Rheumatic fever

Rheumatic FeverRheumatic Fever

05/05/199905/05/1999 Dr.Said AlaviDr.Said Alavi 22
EtiologyEtiology
 Acute rheumatic fever is a systemic disease ofAcute rheumatic fever is a systemic disease of
childhood,often recurrent that follows groupchildhood,often recurrent that follows group
A beta hemolytic streptococcal infectionA beta hemolytic streptococcal infection
 It is a delayed non-suppurative sequelae toIt is a delayed non-suppurative sequelae to
URTI with GABH streptococci.URTI with GABH streptococci.
 It is a diffuse inflammatory disease ofIt is a diffuse inflammatory disease of
connective tissue,primarily involvingconnective tissue,primarily involving
heart,blood vessels,joints, subcut.tissue andheart,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, poorover crowding, poor
sanitation, poverty,sanitation, poverty,
 Incidence more during fall ,winter & earlyIncidence more during fall ,winter & early
springspring

PathogenesisPathogenesis
 Delayed immune response to infection withDelayed immune response to infection with
group.A beta hemolytic streptococci.group.A beta hemolytic streptococci.
 After a latent period of 1-3 weeks, antibodyAfter a latent period of 1-3 weeks, antibody
induced immunological damage occur toinduced immunological damage occur to
heart valves,joints, subcutaneous tissueheart valves,joints, subcutaneous tissue
& basal ganglia of brain& basal ganglia of brain

 Strains that produces rheumatic fever -Strains that produces rheumatic fever - MM
types l, 3, 5, 6,18 & 24types l, 3, 5, 6,18 & 24
 Pharyngitis-Pharyngitis- produced by GABHS can leadproduced by GABHS can lead
to- acute rheumatic fever ,to- acute rheumatic fever ,
rheumatic heart disease &rheumatic heart disease &
post strept. Glomerulonepritispost strept. Glomerulonepritis
 Skin infection-Skin infection- produced by GABHS leads toproduced by GABHS leads to
post streptococcal glomerulo nephritis only. Itpost 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,elbowCommonly involved joints-knee,ankle,elbow
& wrist& wrist
 Occur in 80%,involved joints are exquisitelyOccur in 80%,involved joints are exquisitely
tendertender
 In children below 5 yrs arthritis usually mildIn 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 (ContdClinical Features (Contd((

Manifest asManifest 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 ofCarditis 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

 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 attackMay appear even 6 months after the attack
of rheumatic feverof rheumatic fever
 Clinically manifest as-clumsiness,Clinically manifest as-clumsiness,
deterioration of handwriting,emotionaldeterioration 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 inUnique, 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 ofMainly 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(
 FeverFever –– Low gradeLow 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 value attained at 3 weeks,then(Peak value attained at 3 weeks,then comescomes
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 (ContdLaboratory 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,decreasedLV dilatation,pericardial effusion,decreased
contractilitycontractility

DiagnosisDiagnosis
 Rheumatic fever is mainly a clinical diagnosisRheumatic fever is mainly a clinical diagnosis
 No single diagnostic sign or specificNo single diagnostic sign or specific
laboratory test available for diagnosislaboratory test available for diagnosis

Diagnosis based onDiagnosis based on MODIFIED JONESMODIFIED JONES
CRITERIACRITERIA

Jones Criteria (Revised( for Guidance in the
Diagnosis of Rheumatic Fever*
Major Manifestation Minor
Manifestations
Supporting Evidence
of Streptococal Infection
Clinical LaboratoryCarditis
Polyarthritis
Chorea
Erythema Marginatum
Subcutaneous Nodules
Previous
rheumatic
fever or
rheumatic
heart disease
Arthralgia
Fever
Acute phase
reactants:
Erythrocyte
sedimentation
rate,
C-reactive
protein,
leukocytosis
Prolonged P-
R interval
Increased Titer of Anti-
Streptococcal Antibodies ASO
(anti-streptolysin O(,
others
Positive Throat Culture
for Group A Streptococcus
Recent 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 of
Group 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 complicationsmanagement 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 75-100
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
Step II: Anti inflammatory treatment
Clinical condition Drugs

 Bed restBed 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
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

PrognosisPrognosis
 Rheumatic fever can recur whenever theRheumatic fever can recur whenever the
individual experience new GABHindividual experience new GABH
streptococcal infection,if not on prophylacticstreptococcal infection,if not on prophylactic
medicinesmedicines
 Good prognosis for older age group & if noGood prognosis for older age group & if no
carditis during the initial attackcarditis during the initial attack
 Bad prognosis for younger children & thoseBad prognosis for younger children & those
with carditis with valvar lesionswith carditis with valvar lesions

Rheumatic fever

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