Acute rheumatic fever

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a problem of the heart still seen among young in developing countries

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Acute rheumatic fever

  1. 1. Acute rheumatic fever Inflammatory disease following group A β-hemolytic streptococcal infection, due to cross-reactivity of antigens
  2. 2. Epidemiology  Onset- ~2-3 weeks after streptococcal infection  Rate of development after untreated infection- ~3%  Recurrence with a subsequent untreated infection- 5-50%, more in patients with RHD  Common in children- age 5-15, only 20% first attacks in adults
  3. 3. Pathogenesis  Possible autoimmune disease  Follows pharyngitis due to encapsulated GABH streptococci  A complex interplay of genetically determined host susceptibility, pathogenic GABH streptococcal infection, in a susceptible environment  Best defined virulence factor- M protein  Present on surface of bacteria  Promotes bacterial adherence & resist phagocytosis  Shares homology with cardiac myosin, tropomyosin, keratin, laminin  Types 1,3,5,6,14,18,19,24 associated with ARF  Other-  T cell activation by streptococcal superantigens, leading to granuloma formation
  4. 4. Pathology  Myocardial Aschoff body- a submiliary granuloma, later forms scar  Endocardial verrucous valvulitis, heals with fibrous thickening & adhesions, causing stenosis or regurgitation  Serofibrinous pericarditis  Joint- exudative arthritis  Subcutaneous nodules- granuloma
  5. 5. Modified Jones’ criteria  Used for diagnosis  2 major or 1 major+2 minor or 2 minor (in patient with RHD), with evidence of streptococcal infection  Evidence of s’coccal infection within last 45 days-  Elevated/rising ASO/anti-DNase B titres  Positive throat culture  Rapid Ag test for GABH s’cocci  Recent scarlet fever
  6. 6. Jones’ criteria  Major  Migratory polyarthritis, involves large joints  Carditis- pancarditis  Subcutaneous nodules- painless, extensor  Erythema marginatum- over trunk/arms  Sydenham’s chorea  Minor  Fever  Arthralgia  Raised ESR/CRP  ECG- heart-block- prolonged PR interval  Previous e/o rheumatic fever
  7. 7. Acute carditis  New murmur or change in pre-existing murmur  Apical pansystolic murmur- MR ± Carey Coomb murmur-apical MDM  Basal early diastolic murmur- AR  Tachycardia  Soft heart sounds  New onset CHF- gallop rhythm- S3  Pericardial rub or effusion  Cardiomegaly Within 6 months of acute streptococcal infection
  8. 8. Course 90% attacks subside within 12 weeks <5% persist >6 months
  9. 9. Recurrence With new M-type streptococcal infection Most common within first 5 years Frequency decreases with time
  10. 10. GABH streptococcal pharyngitis Age <15 years, high-grade fever, tonsillar swelling/exudate, tender anterior Cxal LNE, absence of cough
  11. 11. Treatment  Acute streptococcal infection- 1.2 million units of benzathine penicillin G or amoxycillin/1st gen. oral cephalosporin x 10 days  Erythromycin, 500 BD x 10 days, if penicillin sensitive/allergic  Acute arthritis- ASA/NSAIDs  Acute carditis- prednisolone, rest, diuretics, ACEI  Chorea- diazepam, haloperidol, carbamazepine Monitor ESR/CRP for duration of symptomatic Rx
  12. 12. Prophylaxis  1.2 million units of benzathine penicillin G, IM q 3-4 weeks  Sulfadiazine, 500 BD or Erythromycin, 250 BD, if penicillin sensitive  Duration-  Without carditis- X 10 years or upto 21 years of age, whichever is longer (WHO- x 5 yrs./upto 18 yrs. of age)  With carditis/RHD- upto 40 years of age (WHO- X 10 yrs./upto 25 yrs. of age)  Severe RHD/after valve Sx- lifelong  Problem- compliance  No recurrence with proper compliance

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