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Acute Rheumatic Fever
Introduction
 Non-infectious, post-infection inflammatory disease
affecting primarily the heart, joints, subcutaneous
tissues, and CNS
 Occurs as a delayed complication of infection with
certain serotypes of Group A Streptococci
Epidemiology
 Burden: 10-50% of cardiac admission
 Incidence of rheumatic fever following streptococcal
sore throat: 0.3% in western
 Increased to 1-3% in crowded communities
Epidemiology
 Most common age group: 5-15 years
 Sex: nearly equally affected
 Role of
 Overcrowding
 Poverty
Etiopathogenesis
 Incompletely understood
 Strong association with β-hemolytic streptococci
 Post-infectious sequelae
 Autoimmune response
 Genetic susceptibility
Clinical features
 Pancarditis: [50-60% patients]
 Early manifestation
 Occur within first 2 weeks
 Pericarditis
 Precordial pain
 Friction rub
 Associated with mitral or aortic regurgitation murmurs
 Myocarditis
 Cardiomegaly
 Soft S1
 S3 gallop
 CCF
 Carey Coomb’s murmur
Clinical features
 Pancarditis (cont’d………..)
 Endocarditis
 Mitral valve: 100% patients with carditis
 Only Aortic valve: 5-8%
 Tricuspid valve: 10-30%
 Pulmonary valve: Not seen
 Results in regurgitation murmur
 Volume overload left ventricular failure
Clinical features
 Polyarthritis
 Migratory
 Large joints: knees, ankles, elbows
 Early feature
 30-50% of patients [70-75% in western countries]
 Transient
 Tenderness may be out of proportion to the objective findings
and severe enough to result in excruciating pain on touch
 Response to salicylates: very good
 No residual damage to joints
 “licks joints, but bites heart”
Clinical features
 Subcutaneous nodules
 Late manifestation: about 6 weeks
 Occurs in <1% patients
 Over bony prominences: elbows, shin, occiput, spine
 Pinhead to almond size
 Painless
 Lasts for few days to weeks
 Strong association with carditis
Clinical features
 Sydenham’s chorea
 Late manifestation
 10-15% patients
 Occurs after about 3 months
 Purposeless jerky movements, deranged speech, muscular
weakness, gait abnormality
 Emotional lability:
 More frequent in females
 Self-limiting
Clinical features
 Sydenham’s chorea
 Milkmaid’s grip
 Spooning and pronation of hands when extended
 Wormian movements of tongue when protruded
Clinical features
 Erythema marginatum
 Early
 Rare (<3%)
 Starts as red spot with pale center
 Increase in size to coalesce with
adjacents spots to form
serpentiginous outline
 Faint red, not raised above the skin,
non pruritic
 Predominantly seen over trunks
 Evanescent
Diagnosis
 Modified Jones criteria
 Major criteria
 Carditis
 Arthritis
 Subcutaneous nodules
 Erythema marginatum
 Minor criteria
 Clinical
 Fever
 Arthralgia [in absence of
arthritis]
 Laboratory
 Raised ESR, CRP
 Prolonged PR interval
 Essential criteria
 Evidence of recent
streptococcal infection
 Elevated ASO titre
 Positive throat
culture
Diagnosis
 2 major criteria
OR
 1 major criteria and 2 minor criteria
AND
 Presence of recent streptococcal infection
 Exceptions
 Chorea
 Indolent carditis
Treatment
 Supportive treatment
 Bed rest
 Penicillin
 Procaine penicillin daily for 10 days
 Single dose of Benzathine penicillin
 Suppressive therapy
 Aspirin
 Steroids
 Prophylaxis
Treatment
 Aspirin or Steroids?
 Presence of carditis: STEROID
 Caridits without CCF: Steroids preferred
 Absence of steroids: Aspirin preferable
 Duration of suppressive therapy: 12 weeks
Prevention
 Primary prevention
 Identification of streptococcal pharyngitis and effective
treatment
Prevention
 Secondary prevention
 Benzathine penicillin 12 lakh units IM once every 3 weeks
 Erythromycin
 Penicillin V
Prevention
 Secondary prevention
 Duration
 No carditis: 5 years/18yrs of age, whichever is longer
 Mild to moderate carditis and healed carditis: 10 yrs/25 yrs of age,
whichever is longer
 Severe disease or post intervention patients: Life long [One may
opt for secondary prophylaxis up to the age of 40 years]
Querries

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Acute rheumatic fever

  • 2. Introduction  Non-infectious, post-infection inflammatory disease affecting primarily the heart, joints, subcutaneous tissues, and CNS  Occurs as a delayed complication of infection with certain serotypes of Group A Streptococci
  • 3. Epidemiology  Burden: 10-50% of cardiac admission  Incidence of rheumatic fever following streptococcal sore throat: 0.3% in western  Increased to 1-3% in crowded communities
  • 4. Epidemiology  Most common age group: 5-15 years  Sex: nearly equally affected  Role of  Overcrowding  Poverty
  • 5. Etiopathogenesis  Incompletely understood  Strong association with β-hemolytic streptococci  Post-infectious sequelae  Autoimmune response  Genetic susceptibility
  • 6. Clinical features  Pancarditis: [50-60% patients]  Early manifestation  Occur within first 2 weeks  Pericarditis  Precordial pain  Friction rub  Associated with mitral or aortic regurgitation murmurs  Myocarditis  Cardiomegaly  Soft S1  S3 gallop  CCF  Carey Coomb’s murmur
  • 7. Clinical features  Pancarditis (cont’d………..)  Endocarditis  Mitral valve: 100% patients with carditis  Only Aortic valve: 5-8%  Tricuspid valve: 10-30%  Pulmonary valve: Not seen  Results in regurgitation murmur  Volume overload left ventricular failure
  • 8. Clinical features  Polyarthritis  Migratory  Large joints: knees, ankles, elbows  Early feature  30-50% of patients [70-75% in western countries]  Transient  Tenderness may be out of proportion to the objective findings and severe enough to result in excruciating pain on touch  Response to salicylates: very good  No residual damage to joints  “licks joints, but bites heart”
  • 9. Clinical features  Subcutaneous nodules  Late manifestation: about 6 weeks  Occurs in <1% patients  Over bony prominences: elbows, shin, occiput, spine  Pinhead to almond size  Painless  Lasts for few days to weeks  Strong association with carditis
  • 10. Clinical features  Sydenham’s chorea  Late manifestation  10-15% patients  Occurs after about 3 months  Purposeless jerky movements, deranged speech, muscular weakness, gait abnormality  Emotional lability:  More frequent in females  Self-limiting
  • 11. Clinical features  Sydenham’s chorea  Milkmaid’s grip  Spooning and pronation of hands when extended  Wormian movements of tongue when protruded
  • 12. Clinical features  Erythema marginatum  Early  Rare (<3%)  Starts as red spot with pale center  Increase in size to coalesce with adjacents spots to form serpentiginous outline  Faint red, not raised above the skin, non pruritic  Predominantly seen over trunks  Evanescent
  • 13. Diagnosis  Modified Jones criteria  Major criteria  Carditis  Arthritis  Subcutaneous nodules  Erythema marginatum  Minor criteria  Clinical  Fever  Arthralgia [in absence of arthritis]  Laboratory  Raised ESR, CRP  Prolonged PR interval  Essential criteria  Evidence of recent streptococcal infection  Elevated ASO titre  Positive throat culture
  • 14. Diagnosis  2 major criteria OR  1 major criteria and 2 minor criteria AND  Presence of recent streptococcal infection  Exceptions  Chorea  Indolent carditis
  • 15. Treatment  Supportive treatment  Bed rest  Penicillin  Procaine penicillin daily for 10 days  Single dose of Benzathine penicillin  Suppressive therapy  Aspirin  Steroids  Prophylaxis
  • 16. Treatment  Aspirin or Steroids?  Presence of carditis: STEROID  Caridits without CCF: Steroids preferred  Absence of steroids: Aspirin preferable  Duration of suppressive therapy: 12 weeks
  • 17. Prevention  Primary prevention  Identification of streptococcal pharyngitis and effective treatment
  • 18. Prevention  Secondary prevention  Benzathine penicillin 12 lakh units IM once every 3 weeks  Erythromycin  Penicillin V
  • 19. Prevention  Secondary prevention  Duration  No carditis: 5 years/18yrs of age, whichever is longer  Mild to moderate carditis and healed carditis: 10 yrs/25 yrs of age, whichever is longer  Severe disease or post intervention patients: Life long [One may opt for secondary prophylaxis up to the age of 40 years]

Editor's Notes

  1. Number of lesions vary: single to dozen Elbows more frequent site
  2. Movements: more commonly more marked on one side and cease during sleep Emotional: outbursts of inappropriate behavior Diffuse hypotonia
  3. ASO titre in 80% ASO+antiDNAse >90% Throat culture positivity in 25%