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Dr. D. Gunasekaran
Consultant in Paediatrics
Aetiology
Clinical features
Diagnosis
Investigations
Management
Prevention
Introduction
It is a Systemic disease involving 4 organs..
Joints, heart, brain and skin
Sequelae in which organ?
Heart
Ag...
Introduction
Predisposing factors?
Low socio-economic status, Overcrowding,
Poverty, Under nutrition & Poor hygiene
Geneti...
What infection?
Group A Beta hemolytic Streptococci
Throat or skin?
Throat and not skin. Why?
AB production is not intense...
What strains?
Serotypes: M 1,3,5,6,18 & 24
Time interval?
1-5 weeks (3 weeks)
Untreated throat infection; risk is?
0.3 – 3%
When to diagnose Streptococcal throat infection:
 For
 Sudden onset of fever, throat pain
 Pharyngo-tonsillar inflammat...
Pathogenesis
How Group A Streptococci infection of throat
predisposes the child to develop
acute rheumatic fever?
 Theori...
Pathogenesis of ARF
Streptococcal
components
Human tissues
M Protein Heart muscle proteins & brain proteins
Cell wall
Poly...
Name of the criteria?
Whether it applies only for first attack?
How many major & minor criteria?
Modified Jones Criteria
I) Major Criteria:
Incidence:
a) Polyarthritis – 75%
b) Carditis- 50%
c) Chorea (15%)
d) Subcutane...
I) a) Poly arthritis (75%)
Most common (75%) & earliest
Major joint (knee, ankle, elbow, etc) {Cervical &
Hip are not invo...
I) b) Pancarditis (50%)
• Most serious
• Earliest – within 2 weeks
• Clinical: 50%; ECHO evidence in 90%
• Common in Recur...
I) b) Pancarditis
Pericarditis:-
• Chest pain
• Pericardial rub (superficial grating)
• Pericardial effusion (sounds less ...
I) b) Pancarditis
Myocarditis
• Tachycardia
(Out of proportion to fever; no reduction during sleep)
• Soft S1
• S3 gallop
...
I) b) Pancarditis
Endocarditis- Valvulitis:-
Which valve?
Mitral V < Aortic V < Tricuspid V < Pulmonary V
(95% M in which ...
I) b) Pancarditis
Severity of Carditis:-
 Mild: Tachycardia / Murmur
 Moderate: Cardiomegaly
 Severe: CCF / Pericarditi...
I) b) Pancarditis
 70% of carditis recover without residual sequelae
 CC murmur disappears once carditis resolves
 Almo...
Severe carditis
After treatment
I) c) Sydenham’s Chorea (St. Vitus Dance)
15%; late (>3 months)
Common in girls 8-12 years
Caudate nucl involvement
Earlie...
I) c) Sydenham’s Chorea (St. Vitus Dance)
Signs:
 Milk maid grip
 Dinner fork deformity
 Pronator sign
 Jack in the bo...
I) c) Sydenham’s Chorea (St. Vitus Dance)
Fate of Chorea:
 Self-limiting (6 months – 18 months)
 1/3 will develop RHD
I) d) Subcutaneous nodule
I) d) Subcutaneous nodules (2 – 10%)
• Over the extensor aspects of bony prominences,
back, occiput & mastoid
0.5 -2 cm, p...
I) e) Erythema marginatum
I) e) Erythema marginatum
I) e) Erythema marginatum (<3%)
o Early manifestation
o Transient; not appreciated in dark skin people
o Eythematous, serp...
II) Minor manifestations
 Clinical features
Fever (>102° F)
Arthralgia
 Laboratory features
Acute phase reactants:
ESR >...
III) Supportive evidence is a must
 Organism: Positive throat C/S (Not useful)
 Antigen: Rapid streptococcal antigen tes...
To make a clinical diagnosis of ARF
2 major
Or1 major + 2 minor
With Supportive evidence
(ASO / anti DNAse)
For whom this criteria need not be followed?
Chorea
Indolent Carditis
Recurrence RF
Differential diagnosis
 Arthritis
 Rheumatoid arthritis
 Reactive – salmonella, Shigella
 Sickle cell disease
 SLE, M...
Differential diagnosis
Rheumatic Fever Arthritis Rheumatoid Arthritis
Major Joints Minor Joints
Migrating, Asymmetrical Sy...
Differential diagnosis
 Chorea
 Wilson disease
 Cerebral palsy
 Tics
 Huntigton chorea
Treatment
1. Bed rest
2. Treatment of the GAS infection in throat
3. Anti Inflammatory therapy
4. Treatment of Chorea
5. M...
Treatment
1. Bed rest
Until ESR becomes normal
Until CCF is under control
Treatment
2. Treatment of GAS infection in throat: - Antibiotics
(antigens are removed antibodies formation is
reduced  ...
3. Treatment -Anti-Inflammatory therapy
a)Arthritis & carditis without cardiomegaly or CCF: Salicylates
75 mg/kg/day for 1...
Treatment
4. Congestive Cardiac Failure:
a) Bed rest
b) Salt restricted diet
c) O2
d) Diuretics
e) Digoxin
f) ACE inhibito...
Treatment
5. Chorea:
Diazepam, Phenobarbitone, Haloperidol,
Chlorpromazine
PREVENTION OF RHEUMATIC FEVER
Primary prevention
Prevention of initial attacks of ARF
o Community education regarding the
consequences of throat infecti...
Secondary prevention (Prophylaxis)
Prevention of repeated throat infections in a child
who has had 1 episodes RF
How it is...
Secondary prevention
What drug?
Best:
Inj Benzathine Penicillin IM
(0.6 mega units <27kg; 1.2mega units
>27kg). Every 4 we...
Secondary Prevention
How long?– AHA recommendation
No carditis 5 years or
until 21 years of age,
whichever is longer
Cardi...
Acute rheumatic fever
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Acute rheumatic fever

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Acute rheumatic fever

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Acute rheumatic fever

  1. 1. Dr. D. Gunasekaran Consultant in Paediatrics
  2. 2. Aetiology Clinical features Diagnosis Investigations Management Prevention
  3. 3. Introduction It is a Systemic disease involving 4 organs.. Joints, heart, brain and skin Sequelae in which organ? Heart Age? 5-15 years; Peak at 8 years Sex? Male = Female; Chorea is common in adolescent girls
  4. 4. Introduction Predisposing factors? Low socio-economic status, Overcrowding, Poverty, Under nutrition & Poor hygiene Genetic predisposition? HLA DR3 + : risk is more Serum 883-B cell Allo antigen: 12 times more risk.
  5. 5. What infection? Group A Beta hemolytic Streptococci Throat or skin? Throat and not skin. Why? AB production is not intense; neutralization of toxins by skin lipids
  6. 6. What strains? Serotypes: M 1,3,5,6,18 & 24 Time interval? 1-5 weeks (3 weeks) Untreated throat infection; risk is? 0.3 – 3%
  7. 7. When to diagnose Streptococcal throat infection:  For  Sudden onset of fever, throat pain  Pharyngo-tonsillar inflammation  Cervical lymphadenopathy  Against  Coryza, cough, conjunctivitis, hoarseness
  8. 8. Pathogenesis How Group A Streptococci infection of throat predisposes the child to develop acute rheumatic fever?  Theories Immune mimicry – Antibodies to streptococcal bacterial components cross react with tissues of heart, brain and joints
  9. 9. Pathogenesis of ARF Streptococcal components Human tissues M Protein Heart muscle proteins & brain proteins Cell wall Polysaccharide Glycoproteins of heart valves Cytoplasmic membrane Cytoplasm of subthalamic nulceus & caudate nucleus
  10. 10. Name of the criteria? Whether it applies only for first attack? How many major & minor criteria?
  11. 11. Modified Jones Criteria I) Major Criteria: Incidence: a) Polyarthritis – 75% b) Carditis- 50% c) Chorea (15%) d) Subcutaneous nodules (2-10%) 25% e) Erythema marginatum (<3%)
  12. 12. I) a) Poly arthritis (75%) Most common (75%) & earliest Major joint (knee, ankle, elbow, etc) {Cervical & Hip are not involved} Migratory and hence asymmetrical Dramatic response to salicylates in two days Heals in 2 weeks even without drugs Leaves no residue
  13. 13. I) b) Pancarditis (50%) • Most serious • Earliest – within 2 weeks • Clinical: 50%; ECHO evidence in 90% • Common in Recurrent RF • Pancarditis – peri, myo, endo
  14. 14. I) b) Pancarditis Pericarditis:- • Chest pain • Pericardial rub (superficial grating) • Pericardial effusion (sounds less distinct) • CXR: Cardiomegaly • ECG: Inverted T; reduced QRS voltages
  15. 15. I) b) Pancarditis Myocarditis • Tachycardia (Out of proportion to fever; no reduction during sleep) • Soft S1 • S3 gallop • Cardiomegaly • CCF- left heart failure- pulmonary edema
  16. 16. I) b) Pancarditis Endocarditis- Valvulitis:- Which valve? Mitral V < Aortic V < Tricuspid V < Pulmonary V (95% M in which 25% A; 5% pure A) Murmur must be + Carey-Coomb’s murmur - delayed diastolic murmur (Valvulitis with swelling of the cusps and reduction in size of valve orifice) Pansystolic murmur of MR (Loss of valvular structure & shortening of Chordae tendinae) Early diastolic murmur of AR
  17. 17. I) b) Pancarditis Severity of Carditis:-  Mild: Tachycardia / Murmur  Moderate: Cardiomegaly  Severe: CCF / Pericarditis Minimum criteria to diagnose Carditis clinically: Tachycardia and murmur
  18. 18. I) b) Pancarditis  70% of carditis recover without residual sequelae  CC murmur disappears once carditis resolves  Almost 50% of MR murmur disappears in 1 year
  19. 19. Severe carditis
  20. 20. After treatment
  21. 21. I) c) Sydenham’s Chorea (St. Vitus Dance) 15%; late (>3 months) Common in girls 8-12 years Caudate nucl involvement Earliest: Emotional lability, incoordination, poor school performance Latter choreic movement: Involuntary, rapid, irregular, jerky, non-repetitive, quasipurposive movements, involving face, trunk and distal extremities. Exacerbated by stress; Disappear during sleep
  22. 22. I) c) Sydenham’s Chorea (St. Vitus Dance) Signs:  Milk maid grip  Dinner fork deformity  Pronator sign  Jack in the box tongue  Abnormal handwriting
  23. 23. I) c) Sydenham’s Chorea (St. Vitus Dance) Fate of Chorea:  Self-limiting (6 months – 18 months)  1/3 will develop RHD
  24. 24. I) d) Subcutaneous nodule
  25. 25. I) d) Subcutaneous nodules (2 – 10%) • Over the extensor aspects of bony prominences, back, occiput & mastoid 0.5 -2 cm, painless • Almost always associated with carditis • Can occur in JRA, SLE
  26. 26. I) e) Erythema marginatum
  27. 27. I) e) Erythema marginatum
  28. 28. I) e) Erythema marginatum (<3%) o Early manifestation o Transient; not appreciated in dark skin people o Eythematous, serpiginous lesions o Trunk, extremities o Accentuated by warming o Can occur in any Streptococcal infection (not specific for ARF alone)
  29. 29. II) Minor manifestations  Clinical features Fever (>102° F) Arthralgia  Laboratory features Acute phase reactants: ESR >30 mm /1 hour; CRP >6mgs% Prolonged PR interval (>0.2 sec)
  30. 30. III) Supportive evidence is a must  Organism: Positive throat C/S (Not useful)  Antigen: Rapid streptococcal antigen test (NA)  Antibody titers: (Commonly done) a) ASO titer (Child: >320) (1 week 1month2-4 months normal) Usually + in 85% of ARF Response is blunted with Antibiotics & Steroids b) Anti DNAse: 1-2 monthslasts longer c) Anti streptokinase, Anti hyauloronidase d) Streptozyme test –slide agglutination; not standardized
  31. 31. To make a clinical diagnosis of ARF 2 major Or1 major + 2 minor With Supportive evidence (ASO / anti DNAse)
  32. 32. For whom this criteria need not be followed? Chorea Indolent Carditis Recurrence RF
  33. 33. Differential diagnosis  Arthritis  Rheumatoid arthritis  Reactive – salmonella, Shigella  Sickle cell disease  SLE, Malignancy  Carditis  Viral myocarditis,  Infective endocarditis  Kawasaki disease  Congenital heart disease
  34. 34. Differential diagnosis Rheumatic Fever Arthritis Rheumatoid Arthritis Major Joints Minor Joints Migrating, Asymmetrical Symmetrical Morning stiffness - Morning stiffness + TMJ involvement - TMJ involvement + Dramatic response to salicylates Not so Even without treatment, disappears in 2 weeks Not so
  35. 35. Differential diagnosis  Chorea  Wilson disease  Cerebral palsy  Tics  Huntigton chorea
  36. 36. Treatment 1. Bed rest 2. Treatment of the GAS infection in throat 3. Anti Inflammatory therapy 4. Treatment of Chorea 5. Management of Complications 6. Prophylaxis – Secondary
  37. 37. Treatment 1. Bed rest Until ESR becomes normal Until CCF is under control
  38. 38. Treatment 2. Treatment of GAS infection in throat: - Antibiotics (antigens are removed antibodies formation is reduced  further damage is reduced) a) Single dose of Benzathine Penicillin IM (0.6 million units <27kg; 1.2 million units >27 kg) b) Penicillin V 250mg 1qid for 10 days c) Erythromycin 40mg/kg/day for 10 days
  39. 39. 3. Treatment -Anti-Inflammatory therapy a)Arthritis & carditis without cardiomegaly or CCF: Salicylates 75 mg/kg/day for 1 week 50 mg/kg/day for 3 weeks 25 mg/kg/day for 3 weeks b)Carditis with cardiomegaly or CCF: Prednisolone 2 mg/kg/day for 3 weeks 1 mg/kg/day for 3 weeks Taper 5mg every 3 days stop When tapering is begun, add Salicylates 50 mg/kg/day for 6 weeks.
  40. 40. Treatment 4. Congestive Cardiac Failure: a) Bed rest b) Salt restricted diet c) O2 d) Diuretics e) Digoxin f) ACE inhibitors (when valvular regurgitation +)
  41. 41. Treatment 5. Chorea: Diazepam, Phenobarbitone, Haloperidol, Chlorpromazine
  42. 42. PREVENTION OF RHEUMATIC FEVER
  43. 43. Primary prevention Prevention of initial attacks of ARF o Community education regarding the consequences of throat infection o Antibiotics started before 9th day of throat infection prevents 1st attacks of rheumatic fever
  44. 44. Secondary prevention (Prophylaxis) Prevention of repeated throat infections in a child who has had 1 episodes RF How it is done? Continuous antibiotic prophylaxis for many years Why it is necessary? With each throat infection, there is increased risk of rheumatic fever recurrence
  45. 45. Secondary prevention What drug? Best: Inj Benzathine Penicillin IM (0.6 mega units <27kg; 1.2mega units >27kg). Every 4 weeks (in high risk areas: once in 3 weeks) Alternatives: T. Penicillin V 250 mg 1bd daily (efficacy is 10 times less) Others- Erythromycin, Sulfadiazine
  46. 46. Secondary Prevention How long?– AHA recommendation No carditis 5 years or until 21 years of age, whichever is longer Carditis but without residual heart disease 10 years or until 21 years of age whichever is longer Carditis with residual heart disease 10 years or until 40 years of age whichever is longer

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