Rheumatic fever


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Rheumatic fever

  1. 1. Dr.Said Alavi MD,DCH,DNB,FCPS Dept. of Pediatrics and Neonatology Saqr Hospital,Ras Al Khaimah UNITED ARAB EMIRATES E-mail: drsaid@emirates.net.ae
  2. 2. Objectives Etiology Epidemiology Pathogenesis Pathologic lesions Clinical manifestations & Laboratory findings Diagnosis & Differential diagnosis Treatment & Prevention Prognosis References05/05/1999 2 Dr.Said Alavi
  3. 3. Etiology Acute rheumatic fever is a systemic disease of childhood,often recurrent that follows group A beta hemolytic streptococcal infection It is a delayed non-suppurative sequelae to URTI with GABH streptococci. It is a diffuse inflammatory disease of connective tissue,primarily involving heart,blood vessels,joints, subcut.tissue and CNS05/05/1999 3 Dr.Said Alavi
  4. 4. Epidemiology Ages 5-15 yrs are most susceptible Rare <3 yrs Girls>boys Common in 3rd world countries Environmental factors-- over crowding, poor sanitation, poverty, Incidence more during fall ,winter & early spring05/05/1999 4 Dr.Said Alavi
  5. 5. Pathogenesis Delayed immune response to infection with group.A beta hemolytic streptococci. After a latent period of 1-3 weeks, antibody induced immunological damage occur to heart valves,joints, subcutaneous tissue & basal ganglia of brain05/05/1999 5 Dr.Said Alavi
  6. 6. Group A Beta Hemolytic Streptococcus Strains that produces rheumatic fever - M types l, 3, 5, 6,18 & 24 Pharyngitis- produced by GABHS can lead to- acute rheumatic fever , rheumatic heart disease & post strept. Glomerulonepritis Skin infection- produced by GABHS leads to post streptococcal glomerulo nephritis only. It will not result in Rh.Fever or carditis as skin lipid cholesterol inhibit antigenicity05/05/1999 6 Dr.Said Alavi
  7. 7. Diagrammatic structure of the group Abeta hemolytic streptococcus Capsule Antigen of outer protein cell wall Cell wall of GABHS induces antibody Protein antigens response in victim which Group carbohydrate result in autoimmune Peptidoglycan damage to heart valves, Cyto.membrane sub cutaneous tissue,tendons, Cytoplasm joints & basal ganglia of brain…………………………………………………...05/05/1999 7 Dr.Said Alavi
  8. 8. Pathologic Lesions Fibrinoid degeneration of connective tissue,inflammatory edema, inflammatory cell infiltration & proliferation of specific cells resulting in formation of Ashcoff nodules, resulting in- -Pancarditis in the heart -Arthritis in the joints -Ashcoff nodules in the subcutaneous tissue -Basal gangliar lesions resulting in chorea05/05/1999 8 Dr.Said Alavi
  9. 9. Rheumatic Carditis Histology (40X)05/05/1999 9 Dr.Said Alavi
  10. 10. Histology of Myocardium in Rheumatic Carditis (200X)05/05/1999 10 Dr.Said Alavi
  11. 11. Clinical Features 1.Arthritis Flitting & fleeting migratory polyarthritis, involving major joints Commonly involved joints- knee,ankle,elbow & wrist Occur in 80%,involved joints are exquisitely tender In children below 5 yrs arthritis usually mild but carditis more prominent Arthritis do not progress to chronic disease05/05/1999 11 Dr.Said Alavi
  12. 12. Clinical Features (Contd) 2.Carditis Manifest as pancarditis(endocarditis, myocarditis and pericarditis),occur in 40- 50% of cases Carditis is the only manifestation of rheumatic fever that leaves a sequelae & permanent damage to the organ Valvulitis occur in acute phase Chronic phase- fibrosis,calcification & stenosis of heart valves(fishmouth valves)05/05/1999 12 Dr.Said Alavi
  13. 13. Rheumatic heart disease. Abnormal mitral valve. Thick, fused chordae05/05/1999 13 Dr.Said Alavi
  14. 14. Another view of thick and fused mitral valves in Rheumatic heart disease05/05/1999 14 Dr.Said Alavi
  15. 15. Clinical Features (Contd) 3.Sydenham Chorea Occur in 5-10% of cases Mainly in girls of 1-15 yrs age May appear even 6/12 after the attack of rheumatic fever Clinically manifest as-clumsiness, deterioration of handwriting,emotional lability or grimacing of face Clinical signs- pronator sign, jack in the box sign , milking sign of hands05/05/1999 15 Dr.Said Alavi
  16. 16. Clinical Features (Contd) 4.Erythema Marginatum Occur in <5%. Unique,transient,serpiginous-looking lesions of 1-2 inches in size Pale center with red irregular margin More on trunks & limbs & non-itchy Worsens with application of heat Often associated with chronic carditis05/05/1999 16 Dr.Said Alavi
  17. 17. Clinical Features (Contd) 5.Subcutaneous nodules Occur in 10% Painless,pea-sized,palpable nodules Mainly over extensor surfaces of joints,spine,scapulae & scalp Associated with strong seropositivity Always associated with severe carditis05/05/1999 17 Dr.Said Alavi
  18. 18. Clinical Features (Contd) Other features (Minor features) Fever-(upto 101 degree F) Arthralgia Pallor Anorexia Loss of weight05/05/1999 18 Dr.Said Alavi
  19. 19. Laboratory Findings High ESR Anemia, leucocytosis Elevated C-reactive protien ASO titre >200 Todd units. (Peak value attained at 3 weeks,then comes down to normal by 6 weeks) Anti-DNAse B test Throat culture-GABHstreptococci05/05/1999 19 Dr.Said Alavi
  20. 20. Laboratory Findings (Contd) ECG- prolonged PR interval, 2nd or 3rd degree blocks,ST depression, T inversion 2D Echo cardiography- valve edema,mitral regurgitation, LA & LV dilatation,pericardial effusion,decreased contractility05/05/1999 20 Dr.Said Alavi
  21. 21. Diagnosis Rheumatic fever is mainly a clinical diagnosis No single diagnostic sign or specific laboratory test available for diagnosis Diagnosis based on MODIFIED JONES CRITERIA05/05/1999 21 Dr.Said Alavi
  22. 22. Jones Criteria (Revised) for Guidance in the Diagnosis of Rheumatic Fever*Major Manifestation Minor Supporting Evidence Manifestations of Streptococal Infection Carditis Clinical Laboratory Polyarthritis Previous Acute phase Chorea rheumatic reactants: Increased Titer of Anti-Erythema Marginatum fever or Erythrocyte Streptococcal Antibodies ASOSubcutaneous Nodules rheumatic sedimentation (anti-streptolysin O), heart disease rate, others Arthralgia C-reactive Positive Throat Culture Fever protein, for Group A Streptococcus leukocytosis Recent Scarlet Fever Prolonged P- R interval*The presence of two major criteria, or of one major and two minor criteria,indicates a high probability of acute rheumatic fever, if supported by evidence ofGroup A streptococcal nfection. Recommendations of the American Heart Association 05/05/1999 22 Dr.Said Alavi
  23. 23. Exceptions to Jones Criteria  Chorea alone, if other causes have been excluded  Insidious or late-onset carditis with no other explanation  Patients with documented RHD or prior rheumatic fever,one major criterion,or of fever,arthralgia or high CRP suggests recurrence05/05/1999 23 Dr.Said Alavi
  24. 24. Differential Diagnosis Juvenile rheumatiod arthritis Septic arthritis Sickle-cell arthropathy Kawasaki disease Myocarditis Scarlet fever Leukemia05/05/1999 24 Dr.Said Alavi
  25. 25. Treatment Step I - primary prevention (eradication of streptococci) Step II - anti inflammatory treatment (aspirin,steroids) Step III- supportive management & management of complications Step IV- secondary prevention (prevention of recurrent attacks)05/05/1999 25 Dr.Said Alavi
  26. 26. STEP I: Primary Prevention of Rheumatic Fever (Treatment of Streptococcal Tonsillopharyngitis)Agent Dose Mode DurationBenzathine penicillin G 600 000 U for patients Intramuscular Once 27 kg (60 lb) 1 200 000 U for patients >27 kg orPenicillin V Children: 250 mg 2-3 times daily Oral 10 d(phenoxymethyl penicillin) Adolescents and adults: 500 mg 2-3 times dailyFor individuals allergic to penicillinErythromycin: 20-40 mg/kg/d 2-4 times daily Oral 10 dEstolate (maximum 1 g/d) orEthylsuccinate 40 mg/kg/d 2-4 times daily Oral 10 d (maximum 1 g/d) Recommendations of American Heart Association05/05/1999 26 Dr.Said Alavi
  27. 27. Step II: Anti inflammatory treatment Clinical condition Drugs Arthritis only Aspirin 75-100 mg/kg/day,give as 4 divided doses for 6 weeks (Attain a blood level 20- 30 mg/dl) Carditis Prednisolone 2-2.5 mg/kg/day, give as two divided doses for 2 weeks Taper over 2 weeks & while tapering add Aspirin 75 mg/kg/day for 2 weeks. Continue aspirin alone 100 mg/kg/day for another 4 weeks05/05/1999 27 Dr.Said Alavi
  28. 28. 3.Step III: Supportive management & management of complications Bed rest Treatment of congestive cardiac failure: -digitalis,diuretics Treatment of chorea: -diazepam or haloperidol Rest to joints & supportive splinting05/05/1999 28 Dr.Said Alavi
  29. 29. STEP IV : Secondary Prevention of Rheumatic Fever (Prevention of Recurrent Attacks)Agent Dose ModeBenzathine penicillin G 1 200 000 U every 4 weeks* Intramuscular orPenicillin V 250 mg twice daily Oral orSulfadiazine 0.5 g once daily for patients 27 kg (60 lb Oral 1.0 g once daily for patients >27 kg (60 lb)For individuals allergic to penicillin and sulfadiazineErythromycin 250 mg twice daily Oral*In high-risk situations, administration every 3 weeks is justified andrecommended Recommendations of American Heart Association05/05/1999 29 Dr.Said Alavi
  30. 30. Duration of Secondary Rheumatic FeverProphylaxis Category DurationRheumatic fever with carditis and At least 10 y since lastresidual heart disease episode and at leastuntil (persistent valvar disease*) age 40 y, sometimes lifelong prophylaxisRheumatic fever with carditis 10 y or well into adulthood,but no residual heart disease whichever is longer(no valvar disease*)Rheumatic fever without carditis 5 y or until age 21 y, whichever is longer*Clinical or echocardiographic evidence. Recommendations of American Heart Association05/05/1999 30 Dr.Said Alavi
  31. 31. Prognosis Rheumatic fever can recur whenever the individual experience new GABH streptococcal infection,if not on prophylactic medicines Good prognosis for older age group & if no carditis during the initial attack Bad prognosis for younger children & those with carditis with valvar lesions05/05/1999 31 Dr.Said Alavi
  32. 32. References Hoffman JIE: Rheumatic Fever . Rudolphs Pediatrics; 20th Ed: 1518 - 1521,1996. Stollerman GH: Rheumatic Fever . Harrisons Principles Of Internal Medicine; 13th Ed: 1046 - 1052,1995. Special Writing Group of the Committee on Rheumatic Fever,endocarditis & Kawasaki Disease of the Council on Cardiovascular Disease in the Young of the American Heart Association: Guidelines for the Diagnosis of Rheumatic Fever. In Jones Criteria, 1992 Update JAMA 268:2029,1992 Todd J: Rheumatic Fever . Nelsons Textbook Of Pediatrics; 15th Ed: 754 - 760, 1996. Warren R, Perez M, Wilking A: Pediatric Rheumatic Diseases . Pediatric Clinics of North America; 41: 783 - 818,1994. WorldHealth Organization Study Group: Rheumatic Fever & Rheumatic Heart Disease,technical Report Series No. 764.Geneva,world Health Organization, 198805/05/1999 32 Dr.Said Alavi
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