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Rheumatic FeverRheumatic Fever 05/05/199905/05/1999Dr.Said AlaviDr.Said Alavi 2 2 EtiologyEtiology n n Acute rheumatic fever is a systemic disease of Acute rheumatic fever is a systemic disease of childhood,often recurrent that follows group A childhood,often recurrent that follows group A beta hemolytic streptococcal infectionbeta hemolytic streptococcal infection n n It is a delayed non-suppurative sequelae to It is a delayed non-suppurative sequelae to URTI with GABH streptococci. URTI with GABH streptococci. n n It is a diffuse inflammatory disease of It is a diffuse inflammatory disease of connective tissue,primarily involving connective tissue,primarily involving heart,blood vessels,joints, subcut.tissue and heart,blood vessels,joints, subcut.tissue and CNSCNS 05/05/199905/05/1999Dr.Said AlaviDr.Said Alavi 3 3 EpidemiologyEpidemiology n n Ages 5-15 yrs are most susceptibleAges 5-15 yrs are most susceptible n n Rare boysGirlsboys n n Common in 3rd world countriesCommon in 3rd world countries n n Environmental factors-Environmental factors-over crowding, poor over crowding, poor sanitation, poverty,sanitation, poverty, n n Incidence more during fall ,winter & early Incidence more during fall ,winter & early springspring 05/05/199905/05/1999Dr.Said AlaviDr.Said Alavi 4 4 PathogenesisPathogenesis n n Delayed immune response to infection with Delayed immune response to infection with group.A beta hemolytic streptococci.group.A beta hemolytic streptococci. n n After a latent period of 1-3 weeks, antibody After a latent period of 1-3 weeks, antibody induced immunological damage occur toinduced immunological damage occur to heart valves,joints, subcutaneous tissue heart valves,joints, subcutaneous tissue & basal ganglia of brain& basal ganglia of brain 05/05/199905/05/1999Dr.Said AlaviDr.Said Alavi 5 5 n n Strains that produces rheumatic fever - Strains that produces rheumatic fever - M M types l, 3, 5, 6,18 & 24types l, 3, 5, 6,18 & 24 n n Pharyngitis- Pharyngitis- produced by GABHS can lead to- produced by GABHS can lead to- acute rheumatic fever ,acute rheumatic fever , rheumatic heart disease & rheumatic heart disease & post strept. Glomerulonepritispost strept. Glomerulonepritis n n Skin infection-Skin infection- produced by GABHS leads to produced by GABHS leads to post streptococcal glomerulo nephritis only. It post streptococcal glomerulo nephritis only. It will not result in Rh.Fever or carditiswill not result in Rh.Fever or carditis Group A Beta Hemolytic Streptococcus 05/05/199905/05/1999Dr.Said AlaviDr.Said Alavi 6 6 Clinical FeaturesClinical Features n n Migratory polyarthritis, involving major jointsMigratory polyarthritis, involving major joints n n Commonly involved joints-knee,ankle,elbow Commonly involved joints-knee,ankle,elbow & wrist& wrist n n Occur in 80%,involved joints are exquisitely Occur in 80%,involved joints are exquisitely tendertender n n In children below 5 yrs arthritis usually mild In children below 5 yrs arthritis usually mild but carditis more prominentbut carditis more prominent n n Arthritis do not progress to chronic diseaseArthritis do not progress to chronic disease 1.Arthritis 05/05/199905/05/1999Dr.Said AlaviDr.Said Alavi 7 7 Clinical Features (Contd)Clinical Features (Contd) n n Manifest as Manifest as pancarditispancarditis(endocarditis, (endocarditis, myocarditis and pericarditis),occur in 40-myocarditis and pericarditis),occur in 40- 50% of cases50% of cases n n Carditis is the only manifestation of Carditis is the only manifestation of rheumatic fever that leaves a sequelae & rheumatic fever that leaves a sequelae & permanent damage to the organpermanent damage to the organ n n Valvulitis occur in acute phaseValvulitis occur in acute phase n n Chronic phase- fibrosis,calcification & Chronic phase- fibrosis,calcification & stenosis of heart valves.stenosis of heart valves. 2.Carditis 05/05/199905/05/1999Dr.Said AlaviDr.Said Alavi 8 8 Clinical Features (Contd)Clinical Features (Contd) n n Occur in 5-10% of casesOccur in 5-10% of cases n n Mainly in girls of 1-15 yrs ageMainly in girls of 1-15 yrs age n n May appear even 6 months after the attack May appear even 6 months after the attack of rheumatic feverof rheumatic fever n n Clinically manifest as-clumsiness, Clinically manifest as-clumsiness, deterioration of handwriting,emotional deterioration of handwriting,emotional lability or grimacing of facelability or grimacing of face 3.Sydenham Chorea 05/05/199905/05/1999Dr.Said AlaviDr.Said Alavi 9 9 Clinical Features (Contd)Clinical Features (Contd) n n Occur in 200. ASO titre 200. (Peak value attained at 3 weeks,then (Peak value attained at 3 weeks,then comes down to normal by 6 weeks)comes down to normal by 6 weeks) n n Anti-DNAse B test Anti-DNAse B test n n Throat culture-GABHstreptococci Throat culture-GABHstreptococci 05/05/199905/05/1999Dr.Said AlaviDr.Said Alavi1313 Laboratory Findings (Contd)Laboratory Findings (Contd) n n ECG- prolonged PR intervalECG- prolonged PR interval n n Echo - valve edema,mitral regurgitation, LA & Echo - valve edema,mitral regurgitation, LA & LV dilatation,pericardial effusion,decreased LV dilatation,pericardial effusion,decreased contractilitycontractility 05/05/199905/05/1999Dr.Said AlaviDr.Said Alavi1414 DiagnosisDiagnosis n n Rheumatic fever is mainly a clinical diagnosisRheumatic fever is mainly a clinical diagnosis n n No single diagnostic sign or specific No single diagnostic sign or specific laboratory test available for diagnosislaboratory test available for diagnosis n n Diagnosis based on Diagnosis based on MODIFIED JONES MODIFIED JONES CRITERIACRITERIA 05/05/199905/05/1999Dr.Said AlaviDr.Said Alavi1515 Recommendations of the American Heart Association 05/05/199905/05/1999Dr.Said AlaviDr.Said Alavi1616 TreatmentTreatment n n Step IStep I - primary prevention - primary prevention (eradication of streptococci)(eradication of streptococci) n n Step IIStep II - anti inflammatory treatment - anti inflammatory treatment (aspirin,steroids)(aspirin,steroids) n n Step IIIStep III- supportive management & - supportive management & management of complications management of complications n n Step IVStep IV- secondary prevention - secondary prevention (prevention of recurrent attacks)(prevention of recurrent attacks) 05/05/199905/05/1999Dr.Said AlaviDr.Said Alavi1717 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 05/05/199905/05/1999Dr.Said AlaviDr.Said Alavi1818 Step II: Anti inflammatory treatment Clinical condition Drugs 05/05/199905/05/1999Dr.Said AlaviDr.Said Alavi1919 n n Bed rest Bed rest n n Treatment of congestive cardiac failure: Treatment of congestive cardiac failure: - -digitalis,diureticsdigitalis,diuretics n n Treatment of chorea:Treatment of chorea: - -diazepam or haloperidoldiazepam or haloperidol n n Rest to joints & supportive splintingRest to joints & supportive splinting 3.Step III: Supportive management & management of complications 05/05/199905/05/1999Dr.Said AlaviDr.Said Alavi2020 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
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