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  • 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. Objectives q Etiology q Epidemiology q Pathogenesis q Pathologic lesions q Clinical manifestations & Laboratory findings q Diagnosis & Differential diagnosis q Treatment & Prevention q Prognosis q References05/05/1999 2 Dr.Said Alavi
  • 3. Etiology q Acute rheumatic fever is a systemic disease of childhood,often recurrent that follows group A beta hemolytic streptococcal infection q It is a delayed non-suppurative sequelae to URTI with GABH streptococci. q 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. Epidemiology q Ages 5-15 yrs are most susceptible q Rare <3 yrs q Girls>boys q Common in 3rd world countries q Environmental factors-- over crowding, poor sanitation, poverty, q Incidence more during fall ,winter & early spring05/05/1999 4 Dr.Said Alavi
  • 5. Pathogenesis q Delayed immune response to infection with group.A beta hemolytic streptococci. q 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. Group A Beta Hemolytic Streptococcus q Strains that produces rheumatic fever - M types l, 3, 5, 6,18 & 24 q Pharyngitis- produced by GABHS can lead to- acute rheumatic fever , rheumatic heart disease & post strept. Glomerulonepritis q 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. 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. Pathologic Lesions q 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. Rheumatic Carditis Histology (40X)05/05/1999 9 Dr.Said Alavi
  • 10. Histology of Myocardium in Rheumatic Carditis (200X)05/05/1999 10 Dr.Said Alavi
  • 11. Clinical Features 1.Arthritisq Flitting & fleeting migratory polyarthritis, involving major joints q Commonly involved joints- knee,ankle,elbow & wrist q Occur in 80%,involved joints are exquisitely tender q In children below 5 yrs arthritis usually mild but carditis more prominent q Arthritis do not progress to chronic disease05/05/1999 11 Dr.Said Alavi
  • 12. Clinical Features (Contd) 2.Carditis q Manifest as pancarditis(endocarditis, myocarditis and pericarditis),occur in 40-50% of cases q Carditis is the only manifestation of rheumatic fever that leaves a sequelae & permanent damage to the organ q Valvulitis occur in acute phase q Chronic phase- fibrosis,calcification & stenosis of heart valves(fishmouth valves)05/05/1999 12 Dr.Said Alavi
  • 13. Rheumatic heart disease. Abnormal mitral valve. Thick, fused chordae05/05/1999 13 Dr.Said Alavi
  • 14. Another view of thick and fused mitral valves in Rheumatic heart disease05/05/1999 14 Dr.Said Alavi
  • 15. Clinical Features (Contd) 3.Sydenham Chorea q Occur in 5-10% of cases q Mainly in girls of 1-15 yrs age q May appear even 6/12 after the attack of rheumatic fever q Clinically manifest as-clumsiness, deterioration of handwriting,emotional lability or grimacing of face q Clinical signs- pronator sign, jack in the box sign , milking sign of hands05/05/1999 15 Dr.Said Alavi
  • 16. Clinical Features (Contd) 4.Erythema Marginatum q Occur in <5%. q Unique,transient,serpiginous-looking lesions of 1-2 inches in size q Pale center with red irregular margin q More on trunks & limbs & non-itchy q Worsens with application of heat q Often associated with chronic carditis05/05/1999 16 Dr.Said Alavi
  • 17. Clinical Features (Contd) 5.Subcutaneous nodules q Occur in 10% q Painless,pea-sized,palpable nodules q Mainly over extensor surfaces of joints,spine,scapulae & scalp q Associated with strong seropositivity q Always associated with severe carditis05/05/1999 17 Dr.Said Alavi
  • 18. Clinical Features (Contd) Other features (Minor features) q Fever-(upto 101 degree F) q Arthralgia q Pallor q Anorexia q Loss of weight05/05/1999 18 Dr.Said Alavi
  • 19. Laboratory Findingsq High ESRq Anemia, leucocytosisq Elevated C-reactive protienq ASO titre >200 Todd units. (Peak value attained at 3 weeks,then comes down to normal by 6 weeks)q Anti-DNAse B testq Throat culture-GABHstreptococci05/05/1999 19 Dr.Said Alavi
  • 20. Laboratory Findings (Contd) q ECG- prolonged PR interval, 2nd or 3rd degree blocks,ST depression, T inversion q 2D Echo cardiography- valve edema,mitral regurgitation, LA & LV dilatation,pericardial effusion,decreased contractility05/05/1999 20 Dr.Said Alavi
  • 21. Diagnosis q Rheumatic fever is mainly a clinical diagnosis q No single diagnostic sign or specific laboratory test available for diagnosis q Diagnosis based on MODIFIED JONES CRITERIA05/05/1999 21 Dr.Said Alavi
  • 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. 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. Differential Diagnosis q Juvenile rheumatiod arthritis q Septic arthritis q Sickle-cell arthropathy q Kawasaki disease q Myocarditis q Scarlet fever q Leukemia05/05/1999 24 Dr.Said Alavi
  • 25. Treatment q Step I - primary prevention (eradication of streptococci) q Step II - anti inflammatory treatment (aspirin,steroids) q Step III- supportive management & management of complications q Step IV- secondary prevention (prevention of recurrent attacks)05/05/1999 25 Dr.Said Alavi
  • 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. 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. 3.Step III: Supportive management & management of complications q Bed rest q Treatment of congestive cardiac failure: -digitalis,diuretics q Treatment of chorea: -diazepam or haloperidol q Rest to joints & supportive splinting05/05/1999 28 Dr.Said Alavi
  • 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 andrecommended05/05/1999 Recommendations of 29 American Heart Association Dr.Said Alavi
  • 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. Prognosis q Rheumatic fever can recur whenever the individual experience new GABH streptococcal infection,if not on prophylactic medicines q Good prognosis for older age group & if no carditis during the initial attack q Bad prognosis for younger children & those with carditis with valvar lesions05/05/1999 31 Dr.Said Alavi
  • 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
  • 33. 05/05/1999 33 Dr.Said Alavi