ACUTERHEUMATICFEVER

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 RHEUMATICFEVER

Licksthe joint,bitestheheart

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 INTRODUCTION

LeadingcauseofAcquiredHeartdisease

Incidence:Peak515yrs.

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 ORGANISM

GroupAbetahemolyticstreptococci

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 DISEASESCAUSEDBYGROUPASTREPTOCOCCI

Pharyngitis
Impetigo/pyoderma
Pneumonia,Necrotizingfasciitis
Rheumaticfever
Glomerulonephritis
Osteomyelitis
Scarletfever&erysipelas
Toxicshocksyndrome

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 RHEUMATICFEVER

NonsuppurativecomplicationsofGroupA
streptococcalpharyngitis
Latentperiodof13weeks
Adelayedimmuneresponsecausedbyantibody
crossreactivitythatcaninvolvetheheart,joints,
skin,andbrain(basalganglia)
SerotypesMtypes(1,3,5,6,18,24)

IAPUGTeachingslides201516 6
 INTRODUCTION

UntreatedGroupAbetahemolyticstreptococcal
infection
isthecommonestantecedenteventthat
precipitatesanattackofARF.

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 EPIDEMIOLOGY

PrevalenceRHDIndia0.5%.

About50%ofchildrenwithARFwillsufferfrom

RHD

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 EPIDEMIOLOGY

SexBothsexEquallyAffected
DependsonIndividualSusceptibility
SeasonWinter
PredisposingFactors
1.LowSocioeconomicstatus
2.Overcrowding
3.PoorMedicalCare

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 DRAMATICDECLINEINDEVELOPED
COUNTRIES

AAntibioticcoveragehasincreased

BBetterhousing

CConditions(economic&health)haveimproved

DDecreasedbacterialvirulence

EEasyaccesstomedicalcare

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 CLINICALFEATURES&DIAGNOSIS

Jonescriteria(updatedin1992)
5Major
4Minor
2majorsor
1major&2minors
with
Evidenceof(microbiologicorserologic)ofrecent
GroupAbetahemolyticstreptococcalinfection

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 DJC

NotmeantasasubstituteforJudgementbyclinician.
Intendedguidelinestorestrictthediagnosistoan
acceptableclinicalgroup

DJC Judgement

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 WHYJONESCRITERIA

ThereisnospecificlabtestindiagnosisofRF
Aim
Avoidoverdiagnosis
Minimizemissinganopportunityforthesafety
netofsecondaryprophylaxis
AvoidoverdiagnosisinJudgement

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 MAJORS

Carditis Mnemonic
Polyarthritis C2ASE
Erythemamarginatum CCarditis
Subcutaneousnodules CChorea
Chorea AArthritis
SSubcutaneous
nodules
EErythema
marginatum

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 MINORS

Clinicalfeatures
Fever
Arthralgia(intheabsenceofpolyarthritis)

Laboratoryfeatures
Elevatedacutephasereactants
RaisedESR,RaisedCRP
ProlongedPRinterval

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 Evidenceof(microbiologicorserologic)ofrecentGroupA
betahemolyticstreptococcalinfection
(Essentialcriteria)

RaisedorRisingASOTiters
PositiveThroatCultureforGAS
Rapidstreptococcalantigentest
AntiDNaseB,antihyaluronidase
H/oRecentScarletFever

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 3CircumstancesWhereARFdiagnosed
withoutstrictadherencetoJonescriteria

Indolentcarditismaybesolemanifestation
Choreamaybethesolemanifestation
ARFRecurrencemaynotfulfilltheJonescriteria

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 ARTHRITIS

MostCommon30to50%
Largejointinvolvement
MigratoryPolyarthritis
DramaticresponsetoAspirin
Sacroiliac,TemporomandibularandCervicaljointsnot
involved
NoPermanentSequelae
ResolvesinSixweeks

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 CARDITISPRESENTATION

Tachycardiaoutofproportiontofever
Sleepingpulserateraised.

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 CARDITIS

5060%
UsuallyPancarditis
Pericarditisneveroccursinisolation
VariableSeverity
ClinicalSigns
PericarditisEffusion,Rub,Pain
MyocarditisTachycardia,Arrhythmia,
cardiomegaly,failure
EndocarditisMurmurs
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 MURMUR

Highpitchedapicalholosystolicmurmurradiatingtoaxilla

Mitralregurgitation.

Anapicalmiddiastolicmurmur.

Highpitcheddecrescendodiastolicmurmuruppersternal

border

Aorticregurgitation.

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 CARDITISSEQUELAE(CHRONIC)

Mitralinsufficiency
Somelossofvalvularsubstance
Shortening&thickeningofChordaetendinae

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 CARDITISSEQUALAE

Mitralstenosis
TakeslongerdurationtodevelopafteranattackofARF
Fibrosisofmitralring,commissuraladhesions
Contractureofthevalveleaflets,chordae&papillary
muscles
Openingsnap,lowpitched,rumblingmitraldiastolic
murmurwithpresystolicaccentuationendinginloud
firstsound

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 CARDITISSEQUELAE(CHRONIC)CONTD..

Aorticinsufficiency

Sclerosisofaorticvalvedistortion&retractionof
thecup
Characteristicearlydiastolicmurmur
Anapicalpresystolicmurmur(Austinflint)

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 SYDENHAMSCHOREA

1015%ofpatients

Usuallydelayed/oftensolemanifestationofARF

Involuntarymovementsofthefaceandlimbs,

Muscleweakness

Disturbancesofspeechandgait,

Poorscholasticperformance

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 SYDENHAMSCHOREA

Milkmaid'sgrip
irregularcontractionsofthemusclesofthehands
whilesqueezingtheexaminer'sfingers
Spooningandpronationofthehandswhenthe
patient'sarmsareextended(St.VitusDance)
Wormianmovementsofthetongueuponprotrusion
(JackintheBox)
Handwritingtoevaluatefinemotormovements

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 SYDENHAMSCHOREA

Facialgrimacing

Emotionalliability

Exacerbatedbystress

Disappearatsleep

Rarelyleadstopermanentneurologicalsequelae

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 CHOREA

Seenmoreinfemales
Minimum3monthsaftersorethroat

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 ERYTHEMAMARGINATUM

Occurs<10%ofPatients
Macularnonpruriticrash
Serpiginousborder,raisededges,centralclearing
Mostcommonontrunk
Neverseenonface
Evanescent,warmthaccentuateslesion

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ERYTHEMAMARGINATUM

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ERYTHEMAMARGINATUM

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Erythemamarginatumonthetrunk,showingerythematouslesions
withpalecentersandroundedorserpiginousmargins
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Closerviewoferythemamarginatuminthesamepatient
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 SUBCUTANEOUSNODULES

Seeninaround5%

Small,peasized,0.5to2cmsindiameter

Firm,mobile,PAINLESS

SeenovertheextensorsurfaceofWRIST,ELBOW,SPINE

Usuallyseeninindividualswithlongstandingcarditis

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SUBCUTANEOUS
NODULE

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Subcutaneousnoduleontheextensorsurfaceofelbowofa
patientwithacuterheumaticfever
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 LINKOFCARDITISWITH

Polyarthritis - 50 - 75 %
SC Nodule - > 95 %
Chorea - 60 - 75 %

HALF ARE
INAPPARENT
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 DIFFERENTIALDIAGNOSIS
ArthritisRheumatoidarthritis(JRA)
SLE
Reactivearthritis
Shigella,Salmenolosis,Yersenia
Lymesdisease
Chikungunya
Carditisviralmyocarditis,&Pericarditis
Infectiveendocarditis
Congenitalheartlesions
ChoreaHuntingtonchorea
Wilsondisease
Tics
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 INVESTIGATIONS

Testsreflectingtissueinflammation
TC,DC,ESR,CRP(acutephasereactants)

TeststoProveSTREPInfection
ASOTiter,AntiDNAase,Streptozyme,AntiStreptokinase
ThroatCulture

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 INVESTIGATION

Xraychest
ECG
ECHO
Doppler
Bloodculture
Catheterizationstudies

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Chestradiographofan8yearoldpatientwithacute
carditisbeforetreatment
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Chestradiographofan8yearoldpatientwithacute
carditisaftertreatment
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 MANAGEMENT

Bedrest
EradicationofStreptococci
Antiinflammatorytherapy
TreatmentofCCF
TreatmentofChorea
PreventionofRecurrences
SurgicalAcuteandChronic
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 ARTHRITIS

Bedrest
Aspirinonlyfor46Weeks
Localmeasures

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 CARDITIS

CarditisaloneASPIRINOnly

CarditiswithmildCardiomegalyASPIRINOnly

CarditisinfailureASPIRINwithSTEROIDSanddecongestive
measures
Bedrest
Lowsodiumdiet

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 SYDENHAMSCHOREATREATMENT

Antiinflammatoryagentsusuallynotrequired

Phenobarbitol1530mgtidorqidoraldrugofchoice

Haloperidol0.010.03mg/kg/24hrsbdoral

Chlorpromazine0.5mg/kgevery46hrs.oral

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 PREVENTIONINRF

Primordial PreventingStrepThroatvaccine?

Primary TreatingStrepThroatinfection

Secondary PreventingRheumaticrecurrence
bychemoprophylaxis

Tertiary TreatingRHD
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 PRIMARYPROPHYLAXIS

TreatingStreptococcalsorethroatwithAntibiotics
1. OralPenicillin
2. ProcainePenicillinIM
3. BenzathinePenicillin
4. OralErythromycin

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 PRIMARYPROPHYLAXIS

Vulnerablechildrenfrom5to15yrs.withpharyngitis

Oral
Penicillin250500mgbd/tds10days
Erythromycin2040mg/kg/daytds/qid10days
FirstgenerationCephalosporin10days
Azithromycin12mg/kg/daysingledose5daysmax500mg/day

Parenteral
<than27kgsingledoseIMBenzathinepenicillin6,00,000U
>than27kgsingledoseIMBenzathinepenicillin1,20,0000U

Therapyinstitutedbefore9thdayofsymptomsofacutePharyngitis
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 SECONDARYPROPHYLAXIS

Topreventrecurrences
BenzathinePenicillinoncein3weeksIM
OralPenicillindaily
Erythromycindaily
Sulfadiazinedaily

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 SECONDARYPROPHYLAXIS

Route Antibiotic Dose Frequenc

y
IM Benzathine 1200000 Every 3rd
Penicillin U wk.
Oral Penicillin V 250mg BD daily

Oral Erythromycin 250mg BD daily

Oral Sulphadiazine 500mg to BD daily

1000mg 53
 HOWLONGTOGIVE

RF;NoCarditis5yearsfromlastEpisode
ortill21years
RF;Carditis;
NoresidualRHD10yearsfromlastepisode;
ortill25years
RFCarditis;RHD10yearsfromlastepisode;
ortill40years/lifelong

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 NEWMODESOFTREATMENT

?IVIG
??Valproateforchorea
?Anticytokinesadjuvants
??OtherNSAIDS

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THANKYOU

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