1. ACUTE RHEUMATIC FEVER

2. ETIOLOGY
1. Immunologic Streptococcus Beta hemolytic group A 2. Predisposing factors - Family history - Socio economic status - Age years ( peak 8 years)

3. Pathogenesis pathway for ARF and RHD
Lancet 2005; 366: 155–68

4. Clinical Manifestations

5. Clinical Manifestations
MAYOR MANIFESTATION
POLYARTHRITIS MIGRAN
CARDITIS
SYDENHAM’S CHOREA (St. VITUS’ DANCE)
ERYTEMA MARGINATUM
SUBCUTANEUS NODULE

6. Clinical Manifestations
MINOR MANIFESTATION
Fever
Arthralgia
Acute-phase reactant ↑
(LED & CRP, leukocyte)
ECG showed: prolonged PR interval

7. 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

8. Diagnosis of Streptococcal Infection (cont’):
Detection of recent streptococcal infection in 61 consecutive patients
studied within 4 weeks of onset of acute rheumatic fever during 1994
and 1995.
ADNB = anti-DNase B; AHT = antihyaluronidase; ASOT = antistreptolysin-O Titer

9. The Revised Jones Criteria 1992
Highly probable
2 mayor manifestations
1 mayor + 2 minor manifestations
With proved evidence of GAS infection (culture or ASTO)
Doubtful
Without proved evidence of GAS infection

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

11. 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.

12. Echocardiography

13. Role of Echocardiography
Prevalence of Rheumatic Valvular Abnormalities
among Schoolchildren in Cambodia and Mozambique
N Engl J Med 2007;357:470-6.

14. 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

15. Prevention Ideally prophylaxis is indefinite
Benzathin Penicillin (600,000-1,200,000 U) every 28 days, min till age 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

16. Recurrence Rate of RF with different regimen
Taranta & Markowitz,1989

18. RHEUMATIC HEART DISEASE
Affects Mitral valve 75 % Aortic valve 25 % Tricuspid valve rare Pulmonary valve never Stenosis and regurgitation usually occur together

19. 2002–2003 WHO Criteria for The Diagnosis of ARF & RHD (Based on The Revised Jones Criteria)
DIAGNOSTIC CATEGORIES
CRITERIA
Primary episode of RF
2 Mayor/1 Mayor+2 Minor manifestations + evidence of a
preceding GAS infection
Recurrent attack of RF in a patient without established RHD
2 Mayor/1 Mayor+2 Minor manifestations + evidence of a
Recurrent attack of RF in a patient with established RHD
2 Minor plus evidence of a
Rheumatic chorea
Insidious onset of rheumatic carditis
Other major manifestations or evidence of GAS infection not required
Chronic valve lesions of RHD
Do not require any other criteria to be diagnosed as having RHD

20. Thank You
NO PAIN NO GAIN

21. Management of Chorea
The signs and symptoms of chorea do not respond to anti-inflammatory agents
In severe case:
Neuroleptics
Carbamazepine 7–10 mg/kg/day po tid
Phenobarbital 3–5 mg/kg/day po bid
Haloperidol 0.01–0.03 mg/kg/day po bid
Valproic acid 15–20 mg/kg/day po tid

22. Clinical manifestations. Asymptomatic during childhood
Clinical manifestations * Asymptomatic during childhood * Rare : fatigue, palpitation

23. Sign & Symptom of GAS Infection
SIGNS/SYMPTOMS
INFANT
CHILDREN
ADOLESCENT/ADULT
ANTERIOR CERVICAL LYMPHADENITIS (PAIN)
++++
CONTACT
SCARLATINIFORM RASHES +
NOSE EXCORIATION
TONSIL EXUDATE/PHARYNX
THROAT CULTURE (+)
FEVER ++
ACUTE ONSET
ABDOMINAL PAIN
CORYZA
PHARYNX ERYTHEMATOUS
HOARSENESS
COUGH

24. ECG
Normal in mild cases
LVH or LV dominance, with or without LAH
CXR
LA and LV enlarged
Pulmonary congestion pattern in CHF

25. INCIDENCE
Annual specific incidence rate (temporal trend) of first attack of ARF
Heart 2008;94;

26. Changes of JONES Criteria

27. Potential Preventive Measures for Rheumatic Fever and Rheumatic Heart Disease

28. Bed rest and indoor ambulation ____________________________________
Arthritis Mild Moderate Severe
Alone Carditis Carditis Carditis
__________________________________________________________
Bed rest wk wk wk as long as HF +
Indoor ambulation wk wk wk mo
_________________________________________________________
ESR: important for duration of restriction of activities.
Full activity : ESR normal, except significant cardiac involvement _

29. Recommended anti-inflammatory agents
_______________________________________________________________________________________
Arthritis Mild Moderate Severe
alone carditis carditis carditis
__________________________________________________
Prednisone wk*
Aspirin wk wk# wk 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

30. Subcutaneous rheumatic nodules

31. Erythema marginatum

32. Subcutaneous Nodule

33. General Measures Arthritis Mild Carditis Moderate Carditis
Severe Carditis
Bed rest
(Hospitalization)
1-2 weeks
2-3 weeks
(up to 4 weeks)
4-6 weeks
2-4 months
(CHF -)
Indoor ambulation
2-3 months
Outdoor activity
2 weeks
2-4 weeks
1-3 months
2- 3 months
Full activity
After weeks
After 3 (6-10) weeks
After 3-6 months
Variable
These guidelines should be individualized by clinician(s) according to
patient and family circumstances.

34. Suppression of The Inflammatory Process
Arthritis
Mild carditis
Moderate carditis
Severe carditis
Prednisone
2-4 weeks
2-6 weeks
Salicylates
1-2 weeks
6-8 weeks
2-4 months
Prednisone:1–2mg/kg-day, to a maximum of 80mg/day given once daily, or in divided doses). After 2–3 weeks of therapy the dosage may be decreased by 20–25% each week. While reducing the steroid dosage, a period of overlap with aspirin is recommended to prevent rebound of disease activity
Salicylates: mg/kg/day/divided into 4-5 doses for 2 weeks
60–70mg/kg-day for 3–6 weeks

35. Suggested Duration of Secondary Prophylaxis
Category of patient
Duration of prophylaxis
Patient without proven carditis
For 5 years after the last attack, or until years of age (whichever is longer)
Patient with carditis
(mild MR or healed carditis)
For 10 years after the last attack, or until years of age (whichever is longer)
More severe valvular disease
10 years or until 40 years of age
(whichever is longer), sometimes
lifelong prophylaxis
After valve surgery
Lifelong

36. 2002–2003 WHO Criteria for The Diagnosis of ARF & RHD (Based on The Revised Jones Criteria)
DIAGNOSTIC CATEGORIES
CRITERIA
Primary episode of RF
2 Mayor/1 Mayor+2 Minor manifestations + evidence of a
preceding GAS infection
Recurrent attack of RF in a patient without established RHD
2 Mayor/1 Mayor+2 Minor manifestations + evidence of a
Recurrent attack of RF in a patient with established RHD
2 Minor plus evidence of a
Rheumatic chorea
Insidious onset of rheumatic carditis
Other major manifestations or evidence of GAS infection not required
Chronic valve lesions of RHD
Do not require any other criteria to be diagnosed as having RHD

37. Erythema marginatum

38. Group A beta hemolytic Streptococcus
STRUCTURE
Capsule: hyaluronic acid
Cell Wall: outer, middle and inner layer
Outer layer: proteins M, T and R
M component is the most potent & antigenic
Middle layer: specific carbohydrates eg N acetyl glucoamin
Inner layer:peptidoglycan –responsible for cell wall rigidity
Cytoplasm

39. IMMUNOPATHOGENESIS Of RF