Rheumatic Fever PDF

Rheumatic Fever 05/05/1999Dr.Said Alavi 0  Objectives Etiology Epidemiology Pathogenesis Pathologic lesions Clinical manifestations & Laboratory findings Diagnosis & Differential diagnosis Treatment & Prevention Prognosis References...

Rheumatic Fever 05/05/1999Dr.Said Alavi 0  Objectives Etiology Epidemiology Pathogenesis Pathologic lesions Clinical manifestations & Laboratory findings Diagnosis & Differential diagnosis Treatment & Prevention Prognosis References 05/05/1999Dr.Said Alavi 0  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 05/05/1999Dr.Said Alavi 0 05/05/1999Dr.Said Alavi 0 05/05/1999Dr.Said Alavi 0  Epidemiology Ages 5-15 yrs are most susceptible Rare boys Common in 3rd world countries Environmental factors-- over crowding, poor sanitation, poverty, Incidence more during fall ,winter & early spring 05/05/1999 0 05/05/1999Dr.Said Alavi 0 05/05/1999Dr.Said Alavi 0  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 05/05/1999Dr.Said Alavi 0  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 antigenicity 05/05/1999Dr.Said Alavi 0 05/05/1999Dr.Said Alavi 0 Diagrammatic structure of the group A beta hemolytic streptococcus Antigen of outer Capsule protein cell wall Cell wall of GABHS induces antibody Protein antigens response in victim which Group carbohydrate result in Peptidoglycan autoimmune damage to heart Cyto.membrane valves, sub cutaneous Cytoplasm tissue,tendons, joints & basal …………………………………………… ganglia of brain ……... 05/05/1999Dr.Said Alavi 0  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 05/05/1999Dr.Said Alavi 0 Rheumatic Carditis Histology (40X) 05/05/1999Dr.Said Alavi 0 Histology of Myocardium in Rheumatic Carditis (200X) 05/05/1999Dr.Said Alavi 0  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 05/05/1999Dr.Said Alavi 0  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) 05/05/1999Dr.Said Alavi 0 05/05/1999Dr.Said Alavi 0  Rheumatic heart disease. Abnormal mitral valve. Thick, fused chordae 05/05/1999Dr.Said Alavi 0  Another view of thick and fused mitral valves in Rheumatic heart disease 05/05/1999Dr.Said Alavi 0 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 05/05/1999Dr.Said Alavi 0 05/05/1999Dr.Said Alavi 0 05/05/1999Dr.Said Alavi 0  Clinical Features (Contd) 4.Erythema Marginatum Occur in 200 Todd units. (Peak value attained at 3 weeks,then comes down to normal by 6 weeks) Anti-DNAse B test Throat culture-GABHstreptococci 05/05/1999Dr.Said Alavi 0 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 05/05/1999Dr.Said Alavi 0 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 05/05/1999Dr.Said Alavi 0  Jones Criteria (Revised) for Guidance in the 05/05/1999 0 Dr.Said Alavi Diagnosis of Rheumatic Fever* Major Manifestation Minor Supporting Evidence Manifestations of Streptococal Infection Carditis Clinical Laboratory Polyarthritis Previous Acute phase Chorea rheumatic reactants: Increased Titer of Anti- Erythema Marginatum fever or Erythrocyte Streptococcal Antibodies ASO Subcutaneous Nodules rheumatic sedimentation (anti-streptolysin O), heart disease rate, others Arthralgia C-reactive Positive Throat Culture Fever 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 a high probability of acute rheumatic fever, if supported by evidence of Group A streptococcal nfection. Recommendations of the American Heart Association  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 05/05/1999Dr.Said Alavi 0 05/05/1999Dr.Said Alavi 0  Differential Diagnosis Juvenile rheumatiod arthritis Septic arthritis Sickle-cell arthropathy Kawasaki disease Myocarditis Scarlet fever Leukemia 05/05/1999Dr.Said Alavi 0  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) 05/05/1999Dr.Said Alavi 0 STEP I: Primary Prevention of Rheumatic Fever (Treatment of 05/05/1999 0 Streptococcal Tonsillopharyngitis) Dr.Said Alavi 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  05/05/1999 0 Step II: Anti inflammatory treatment Dr.Said Alavi 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 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 05/05/1999Dr.Said Alavi 0 STEP IV : Secondary Prevention of Rheumatic Fever (Prevention of Recurrent Attacks) 05/05/1999 0 Dr.Said Alavi 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  05/05/1999 0 Duration of Secondary Rheumatic Fever Dr.Said Alavi 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  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 05/05/1999Dr.Said Alavi 0 References Hoffman JIE: Rheumatic Fever. Rudolph's Pediatrics; 20th Ed: 1518 - 1521,1996. StollermanGH: Rheumatic Fever. Harrison's Principles Of Internal Medicine; 13th Ed: 1046 - 1052,1995. SpecialWriting 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. WorldHealth Organization Study Group: Rheumatic Fever & Rheumatic Heart Disease,technical Report Series No. 764.Geneva,world Health Organization, 1988 05/05/1999Dr.Said Alavi 0 Thank You 05/05/1999Dr.Said Alavi 0