Acute rheumatic fever is leading cause of cardiac diseases

1. Group A strep
Pharyngitis,
Rheumatic Fever and
Heart Disease

2. Rheumatic fever is an acute, immunologically mediated,
multisystem inflammatory disease that occurs after group
A β-hemolytic streptococcal infections (usually pharyngitis, but also
occasionally infections at other sites, such as
skin). Rheumatic heartdisease is the cardiac manifestation of
rheumatic fever.

4. Cardiac surgery, stroke, death

5. Pharyngitis/Tonsillitis
• V common in children and youn
g adults
• 70% caused by viruses AND 30
% are caused by GAS

6. • The incidence of GAS pharyngitis
depends on: season, age, socioecon
omic conditions, environmental fac
tors and the quality of health care

9. Clinical Features of GAS
Pharyngitis
• Sore throat in patients 5-15 years
• No runny nose or cough
• Fever, pharyngeal exudates, cervical lymp
h node and skin rash: may be present.

10. • Pharyngitis includes
inflammation of ton
sils and paratonsilar
tissues
• Tonsillectomy does
not prevent GAS ph
aryngitis

11. Diagnosis of GAS Pharyngitis
• Definite diagnosis: throat cultur
e: Not practical, takes 3-7 days, e
xpensive
• Rapid antigen test: expensive, not relia
ble
We rely on clinical diagnosis

13. Treatment
• One injection of benzathine penicillin afte
r sensitivity test.
• Oral penicillin (10 Days)
Primary prevention of acute rheumatic
fever

14. Primary prevention of
acute rheumatic fever
Prompt diagnosis and
treatment of GAS
pharyngitis

15. Can we prevent GAS
pharyngitis?
•Vaccine research is going on
•Not yet ready

17. Acute Rheumatic Fever

19. Acute Rheumatic Fever (ARF)
• Syndrome that follows GAS
pharyngitis
• Only 0.3% of cases of patients
with GAS develop ARF.

20. ARF IS COMMON IN SUDAN
• Prevalence in Sudan 11/1000
• Egypt 5/1000
• United States 0.05/ 1000

21. World wide…
•233 000 deaths/year.
•Most affected children
developing countries:
Africa, India and similar
areas.

22. Pathogenesis
• RF is a delayed autoimmune respons
e to GAS pharyngitis.
• Clinical manifestation is determined by
:
1.Host genetic susceptibility
2.Virulence of the infecting organism
3.Environment

23. Only 0.3–3% of individuals with GAS
pharyngitis develop RF
• Pathogenetic mechanisms of ARF has not
been well defined.
• HLA antigens: Class II.
• T-cell lymphocytes: cytokines
• Particular M types of group A streptococc
i have rheumatogenic potential.

24. Genetic Mimickery
• M protein shares structural similarity
with cardiac myosin and laminin (valve
s)

25. Determinants of Disease Burden
1. Socioeconomic and environmental factors:
- Crowding
- Malnutrition
- Low awareness
2. Health system factors:
- Shortage of health care providers
- Late treatment of GAS pharyngits
- Lack of control programs

26. Clinical Manifestations

27. •5-15 years of age
•Poor socio economic
background

28. Clinical Manifestations and
Diagnosis
•The diagnosis of ARF is
clinical by application of
the Jone’s Criteria.

29. Diagnosis of ARF
• No single diagnostic test
• Depends on the Jone’s Criteria

30. Jone’s Criteria
2015 MODEFICATION
Major Criteria:
1.Flitting arthritis , monoarthritis or
polyarthrlagia
2.Carditis: clinical or subclinical (echo)
3. Chorea
4. Erythema marginatum
5. Subcutaneous nodules

31. Minor Criteria
1. Fever
2. Leukocytosis, high ESR, high
CRP.
3. Mono arthrlalgia
4. Prolonged PR interval on ECG

32. Major Criteria

33. Arthritis
• 70-75%
• Migratory/flitting
• Severe pain in Large joints
• Maximum severity in 12-24 hours, persists
for 2-6 days and resolve.
• Responds rapidly to aspirin.
• No permanent damage

34. Carditis
• Occur in 50% of cases
• Pathologically: pancarditis,Achoff
nodules seen in endocardium,
myocardium and pericardium.
• Clinically: Mainly affect valves , rarely
pericarditis
• Permanent damage

35. Valvulitis
• Inflammation of heart valves
• Mitral Regurgitation (MR) is the most
common lesion.
• Combined MR and aortic regurgitation is
the second most common
• Mitral stenosis occurs in older children

36. Clinical Features of Carditis
Symptoms:
Palpitations, shortness of breathing,
exercise intolerance, oedema
Signs:
Congestive heart failure due to valvulitis:
tachycardia, tachypnea, cardiomegaly, hepatomegaly and
raised jugular venous pressure

37. Inspection
• Heart failure: distress, high JVP
• Corrigan sign: prominent pulse at supraste
rnal notch
• Active precordium

38. Palpation
• The pulse:
Small volume: Severe MR
Large volume: Severe AR
Irregular pulse: Atrial fibrillation (MS, MR)
• The apex:
Displaced and heaving: MR
Not displaced and tapping: MS (older children)
• Apical thrills

39. Auscultation
1.Pansystolic murmur at the apex (MR)
2. Mid diastolic murmur at the apex
(Carey Coombs murmur) due to
oedema of the valve
3.Early diastolic murmur of AR.
4.Pericarditis: pericardial rub

40. Sydenham Chorea
• 10-30%.
• Involuntary, purposeless movements.
emotional lability. Hyperextended joints,
hypotonia, diminished reflexes
• Tongue fasciculations "bag of worms"),
• relapsing grip. "milk sign"

41. • More common in females.
• improvement in 1-2 weeks and full
recovery in 2-3 months

42. Erythema Marginatum
• Erythema annulare
• 5-13%
• Pink-to-red nonpruritic
macules or papules on
trunk and proximal limbs
but never on the face.
• Exacerbated by heat
• Sepsis, drug reactions,
glomerulonephritis.

43. Subcutaneous Nodules
• Infrequent: 0-8%
• Extensor surfaces.
• Firm, nontender, not attached to the
skin.

44. Rare manifestations
• Pediatric autoimmune neuropsychiatric
disorders associated with streptococcal
infections (PANDAS)
• obsessive-compulsive disorder , tic
,cognitive defects and motor hyperactivity
• Pneumonia, pleural effusion
• Epistaxis

46. Diagnosis: For first episode
OR

47. Diagnosis: recurrent episode

48. Diagnosis: Probable ARF
• Criteria less than required in end
emic areas

49. 3 DIGNOSTIC CATEGORIES

50. Evidence of streptococcal infection
•High or rising ASO titre.
• Positive throat swab or rapid
streptococcal antigen test
• Clinically documented GAS

51. Exceptions to the Criteria:
1.Chorea
2.Recurrent attacks.

52. Management of ARF

53. 1. Eliminating GAS: Benzathine
Penicillin(patients allergic to penicillin:
erythromycin).
2. Suppressing inflammation :Aspirin in
anti-inflammatory doses (75-
100mg/kg/d) for 4-6 weeks
3. Supportive ttt

54. Supportive ttt
• Bed rest 4-6 weeks.
• Treatment of congestive heart
failure.

55. Indications for Steroids
• Carditis not responsive to
antifailure treatment.
• Patients not tolerating aspirin
• Oral prednisone 2 mg/kg/d for 2-
4 weeks

56. Indication for Surgery
• Valvular regurgitation not
responsive to medical treatment
• Chronic significant valve lesions
• Valve repair is preferred if not
possible valve replacement.

58. RHD
• ARF can lead to permanent he
art valve damage that cannot b
e treated
• All efforts should be made to pre
vent ARF before it affects the he
art

59. Types of RHD
Subclinical: Diagnosed by echo

60. Rheumatic MV Normal MV
‘ARF is a disease that licks the
joints and bites the heart’
Lesegues 1885

62. Mitral Regurg
• Heart Murmur: Pansyst
olic apical radiate to axi
lla
• CHF

63. Aortic Regur
• Early diastolic murm
ur in A2
• Functional systolic m
urmur

64. Mitral Stenosis

65. Decompensation

66. Management
•Medical:
Heart failure ttt
• Surgical : valve rep
air or replacement
• Catheter: balloon
dilatation of MS

67. Prognosis
• V POOR outcome of RHD: Follow up rate
30%, Mortality 14%
• Post op: need to continue anti coagulatio
n FOR LIFE
• Poor compliance

68. RHD Control Program
• World wide effort
• Sudan’s program

69. Levels of Prevention of RHD

70. SUR I CAAN!

71. Integration in
MOH
Advocacy
Training
Awareness
Collaboration
Surveillance
SUR I CAAN

72. Public Awareness

73. Clinical and Echo Data
• Early detection
• Echo screening
• Registries: Manual or
electronic

75. Kordofan RHD Control Center

76. Thanks