Rheumatic fever in children

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Overview of rheumatic fever in children with recent treatment and prevention.

Published in: Health & Medicine

Rheumatic fever in children

  1. 1. Dr.Youssef Quda M.B.B.ch (Cairo University) MSc.(Zazazig University) www.facebook.com/dryousefqudawww.facebook.com/dryousefquda
  2. 2. Dr.Youssef Quda M.B.B.ch (Cairo University) MSc.(Zazazig University) www.facebook.com/dryousefqudawww.facebook.com/dryousefquda
  3. 3. ObjectivesObjectives Etiology Epidemiology Pathogenesis Pathologic lesions Clinical manifestations & Laboratory findings Diagnosis & Differential diagnosis Treatment & Prevention Prognosis
  4. 4. 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
  5. 5. 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
  6. 6. 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 brain
  7. 7. 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
  8. 8. Diagrammatic structure of the group A beta hemolytic streptococcus Capsule Cell wall Protein antigens Group carbohydrate Peptidoglycan Cyto.membrane Cytoplasm …………………………………………… ……... 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
  9. 9. 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 chorea
  10. 10. Clinical Features Flitting & fleeting migratory polyarthritis, involving big joints. Commonly involved joints- knee,ankle,elbow & wrist joints are red , hot, tender with limitation of movement. Dramatic response to Salicylate. Resolve without treatment. 1.Arthritis (75%)
  11. 11. Clinical Features (Contd) 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. 2.Carditis
  12. 12. Clinical Features (Contd) - Endocarditis: Valvulitis affecting commonly mitral valve: 1- Mitral valve: - leaflet oedema leads to transient mitral stenosis( Carey Combs murmur) - leaflet destruction leads to MR. 2. Aortic valve : AR. 2.Carditis
  13. 13. Clinical Features (Contd) - Myocarditis: - Tachycardia out of proportion to age &fever - Heart failure - Pericarditis: - dry pericarditis ------stitching pain. - pericardial effusion 2.Carditis
  14. 14. Clinical Features (Contd) Occur in 5-10% of cases Mainly in girls of 1-15 yrs age May appear even 6m after the attack of rheumatic fever Clinically manifest as-clumsiness, deterioration of handwriting,emotional lability or grimacing of face Clinical signs- pronator sign, milking sign of hands 3.Rheumatic Chorea (Sydenham Chorea)
  15. 15. Clinical Features (Contd) 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 4.Erythema Marginatum
  16. 16. 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
  17. 17. Clinical Features (Contd) Other features (Minor features) Fever Arthralgia Pallor Anorexia Loss of weight
  18. 18. 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
  19. 19. 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
  20. 20. 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
  21. 21. Jones Criteria (Revised) for Guidance in the Diagnosis of Rheumatic Fever* Major Manifestation Minor Manifestations Supporting Evidence of Streptococal Infection Clinical LaboratoryCarditis Polyarthritis Chorea Erythema Marginatum Subcutaneous Nodules Previous rheumatic fever or rheumatic heart disease Arthralgia Fever Acute phase reactants: Erythrocyte sedimentation rate, C-reactive protein, leukocytosis Prolonged P- R interval Increased Titer of Anti- Streptococcal Antibodies ASO (anti-streptolysin O), others Positive Throat Culture for Group A Streptococcus Recent Scarlet Fever *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 of Group A streptococcal nfection. Recommendations of the American Heart Association
  22. 22. 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
  23. 23. Differential Diagnosis 1.Other causes of arthritis: - Rheumatic arthritis - Infection:viral,bacterial,T.B. -Hematologic:acute leukemia, hemophilia - Immunologic: HSP,SLE.
  24. 24. Differential Diagnosis 2. Other causes of carditis: - Viral carditis. - Infective endocarditis - Drug induced. 3.Other causes of chorea: - Wilson disease. - Huntington chorea. - Cerebral palsy.
  25. 25. Complications of rheumatic fever 1.Congestive heart failure. 2.Cardiomegaly. 3.Chronic valve disease. 4.Rheumatic activity (recurrence). 5.Pulmonary hypertension.
  26. 26. 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)
  27. 27. 30 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
  28. 28. 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 weeks Step II: Anti inflammatory treatment Clinical condition Drugs
  29. 29. 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
  30. 30. 33 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 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
  31. 31. 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
  32. 32. Prognosis 1.Arthritis subside within days to weeks even without treatment. 2.Chorea subside within few months without residuals.

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