Rheumatic fever

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Rheumatic fever

  1. 1. Rheumatic Fever Dr.B.BALAGOBI
  2. 2. Objectives • Introduction • Etiology • Epidemiology • Pathogenesis • Pathologic lesions • Clinical manifestations & Laboratory findings • Diagnosis & Differential diagnosis • Treatment & Prevention • Prognosis10/27/2012 2
  3. 3. Acute Rheumatic Fever...• A connective tissue disease• Acquired heart disease• Mainly in Developing countries• Significant morbidity and mortality• Association with pharyngitis - group A haemolytic streptococci• High risk of recurrence –So prophylaxis is needed 10/27/2012 3
  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 CNS10/27/2012 4
  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 spring10/27/2012 5
  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 brain10/27/2012 6
  7. 7. Group A streptococcal pharyngitis10/27/2012 7
  8. 8. 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 antigenicity10/27/2012 8
  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 chorea10/27/2012 9
  10. 10. 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 disease10/27/2012 10
  11. 11. 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)10/27/2012 11
  12. 12. Rheumatic heart disease. Abnormal mitral valve. Thick, fused chordae10/27/2012 12
  13. 13. 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 hands10/27/2012 13
  14. 14. 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 carditis10/27/2012 14
  15. 15. 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 carditis10/27/2012 15
  16. 16. Clinical Features (Contd) Other features (Minor features) • Fever-(upto 101 degree F) • Arthralgia • Pallor • Anorexia • Loss of weight10/27/2012 16
  17. 17. 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-GABHstreptococci10/27/2012 17
  18. 18. 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 contractility10/27/2012 18
  19. 19. Diagnosis• Rheumatic fever is mainly a clinical diagnosis• No single diagnostic sign or specific laboratory test available for diagnosis• Diagnosis based on MODIFIED JONES CRITERIA10/27/2012 19
  20. 20. Guidelines for diagnosis of the initial attack of rheumatic fever. Duckett Jones criteria, 1992 update - American Heart Association • 2 major manifestations or • 1 major and 2 minor manifestations • supported by – Evidence of antecedent streptococcal infection10/27/2012 20
  21. 21. Major manifestations...• Polyarthritis• Carditis• Chorea• Subcutaneous nodules• Erythema marginatum10/27/2012 21
  22. 22. Minor manifestations...• Clinical – Arthralgia – Fever• Laboratory – Elevated acute-phasereactants (ESR,CRP) – Prolonged PR interval10/27/2012 22
  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 recurrence10/27/2012 23
  24. 24. Differential Diagnosis• Juvenile rheumatiod arthritis• SLE• Septic arthritis• Sickle-cell arthropathy• Kawasaki disease• Myocarditis• Scarlet fever• Leukemia10/27/2012 24
  25. 25. Management... Average course of 6-8 weeks• Admit - confirmation, education, drugs• Investigations• Bed rest - CCF - strict bed rest• Antibiotics - oral penicillin for 10 days or IM Benzathine penicillin• Anti rheumatic drugs - aspirin / steroids• Aspirin - dose/administration/side effects• Duration: RF: ~ 6 weeks and tail off over ~ 2wks RC: 8 -10 weeks and tail off over ~ 2 wks• Steroids - no effect on long term prognosis CCF / impending heart failure 10/27/2012 25
  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)10/27/2012 26
  27. 27. 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 Association10/27/2012 27
  28. 28. 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 weeks 2810/27/2012
  29. 29. 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 splinting10/27/2012 29
  30. 30. Why prophylaxis..?• To prevent streptococcal infections which precipitate recurrences of rheumatic fever• Prevent development of chronic rheumatic heart disease• If recurrences are prevented, 70% of patients with carditis in the initial attack will eventually have normal hearts• No documented evidence of resistance of group A streptococci to penicillin 10/27/2012 30
  31. 31. Prophylaxis...• Primary - Adequate treatment of streptococcal sore throats - oral penicillin for 10 days Clinical differentiation of viral/bacterial sore throats is difficult Throat swab for culture and ABST Erythromycin10/27/2012 31
  32. 32. Prophylaxis ctd...• Secondary -• Benzathine penicillin 1.2 mega units IM ( ARF - 4 weekly/RC - 3 weekly )• Duration - ARF - 18 / 21yrs or 5yrs after last attack• Carditis - (extent of damage) ~ 25• Chronic valvular heart disease - life long• Infective endocarditis prophylaxis - life long 10/27/2012 32
  33. 33. 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 lesions10/27/2012 33
  34. 34. T/F In Rheumatic fever?A. is causing deformity in jointsB. small joints of the hands are commonly affectedC. Anti streptolysin O is elevatedD. Aspirin treatment prevents the cardiac involvementE. Sleeping pulse rate is elevated in Carditis10/27/2012 34
  35. 35. T/F Features of rhematic carditis?A. Pericardial rubB. Congestive heart failureC. Coronary artery aneurysmD. Mid diastolic murmurE. tachycardia10/27/2012 35
  36. 36. T/F which of the following are the minor criteria of Rheumatic fever?A. sub cutaneous noduleB. ArthritisC. Elevated ASOTD. Raised ESRE. FeverF. Prolonged PR interval in ECG10/27/2012 36
  37. 37. T/F regarding Rheumatic fever?A. Chorea is associated with subcutaneous noduleB. Prolong PR interval in ECG indicates the underlying carditisC. Erythema nodosum is a major criteriaD. IM Benzathine penicillin given 3 weekly if carditis is presentE. Mitral stenosis is common at the acute stage of the disease10/27/2012 37
  38. 38. T/F regarding Rheumatic fever?A. Steroids are superior to salicylates in prevention of carditisB. Subcutaneous nodules are associated with bad prognosisC. History of sore throat is essential for the diagnosisD. Can Cause early diastolic murmur at left lower sternal edgeE. Can cause cardiomegaly10/27/2012 38
  39. 39. T/F regarding Rheumatic fever?A. In patient with Rheumatic valvular heart disease antibiotic prophylaxis monthly given to up to 21 years of ageB. In patient with Rheumatic valvular heart disease antibiotic prophylaxis monthly given to prevent infective endocarditisC. Emotional lability is a feature of ChoreaD. Aortic valve involvement is commoner than mitral valve involvement.E. New onset Pansystolic murmur is a feature.10/27/2012 39
  40. 40. T/F Rheumatic fever?A. Low dose aspirin is used in the treatmentB. Common in children than adultsC. Cause erosive arthritisD. Seen in 15% of children with phayrngitisE. There are no recurrence10/27/2012 40
  41. 41. T/F Rheumatic fever?A. Associated with β haemolytic streptococciB. Can not be diagnosed if normal ASOTC. Chorea is a late featureD. Commonly affects the endocardium of the heartE. Chorea is common in boys10/27/2012 41
  42. 42. T/F regarding Acute Rheumatic fever?• Salicylates or steroids should not be started until diagnosis is confirmed• Antibiotic therapy during acute infection can alter the severity of cardiac involvement• Compared to salicylates ;steroids use significantly reduce rheumatic valvular disease• Prophylaxis with Oral penicillin /IM benzathine penicillin are equally effective• Effective serum concentration of drug detected up to 4 wks after IM Benzathine penicillin10/27/2012 42

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