rheumatic fever rheumatic fever. 05/05/1999dr.said alavi2 etiology acute rheumatic fever is a...

Rheumatic Fever

05/05/199905/05/1999 Dr.Said AlaviDr.Said Alavi 22

EtiologyEtiology Acute rheumatic fever is a systemic disease of Acute rheumatic fever is a systemic disease of

childhood,often recurrent that follows group childhood,often recurrent that follows group A beta hemolytic streptococcal infectionA beta hemolytic streptococcal infection

It is a delayed non-suppurative sequelae to It is a delayed non-suppurative sequelae to URTI with GABH streptococci. URTI with GABH streptococci.

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


EpidemiologyEpidemiology

Ages 5-15 yrs are most susceptibleAges 5-15 yrs are most susceptible Rare <3 yrsRare <3 yrs Girls>boysGirls>boys Common in 3rd world countriesCommon in 3rd world countries Environmental factors--Environmental factors-- over crowding, over crowding,

poor sanitation, poverty,poor sanitation, poverty, Incidence more during fall ,winter & early Incidence more during fall ,winter & early

springspring


PathogenesisPathogenesis

Delayed immune response to infection with Delayed immune response to infection with group.A beta hemolytic streptococci.group.A beta hemolytic streptococci.

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


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

Pharyngitis- Pharyngitis- produced by GABHS can lead produced by GABHS can lead to- acute rheumatic fever ,to- acute rheumatic fever , rheumatic heart disease & rheumatic heart disease & post post strept. Glomerulonepritisstrept. Glomerulonepritis

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


Clinical FeaturesClinical Features

Migratory polyarthritis, involving major jointsMigratory polyarthritis, involving major joints Commonly involved joints-knee,ankle,elbow Commonly involved joints-knee,ankle,elbow

& wrist& wrist Occur in 80%,involved joints are exquisitely Occur in 80%,involved joints are exquisitely

tendertender In children below 5 yrs arthritis usually mild In children below 5 yrs arthritis usually mild

but carditis more prominentbut carditis more prominent Arthritis do not progress to chronic diseaseArthritis do not progress to chronic disease

1.Arthritis


Clinical Features (Contd)Clinical Features (Contd)

Manifest as Manifest as pancarditispancarditis(endocarditis, (endocarditis, myocarditis and pericarditis),occur in 40-myocarditis and pericarditis),occur in 40-50% of cases50% of cases

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

Valvulitis occur in acute phaseValvulitis occur in acute phase Chronic phase- fibrosis,calcification & Chronic phase- fibrosis,calcification &

stenosis of heart valves.stenosis of heart valves.

2.Carditis


Clinical Features (Contd)Clinical Features (Contd)Clinical Features (Contd)Clinical Features (Contd)

Occur in 5-10% of casesOccur in 5-10% of cases Mainly in girls of 1-15 yrs ageMainly in girls of 1-15 yrs age May appear even 6 months after the attack May appear even 6 months after the attack

of rheumatic feverof rheumatic fever 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



Occur in <5%.Occur in <5%. Unique, transient lesions of 1-2 inches in Unique, transient lesions of 1-2 inches in

sizesize Pale center with red irregular marginPale center with red irregular margin More on trunks & limbs & non-itchyMore on trunks & limbs & non-itchy Worsens with application of heatWorsens with application of heat Often associated with chronic carditisOften associated with chronic carditis

4.Erythema Marginatum



Occur in 10%Occur in 10% Painless,pea-sized,palpable nodulesPainless,pea-sized,palpable nodules Mainly over extensor surfaces of Mainly over extensor surfaces of

joints,spine,scapulae & scalpjoints,spine,scapulae & scalp Associated with strong seropositivityAssociated with strong seropositivity Always associated with severe carditisAlways associated with severe carditis

5.Subcutaneous nodules



Other features (Minor features)

Fever Fever –– Low grade Low grade ArthralgiaArthralgia PallorPallor AnorexiaAnorexia Loss of weightLoss of weight


Laboratory FindingsLaboratory Findings High ESRHigh ESR Anemia, leucocytosisAnemia, leucocytosis Elevated C-reactive protienElevated C-reactive protien ASO titre >200. ASO titre >200. (Peak (Peak

value attained at 3 weeks,then value attained at 3 weeks,then comes comes down to normal by 6 weeks)down to normal by 6 weeks)

Anti-DNAse B testAnti-DNAse B test Throat culture-GABHstreptococciThroat culture-GABHstreptococci


Laboratory Findings (Contd)Laboratory Findings (Contd) ECG- prolonged PR intervalECG- prolonged PR interval Echo - valve edema,mitral regurgitation, LA & Echo - valve edema,mitral regurgitation, LA &

LV dilatation,pericardial effusion,decreased LV dilatation,pericardial effusion,decreased contractilitycontractility


DiagnosisDiagnosis Rheumatic fever is mainly a clinical diagnosisRheumatic fever is mainly a clinical diagnosis No single diagnostic sign or specific No single diagnostic sign or specific

laboratory test available for diagnosislaboratory test available for diagnosis Diagnosis based on Diagnosis based on MODIFIED JONES MODIFIED JONES

CRITERIACRITERIA


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


TreatmentTreatment Step IStep I - primary prevention - primary prevention

(eradication of streptococci)(eradication of streptococci) Step IIStep II - anti inflammatory treatment - anti inflammatory treatment

(aspirin,steroids)(aspirin,steroids) Step IIIStep III- supportive management & - supportive management &

management of complications management of complications Step IVStep IV- secondary prevention - secondary prevention

(prevention of recurrent attacks)(prevention of recurrent attacks)


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


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


Bed rest Bed rest Treatment of congestive cardiac failure: Treatment of congestive cardiac failure:

--digitalis,diureticsdigitalis,diuretics Treatment of chorea:Treatment of chorea:

- -diazepam or haloperidoldiazepam or haloperidol Rest to joints & supportive splintingRest to joints & supportive splinting

3.Step III: Supportive management & management of complications


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


PrognosisPrognosis

Rheumatic fever can recur whenever the Rheumatic fever can recur whenever the individual experience new GABH individual experience new GABH streptococcal infection,if not on prophylactic streptococcal infection,if not on prophylactic medicinesmedicines

Good prognosis for older age group & if no Good prognosis for older age group & if no carditis during the initial attackcarditis during the initial attack

Bad prognosis for younger children & those Bad prognosis for younger children & those with carditis with valvar lesionswith carditis with valvar lesions

rheumatic fever rheumatic fever. 05/05/1999dr.said alavi2 etiology acute rheumatic fever is a...

Documents

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