Acute Rheumatic Fever

Acute Rheumatic Fever

It is an immunological disorder which occurs when antibiotics produced against streptococcal cell wall proteins & sugars react with connective tissue of the body

Causative Organism

Group A beta hemolytic Streptococci (GABS)

Epidemiology

  • Age – 5 – 15 years
  • Sex – Equally affects both sexes
  • Predisposing factors – Poor socio-economic conditions, unhygienic living conditions & overcrowding

Pathogenesis

ARF Pathogenesis

  • GABS Pharyngitis → Antibiotics formed against streptococcal antigen → Molecular Mimicry between M Protein of GABS & Myosin in Myocardium → Antibodies attach to the valves of heart
  • Latent period between sore throat and clinical manifestations is 10 days – several weeks
  • Aschoff Bodies are pathognomic of Acute Rheumatic Fever (ARF)

Clinical Features

Major criteria

Arthritis

Early manifestation.

Characteristics

  • Migratory polyarthritis
  • Involves large joints (ankle, knee, wrist, elbow)
  • There is swelling, pain, warmth & tenderness associated
  • Responds well to salicylates

Carditis

90% of the cases, an irreversible condition. Early manifestation (within 2 weeks of onset).

Characteristics

  • Pancarditis – Pain in precordial area
  • Pericarditis – Pericardial rub and effusion, ECHO shows ST & T changes
  • Myocarditis – Resting tachycardia, Soft S1, S3 gallop seen
  • Endocarditis –
      • Mitral systolic murmur (Pan systolic murmur of mitral regurgitation)
      • Carey Coombs (Mitral diastolic) murmur heard
      • Aortic diastolic murmur
      • Tricuspid regurgitation may also be present

Chorea

Late Onset after 6 months

Characteristics

  • Syndeham’s Chorea or St. Vitus Dance
  • Semi purposive, involuntary movements with jerky speech
  • Affects girls more. Affected child are emotionally labile
  • Pronator & Spooning sign, Darting tongue, Milk maids grip

Subcutaneous Nodules

Early Manifestation

Characteristics

  • Hard, painless, freely mobile nodules present on extensor aspects of bony prominences, associated with carditis

Erythema Marginatum

Early Manifestation

  • Over the trunk, non- itching.
  • Starts as a red spot with pale center
  • Coalesce with adjacent spots to form a serpiginous outline
Minor
  • Fever – >38° C
  • Arthralgia –  Subjective Pain
Essential

Evidence of recent streptococcal infection

Characteristics

  • ASO titre that goes up to 250U/dl
  • Positive throat culture

Investigation

  • Acute Phase Reactants
    • Leukocyte count – 10,000 – 15,000 / mm³
    • Elevated ESR >30 mm/hr
    • Elevated CRP (subsides with steroids) > 3 mg/dl
  • ECG – Prolonged PR > 0.16s
  • Previous Streptococcal Infection
    • Anti Streptolysin O Antibody
    • Anti DNase B
    • Throat culture
  • Chest X-Ray – To see cardiomegaly
  • ECHO – To assess status of valves

Treatment

  1. Bed Rest
  2. Penicillin –
    • 1 Inj. benzathine penicillin ( < 30 kg = 6,00,000 U, ≥30 kg = 12,00,000 U)
    • Or, Penicillin V 250mg orally QiD x 10 days
    • Or, for Penicillin allergy, Erythromycin 250mg orally QiD x 10 days
  3. Suppressive therapy –
    • 12 weeks therapy
    • Carditis with congestive cardiac failure – Corticosteroids
    • Carditis without congestive cardiac failure – Aspirin or Corticosteroids
    • No Carditis – Aspirin
    • Aspirin – 90-120 mg / kg daily x 10 weeks then tapered for next 2 weeks
    • Prednisolone – 2 mg/ kg daily x 3 weeks then tapered for next 9 weeks
  4. Surgical replacement of aortic or mitral valve indicated in severe carditis.

Prevention

  1. Primary –
    • Prompt identification of sore throat
    • Rapid confirmation of streptococcal etiology
    • Availability of penicillin
  2. Secondary –
    • Duration of Secondary Prophylaxis –
      • No Carditis – 5 yr / 25 yr of age, whichever is longer
      • Mild to Moderate & Healed carditis – 10 yr/ 25 yr of age, whichever is longer
      • Severe disease or post intervention patients – Lifelong. One may opt for secondary prophylaxis up to age of 40 years.
  3. Regimen
    • Penicillin
      • 2 Inj. benzathine penicillin. Interval of 3-4 weeks required between 2 doses ( < 30 kg = 6,00,000 U, ≥30 kg = 12,00,000 U)
      • Or, Penicillin V 250mg orally BD
      • Or, for Penicillin allergy, Erythromycin 250mg orally BD
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