepine
Monitor ESR/CRP for duration of symptomatic
Rx

12. Prophylaxis
 1.2 million units of benzathine penicillin G,
IM q 3-4 weeks
 Sulfadiazine, 500 BD or Erythromycin, 250 BD,
if penicillin sensitive
 Duration-
 Without carditis- X 10 years or upto 21 years of age,
whichever is longer (WHO- x 5 yrs./upto 18 yrs. of age)
 With carditis/RHD- upto 40 years of age
(WHO- X 10 yrs./upto 25 yrs. of age)
 Severe RHD/after valve Sx- lifelong
 Problem- compliance
 No recurrence with proper compliance