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Dr.Said Alavi
MD,DCH,DNB,FCPS
Dr.Said Alavi
MD,DCH,DNB,FCPS
Dept. of Pediatrics and NeonatologyDept. of Pediatrics and Neonatology
Saqr Hospital,Ras Al KhaimahSaqr Hospital,Ras Al Khaimah
UNITED ARAB EMIRATESUNITED ARAB EMIRATES
E-mail: drsaid@emirates.net.aeE-mail: drsaid@emirates.net.ae
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ObjectivesObjectivesObjectivesObjectives Etiology Epidemiology Pathogenesis Pathologic lesions Clinical manifestations & Laboratory
findings Diagnosis & Differential diagnosis Treatment & Prevention Prognosis References
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EtiologyEtiology 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
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EpidemiologyEpidemiology
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 spring
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PathogenesisPathogenesis
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
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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
Group A Beta Hemolytic Streptococcus
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Diagrammatic structure of the group A beta hemolytic streptococcus
Capsule
Cell wall
Protein antigens
Group carbohydrate
Peptidoglycan
Cyto.membrane
Cytoplasm
…………………………………………………...
Antigen of outer protein cell wall of GABHS induces antibody response in victim which result in autoimmune damage to heart valves, sub cutaneous tissue,tendons, joints & basal ganglia of brain
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Pathologic LesionsPathologic 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
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Rheumatic Carditis Histology (40X)
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Histology of Myocardium in Rheumatic Carditis (200X)
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Clinical FeaturesClinical Features
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
1.Arthritis
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Clinical Features (Contd)Clinical Features (Contd)
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)
2.Carditis
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Rheumatic heart disease. Abnormal mitral valve. Thick, fused chordae
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Another view of thick and fused mitral valves in Rheumatic heart disease
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Clinical Features (Contd)Clinical Features (Contd)
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
3.Sydenham Chorea
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Clinical Features (Contd)Clinical Features (Contd)
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 carditis
4.Erythema Marginatum
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Clinical Features (Contd)Clinical Features (Contd)
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 carditis
5.Subcutaneous nodules
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Clinical Features (Contd)Clinical Features (Contd)
Other features (Minor features)
Fever-(upto 101 degree F) Arthralgia Pallor Anorexia Loss of weight
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Laboratory FindingsLaboratory 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-GABHstreptococci
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Laboratory Findings (Contd)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
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DiagnosisDiagnosis Rheumatic fever is mainly a clinical
diagnosis No single diagnostic sign or specific
laboratory test available for diagnosis Diagnosis based on MODIFIED
JONES CRITERIA
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Jones Criteria (Revised) for Guidance in theDiagnosis of Rheumatic Fever*
Major Manifestation MinorManifestations
Supporting Evidence of Streptococal Infection
Clinical LaboratoryCarditisPolyarthritis
ChoreaErythema Marginatum
Subcutaneous Nodules
Previousrheumaticfever orrheumaticheart diseaseArthralgiaFever
Acute phasereactants:Erythrocytesedimentationrate, C-reactiveprotein,leukocytosis Prolonged P-R interval
Increased Titer of Anti-Streptococcal Antibodies ASO (anti-streptolysin O),othersPositive Throat Culture for Group A StreptococcusRecent Scarlet Fever
*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 ofGroup A streptococcal nfection.
Recommendations of the American Heart Association
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Exceptions to Jones CriteriaExceptions 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
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Differential DiagnosisDifferential Diagnosis
Juvenile rheumatiod arthritis Septic arthritis Sickle-cell arthropathy Kawasaki disease Myocarditis Scarlet fever Leukemia
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TreatmentTreatment 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)
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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 Association
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Arthritis only Aspirin 75-100mg/kg/day,give as 4divided doses for 6weeks(Attain a blood level 20-30 mg/dl)
Carditis Prednisolone 2-2.5mg/kg/day, give as twodivided doses for 2weeksTaper over 2 weeks &while tapering addAspirin 75 mg/kg/dayfor 2 weeks.Continue aspirin alone100 mg/kg/day foranother 4 weeks
Step II: Anti inflammatory treatmentClinical condition Drugs
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Bed rest Treatment of congestive cardiac failure:
-digitalis,diuretics Treatment of chorea:
-diazepam or haloperidol Rest to joints & supportive splinting
3.Step III: Supportive management & management of complications
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STEP IV : Secondary Prevention of Rheumatic Fever (Prevention of Recurrent Attacks)
Agent Dose Mode
Benzathine 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 sulfadiazine
Erythromycin 250 mg twice daily Oral
*In high-risk situations, administration every 3 weeks is justified and recommended
Recommendations of American Heart Association
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Duration of Secondary Rheumatic Fever 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
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PrognosisPrognosis
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
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ReferencesReferencesHoffman JIE: Rheumatic Fever . Rudolph's Pediatrics; 20th Ed: 1518 - 1521,1996.
Stollerman GH: Rheumatic Fever . Harrison's Principles Of Internal Medicine; 13th Ed: 1046 - 1052,1995.
Special Writing 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.
World Health Organization Study Group: Rheumatic Fever & Rheumatic Heart Disease,technical Report Series No. 764.Geneva,world Health Organization, 1988
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