Rheumatic fever


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Rheumatic fever

  2. 2. DEFINITION:Acute rheumatic fever is a systemic disease of childhood ,often recurrent that follows group A beta hemolytic streptococcal infectionIt is a diffuse inflammatory disease of connective tissue primarily involving heart, blood vessels, joints, subcut.tissue and CNS 05/05/1999 Dr.Said Alavi 2
  3. 3. EtiologyBetahemolytic streptococci.Genetic 05/05/1999 Dr.Said Alavi 3
  4. 4. EpidemiologyAges 5-15 yrs are most susceptibleRare <3 yrsGirls>boysEnvironmental factors--over crowding, poor sanitation, poverty,Incidence more during fall ,winter & early spring 05/05/1999 Dr.Said Alavi 4
  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 brain. 05/05/1999 Dr.Said Alavi 5
  6. 6. 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 05/05/1999 Dr.Said Alavi 6
  7. 7. Clinical Features1.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 05/05/1999 Dr.Said Alavi 7
  8. 8. 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 Dr.Said Alavi 8
  9. 9. 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 hands 05/05/1999 Dr.Said Alavi 9
  10. 10. 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 05/05/1999 Dr.Said Alavi 10
  11. 11. 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 05/05/1999 Dr.Said Alavi 11
  12. 12. Clinical Features (Contd)Other features (Minor features)Fever-(upto 101 degree F)ArthralgiaPallorAnorexiaLoss of weight 05/05/1999 Dr.Said Alavi 12
  13. 13. 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)Throat culture-BHstreptococci 05/05/1999 Dr.Said Alavi 13
  14. 14. 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, and decreased contractility 05/05/1999 Dr.Said Alavi 14
  15. 15. 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 05/05/1999 Dr.Said Alavi 15
  16. 16. 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 fever reactants: Increased Titer of Anti-Streptococcal Erythema Marginatum or rheumatic Erythrocyte Antibodies ASO Subcutaneous Nodules heart disease sedimentation (anti-streptolysin O), Arthralgia rate, others Fever C-reactive Positive Throat Culture 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 ahigh probability of acute rheumatic fever, if supported by evidence of Group Astreptococcal nfection. Recommendations of the American Heart Association 05/05/1999 Dr.Said Alavi 16
  17. 17. 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 Dr.Said Alavi 17
  18. 18. 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 Association 05/05/1999 Dr.Said Alavi 18
  19. 19. 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 05/05/1999 Dr.Said Alavi 19
  20. 20. 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 splinting 05/05/1999 Dr.Said Alavi 20
  21. 21. 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 Association 05/05/1999 Dr.Said Alavi 21
  22. 22. 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 Association 05/05/1999 Dr.Said Alavi 22
  23. 23. PrognosisRheumatic fever can recur whenever the individual experience new BH 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 05/05/1999 Dr.Said Alavi 23
  24. 24. THANK YOU 05/05/1999 Dr.Said Alavi 24
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