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Dr.Said Alavi MD,DCH,DNB,FCPS

Rheumatic Fever. 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. Objectives. Etiology Epidemiology Pathogenesis Pathologic lesions Clinical manifestations & Laboratory findings

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Dr.Said Alavi MD,DCH,DNB,FCPS

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  1. Rheumatic Fever Dr.Said AlaviMD,DCH,DNB,FCPS Dept. of Pediatrics and Neonatology Saqr Hospital,Ras Al Khaimah UNITED ARAB EMIRATES E-mail: drsaid@emirates.net.ae

  2. Objectives • Etiology • Epidemiology • Pathogenesis • Pathologic lesions • Clinical manifestations & Laboratory findings • Diagnosis & Differential diagnosis • Treatment & Prevention • Prognosis • References Dr.Said Alavi

  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 CNS Dr.Said Alavi

  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 spring Dr.Said Alavi

  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 toheart valves,joints, subcutaneous tissue & basal ganglia of brain Dr.Said Alavi

  6. 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 antigenicity Group A Beta Hemolytic Streptococcus Dr.Said Alavi

  7. Diagrammatic structure of the group A beta hemolytic streptococcus Antigen of outer protein cell wall of GABHS induces antibody response in victim which result in autoimmune damage to heart valves, sub cutaneous tissue,tendons, joints & basal ganglia of brain Capsule Cell wall Proteinantigens Group carbohydrate Peptidoglycan Cyto.membrane Cytoplasm …………………………………………………... Dr.Said Alavi

  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 nodulesin the subcutaneous tissue -Basal gangliar lesions resulting in chorea Dr.Said Alavi

  9. Rheumatic Carditis Histology (40X) Dr.Said Alavi

  10. Histology of Myocardium in Rheumatic Carditis (200X) Dr.Said Alavi

  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 disease Dr.Said Alavi

  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) Dr.Said Alavi

  13. Rheumatic heart disease. Abnormal mitral valve. Thick, fused chordae Dr.Said Alavi

  14. Another view of thick and fused mitral valves in Rheumatic heart disease Dr.Said Alavi

  15. 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 hands Clinical Features (Contd) 3.Sydenham Chorea Dr.Said Alavi

  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 carditis Dr.Said Alavi

  17. Clinical Features (Contd) • 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 carditis 5.Subcutaneous nodules Dr.Said Alavi

  18. Clinical Features (Contd) Other features (Minor features) • Fever-(upto 101 degree F) • Arthralgia • Pallor • Anorexia • Loss of weight Dr.Said Alavi

  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-GABHstreptococci Dr.Said Alavi

  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 contractility Dr.Said Alavi

  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 CRITERIA Dr.Said Alavi

  22. Recommendations of the American Heart Association 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 recurrence Dr.Said Alavi

  24. Differential Diagnosis • Juvenile rheumatiod arthritis • Septic arthritis • Sickle-cell arthropathy • Kawasaki disease • Myocarditis • Scarlet fever • Leukemia Dr.Said Alavi

  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) Dr.Said Alavi

  26. STEP I: Primary Prevention of Rheumatic Fever (Treatment of Streptococcal Tonsillopharyngitis) Agent Dose Mode Duration Benzathine penicillin G 600 000 U for patients Intramuscular Once 27 kg (60 lb) 1 200 000 U for patients >27 kg or Penicillin V Children: 250 mg 2-3 times daily Oral 10 d (phenoxymethyl penicillin) Adolescents and adults: 500 mg 2-3 times daily For individuals allergic to penicillin Erythromycin: 20-40 mg/kg/d 2-4 times daily Oral 10 d Estolate (maximum 1 g/d) or Ethylsuccinate 40 mg/kg/d 2-4 times daily Oral 10 d (maximum 1 g/d) Recommendations of American Heart Association Dr.Said Alavi

  27. Step II:Anti inflammatory treatment Clinical condition Drugs Dr.Said Alavi

  28. Bed rest Treatment of congestive cardiac failure: -digitalis,diuretics Treatment of chorea: -diazepam or haloperidol Rest to joints & supportive splinting 3.Step III: Supportive management & management of complications Dr.Said Alavi

  29. STEP IV : Secondary Prevention of Rheumatic Fever (Prevention of Recurrent Attacks) Agent Dose Mode Benzathine penicillin G 1 200 000 U every 4 weeks* Intramuscular or Penicillin V 250 mg twice daily Oral or Sulfadiazine 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 sulfadiazine Erythromycin 250 mg twice daily Oral *In high-risk situations, administration every 3 weeks is justified and recommended Recommendations of American Heart Association Dr.Said Alavi

  30. Duration of Secondary Rheumatic Fever Prophylaxis Category Duration Rheumatic fever with carditis and At least 10 y since last residual heart disease episode and at least until (persistent valvar disease*) age 40 y, sometimes lifelong prophylaxis Rheumatic 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 Association Dr.Said Alavi

  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 lesions Dr.Said Alavi

  32. References • Hoffman JIE: Rheumatic Fever . Rudolph's Pediatrics; 20th Ed: 1518 - 1521,1996. • Stollerman GH: Rheumatic Fever . Harrison's 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 . Nelson's 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. • World Health Organization Study Group: Rheumatic Fever & Rheumatic Heart Disease,technical Report Series No. 764.Geneva,world Health Organization, 1988 Dr.Said Alavi

  33. Thank You Dr.Said Alavi

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