1. Rheumatic Fever Rheumatic Fever

2. 05/05/1999Dr.Said Alavi2 Etiology Acute rheumatic fever is a systemic disease of childhood,often recurrent that follows group A beta hemolytic streptococcal infection 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 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 It is a diffuse inflammatory disease of connective tissue,primarily involving heart,blood vessels,joints, subcut.tissue and CNS

3. 05/05/1999Dr.Said Alavi3 Epidemiology Ages 5-15 yrs are most susceptible Ages 5-15 yrs are most susceptible Rare <3 yrs Rare <3 yrs Girls>boys Girls>boys Common in 3rd world countries Common in 3rd world countries Environmental factors-- over crowding, poor sanitation, poverty, Environmental factors-- over crowding, poor sanitation, poverty, Incidence more during fall,winter & early spring Incidence more during fall,winter & early spring

4. 05/05/1999Dr.Said Alavi4 Pathogenesis Delayed immune response to infection with group.A beta hemolytic streptococci. 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 After a latent period of 1-3 weeks, antibody induced immunological damage occur to heart valves,joints, subcutaneous tissue & basal ganglia of brain

5. 05/05/1999Dr.Said Alavi5 Strains that produces rheumatic fever - M types l, 3, 5, 6,18 & 24 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 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 Skin infection- produced by GABHS leads to post streptococcal glomerulo nephritis only. It will not result in Rh.Fever or carditis Group A Beta Hemolytic Streptococcus

6. 05/05/1999Dr.Said Alavi6 Clinical Features Migratory polyarthritis, involving major joints Migratory polyarthritis, involving major joints Commonly involved joints-knee,ankle,elbow & wrist Commonly involved joints-knee,ankle,elbow & wrist Occur in 80%,involved joints are exquisitely tender Occur in 80%,involved joints are exquisitely tender In children below 5 yrs arthritis usually mild but carditis more prominent In children below 5 yrs arthritis usually mild but carditis more prominent Arthritis do not progress to chronic disease Arthritis do not progress to chronic disease 1.Arthritis

7. 05/05/1999Dr.Said Alavi7 Clinical Features (Contd) Manifest as pancarditis (endocarditis, myocarditis and pericarditis),occur in 40- 50% of cases 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 Carditis is the only manifestation of rheumatic fever that leaves a sequelae & permanent damage to the organ Valvulitis occur in acute phase Valvulitis occur in acute phase Chronic phase- fibrosis,calcification & stenosis of heart valves. Chronic phase- fibrosis,calcification & stenosis of heart valves. 2.Carditis

8. 05/05/1999Dr.Said Alavi8 Clinical Features (Contd) Occur in 5-10% of cases Occur in 5-10% of cases Mainly in girls of 1-15 yrs age Mainly in girls of 1-15 yrs age May appear even 6 months after the attack of rheumatic fever May appear even 6 months after the attack of rheumatic fever Clinically manifest as-clumsiness, deterioration of handwriting,emotional lability or grimacing of face Clinically manifest as-clumsiness, deterioration of handwriting,emotional lability or grimacing of face 3.Sydenham Chorea

9. 05/05/1999Dr.Said Alavi9 Clinical Features (Contd) Occur in <5%. Occur in <5%. Unique, transient lesions of 1-2 inches in size Unique, transient lesions of 1-2 inches in size Pale center with red irregular margin Pale center with red irregular margin More on trunks & limbs & non-itchy More on trunks & limbs & non-itchy Worsens with application of heat Worsens with application of heat Often associated with chronic carditis Often associated with chronic carditis 4.Erythema Marginatum

10. 05/05/1999Dr.Said Alavi10 Clinical Features (Contd) Occur in 10% Occur in 10% Painless,pea-sized,palpable nodules Painless,pea-sized,palpable nodules Mainly over extensor surfaces of joints,spine,scapulae & scalp Mainly over extensor surfaces of joints,spine,scapulae & scalp Associated with strong seropositivity Associated with strong seropositivity Always associated with severe carditis Always associated with severe carditis 5.Subcutaneous nodules

11. 05/05/1999Dr.Said Alavi11 Clinical Features (Contd) Other features (Minor features) Fever – Low grade Fever – Low grade Arthralgia Arthralgia Pallor Pallor Anorexia Anorexia Loss of weight Loss of weight

12. 05/05/1999Dr.Said Alavi12 Laboratory Findings High ESR High ESR Anemia, leucocytosis Anemia, leucocytosis Elevated C-reactive protien Elevated C-reactive protien ASO titre >200. (Peak value attained at 3 weeks,then comes down to normal by 6 weeks) ASO titre >200. (Peak value attained at 3 weeks,then comes down to normal by 6 weeks) Anti-DNAse B test Anti-DNAse B test Throat culture-GABHstreptococci Throat culture-GABHstreptococci

13. 05/05/1999Dr.Said Alavi13 Laboratory Findings (Contd) ECG- prolonged PR interval ECG- prolonged PR interval Echo - valve edema,mitral regurgitation, LA & LV dilatation,pericardial effusion,decreased contractility Echo - valve edema,mitral regurgitation, LA & LV dilatation,pericardial effusion,decreased contractility

14. 05/05/1999Dr.Said Alavi14 Diagnosis Rheumatic fever is mainly a clinical diagnosis Rheumatic fever is mainly a clinical diagnosis No single diagnostic sign or specific laboratory test available for diagnosis No single diagnostic sign or specific laboratory test available for diagnosis Diagnosis based on MODIFIED JONES CRITERIA Diagnosis based on MODIFIED JONES CRITERIA

15. 05/05/1999Dr.Said Alavi15 Recommendations of the American Heart Association

16. 05/05/1999Dr.Said Alavi16 Treatment Step I - primary prevention (eradication of streptococci) Step I - primary prevention (eradication of streptococci) Step II - anti inflammatory treatment (aspirin,steroids) Step II - anti inflammatory treatment (aspirin,steroids) Step III- supportive management & management of complications Step III- supportive management & management of complications Step IV- secondary prevention (prevention of recurrent attacks) Step IV- secondary prevention (prevention of recurrent attacks)

17. 05/05/1999Dr.Said Alavi17 STEP I: Primary Prevention of Rheumatic Fever (Treatment of Streptococcal Tonsillopharyngitis) Agent Dose Mode Duration Benzathine penicillin G600 000 U for patients Intramuscular Once 27 kg (60 lb) 1 200 000 U for patients >27 kg or Penicillin VChildren: 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 Ethylsuccinate40 mg/kg/d 2-4 times daily Oral 10 d (maximum 1 g/d) Recommendations of American Heart Association

18. 05/05/1999Dr.Said Alavi18 Step II: Anti inflammatory treatment Clinical condition Drugs

19. 05/05/1999Dr.Said Alavi19 Bed rest Bed rest Treatment of congestive cardiac failure: -digitalis,diuretics Treatment of congestive cardiac failure: -digitalis,diuretics Treatment of chorea: -diazepam or haloperidol Treatment of chorea: -diazepam or haloperidol Rest to joints & supportive splinting Rest to joints & supportive splinting 3.Step III: Supportive management & management of complications

20. 05/05/1999Dr.Said Alavi20 STEP IV : Secondary Prevention of Rheumatic Fever (Prevention of Recurrent Attacks) Agent DoseMode Benzathine penicillin G1 200 000 U every 4 weeks*Intramuscular or Penicillin V250 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 Erythromycin250 mg twice daily Oral *In high-risk situations, administration every 3 weeks is justified and recommended Recommendations of American Heart Association

21. 05/05/1999Dr.Said Alavi21 Prognosis Rheumatic fever can recur whenever the individual experience new GABH streptococcal infection,if not on prophylactic medicines 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 Good prognosis for older age group & if no carditis during the initial attack Bad prognosis for younger children & those with carditis with valvar lesions Bad prognosis for younger children & those with carditis with valvar lesions