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AcuteRheumaticFever:GlobalPersistenceofaPreventableDisease
FrancineBonoNeri,MA,RN,PNP

JPediatrHealthCare.201731(3):275284.

AbstractandIntroduction
Abstract

Thepersistenceofacuterheumaticfevercontinuestobeseenglobally.Oncethoughttobeeradicatedinvariouspartsoftheworld,
thediseasecamebackwithavengeancesecondarytoalackofdiligenceonthepartofproviders.Today,theglobalburdenofgroup
Astreptococcalinfection,theculpritofthenumeroussequelaemanifestedinacuterheumaticfever,isconsiderable.Althougha
completelypreventabledisease,rheumaticfevercontinuestoexist.Itisadevastatingdiseasethatinvolveslongterm,multisystem
treatmentandmonitoringforpatientswhowereunsuccessfulateradicatingtheprecipitatinggroupAstreptococcalinfection.
Preventionisthekeytoresolvingthedilemmaofthedisease'sglobalburden,yetthemethodtoyielditspreventionstillremains
unknown.Thus,meticulousattentiontoimplementingpropertreatmentisthemainstayandremainsatoppriority.

Introduction

DespitetheefficacyofantibioticsagainstgroupAStreptococcusspeciesinreducingtheincidenceofacuterheumaticfever(ARF),
theglobalburdenandchronicsequelaeofthediseasecontinuetoexist.AlthoughNorthAmericaandEuropehaveseenareduced
frequencyinacuterheumaticfeveroverthepastseveraldecades(Bach,2015),providersmustnotexcludethisdiagnosisfromtheir
differential.Notwithstandingaccessibilitytoantimicrobials,countriescontinuetoseeARFasamajorcauseofseriousvalvularheart
disease(Parnaby&Carapetis,2010).Researchandnewguidelinescontinuetoevolvebecauseofitspersistentprevalence.

IntheUnitedStates,adeclineinARFwasseeninthe30yearsafterWorldWarII.Theannualoccurrencedroppedbymorethan
90%,whichwasbelievedtoreflectamelioratedlivingconditions,overallimprovedhygiene,andtheuseofantibiotics(Congeni,
1992).Bytheearly1980s,ARFhadreachedsuchanalltimelowintheUnitedStatesthatsomeprovidersbegantoquestiontheir
ardencyintreatingstreptococcalpharyngitis.Itwasnotuntilthemajorepidemicatthebeginningof1984thataresurgencewas
witnessedinvariousregionsoftheUnitedStates(Congeni,Rizzo,Congeni,&Sreenivasan,1987Hosier,Craenen,Teske,&
Wheller,1987Veasyetal.,1987Wald,Dashefsky,Fedit,Chiponis,&Byers,1987)..Unlikethetraditionaloutbreaksfoundin
crowdedandimpoverishedinnercityghettos,thesecasesoccurredprimarilyinchildrenofWhite,middleclassfamilies,manyof
whomresidedinsuburbanorruralenvironments(Congeni,1992).Inthepresentday,althoughisolatedcasesofARFcontinuetobe
seeninmodernizedcountries,mostarefoundincountrieswithlimitedresourcesandinpoorlyrepresentedaboriginalgroups(World
HealthOrganization[WHO],2005).VariousregionsofSouthAmerica,theMiddleEast,India,andAfricaareshowingparticularrisk
ofARFforchildren(Casey,Solomon,Gaziano,Miller,&Loscalzo,2013Tibazarwa,Volmink,&Mayosi,2008).

Globalburdenofdisease,upuntiltheearly1990s,wasassessedusinganarrowcriterion.Globalstudiesofpopulationmortality
werethemainfocus.Thesestudiesfailedtoconsidermorbiditythatarosefromdisordersandinjuriesthatwerenotfatalbut
neverthelessaffectedaperson'sfunctioninginanadversemanner(Degenhardt,Whiteford,&Hall,2014).Measuringtheimpactof
diseaseanditsglobalburdenwasradicallytransformedin1993,whenestimatesofcausesofglobaldiseaseburdenusedanew
summarymeasure.Thisnewmeasure,knownasDisabilityAdjustedLifeYears,"simultaneouslyaccountedforbothpremature
mortalityandtheprevalence,durationandseverityofthenonfatalconsequencesofdiseaseandinjury"(Lopez,2005,Abstract,
para.1).TheWorldHealthReport(WorldHealthOrganization,2005)statesthatapproximately18.1millionpeopleweresuffering
fromaseriousgroupAStreptococcus(GAS)diseaseandthatapproximately1.78millionnewcaseswerebeingseenannuallyasof
2005.Inaddition,thereportalsostatesthatGASinfectionswereidentifiedastheninthleadingcauseofworldwidemortalityfroma
singlepathogen.Globalburden,definedbytheWHOasthoseafflicted,foundanoverallburdenof471,000annualcasesofARF
(Zhlke&Steer,2013).Althoughpromptdiagnosisandtreatmentareimperative,preventionispivotaltosuccess.

WhatIsARF?

ARFisanonsuppurativesequelathatmanifests2to4weeksafteruntreatedpharyngitisandiscausedbyGAS(Caseyetal.,2013).
Somespeculatethatinvariousresourcelimitedtropicalregions,ARFhasbeenseenafterpyoderma(Parks,Smeesters,&Steer,
2012)however,forthepurposeofthisarticle,onlyGASpharyngitiswillbeaddressed.Itspredispositiontowardinflammationof
connectivetissueclassifiesARFasacollagendisease.Theheart,joints,subcutaneoustissue,andcentralnervoussystemare
involved.Wheninvolvementoftheheartispresent,"damagetoaffectedcardiacvalvesmaybechronicandprogressive"(Gibofsky,
2016,para.1),yieldingdeteriorationincardiacfunction.AlthoughtheexactpathogenesisofARFremainsunclear,antibodies
producedafterGASinfectionreactwiththebody'scellsandproducecharacteristiclesionsintargetareas.Themajorbenefitsof
adequatetreatmentforGASpharyngitisisthepreventionofARFandreductionofcommunicability.

BeforeexaminingthesymptomatologyofARF,certainkeypointsneedtobeaddressed.First,acuterheumaticfeveroccursafteran
untreatedGASpharyngitis(Beaudoinetal.,2015).Second,thepreventionofARFcanbeaccomplishedwiththeuseofappropriate
antibiotictreatmentupto9daysaftertheonsetofpharyngitis(Watson,Jallow,Doare,Pushparajah,&Anderson,2015).Third,the
peakincidenceofpharyngitiscausedbyGASisinchildrenages5to15years(Wessels,2011)andaccountsfor20%to30%or
moreofacutepharyngitiscasesinthispopulation(Shulmanetal.,2012).

DiagnosisofGASpharyngitisisparamounttopreventingfuturesequelae.Findingshighlysuggestiveofaviralpharyngitisinclude
conjunctivitis,coryza,cough,hoarseness,anteriorstomatitis,and/ordiarrhea(Shulmanetal.,2012).Clinicalsignssuggestiveof
GASpharyngitisincludefever,headache,palatalpetechiae,patchytonsillopharyngealexudates,anteriorcervicallymphadenitis,

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scarlatiniformrash,nausea,vomiting,abdominalpain,andhistoryofsuddenonsetofsorethroat.Epidemiologicfindingsincludean
ageof5to15yearsold,historyofillcontactswithGASpharyngitis,prevalenceinthecommunity,andseasonaltimingofwinterand
earlyspringforareaswithtemperateclimates(Gerberetal.,2009).Thesefindingsarehighlyindicativeofthediagnosis,yetthe
criterionstandardforconfirmationtodeterminepositiveGAScolonizationisbytherapidantigendetectiontestand/orculture
(Shulmanetal.,2012).Althougharudimentarytest,itmustbecorrectlyexecutedbyvigorouslyswabbingbothtonsilsandthe
posteriorpharynx(Gerberetal.,2009).Withmostrapidantigendetectiontests,anegativeresultdoesnotruleoutthepresenceof
GAStherefore,athroatcultureshouldbeperformedaswell(Gerberetal.,2009).IftheresultsarepositiveforGAS,treatmentmust
beinitiated.Treatmentoptionsareaslistedin.Notonlyisitessentialtoprescribetheappropriateantibioticforyourpatient,butitis
alsocrucialtofindonethatwillelicittotalcompliancetoensureeradication.Hence,somepractitionerswilluseaoncedailydosing
suchasazithromycinoraonetimeintramuscularinjectionwherecompliancewouldhaveposedasamajorconcern.Itisessential
thatproviderseducatepatientsandparentsoncompletetreatmentadherence.

Table1.TreatmentofpharyngitisduetogroupAStreptococcus

Note.ReproducedwithpermissionfromGibofskyA.Acuterheumaticfever:Treatmentandprevention.In:UpToDate,PostTW(Ed),
UpToDate,Waltham,MA.(Accessedon[Date].)Copyrightq2016UpToDate,Inc.Formoreinformationvisitwww.uptodate.com.
Datafrom:Gerberetal.(2009),AmericanAcademyofPediatrics(2015),Shulmanetal.(2012),andCohenetal.(2002).

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WhatIstheClinicalPresentationofARF,andHowIsItDiagnosed?

OnJune16,1944,thefirstspeakerattheAnnualSessionoftheAmericanMedicalAssociationSymposiumonRheumaticFever
wasaphysiciannamedT.DuckettJones.Hepresentedinarevolutionaryandclinicallydocumentedmannerthroughwhichhe
outlinedthediagnosticcriteriaforrheumaticfever.Historically,thesecriteriahavedepictedtheclinicalstandardstoestablishthe
diagnosisofARF.PublicizedintheJournaloftheAmericanMedicalAssociation,theJonescriteriawereaugmentedbytheAmerican
HeartAssociation(AHA)in1992.AtaworkshopsponsoredbytheAHAin2000,thecriteriawerereaccreditedandremainedan
invaluabletoolindiagnosingARF.However,becauseoftheadvancesinimagingstudiesandtheevolvingroleofechocardiography,
areexaminationofthetraditionalJonescriteriawasprecipitated,thusleadingtotheupdatessetforthin2015(Gewitzetal.,2015).

DiagnosisofARFismadeusingtherevisedJonescriteria,whichconsistofthreegroupsofclinicalandlaboratoryfindings.There
arefivemajormanifestations(criteria)andfourminormanifestations(criteria),plustheessentialcriterionofaprecedingGAS
infection.Diagnosisismadewhentwomajormanifestationsoronemajorandtwominormanifestationsarenotedandevidenceof
precedingstreptococcalinfectionexists(Gewitzetal.,2015).Theageofthepatientshouldbeconsidered,becausetheagerangeof
childrenwithARF,coincidingwiththeprevalenceofGASpharyngitis,isapproximately5to15years(Webb,Grant,&Harnden,
2015).Theinitial,nonspecificgroupofsymptomsisagradualonsetoffever,weightloss,andmalaise.Othersignsandsymptoms
includeunexplainedepistaxis,abdominalpain,fatigue,pallor,andanorexia.

MajorManifestations
Carditis

Carditisisseeninapproximately50%to65%ofpatients(Webbetal.,2015),withtypicalpresentationwithin3weeksofan
untreatedGASpharyngitis.Althoughconsideredapancarditisinnature,withinvolvementincludingthepericardium,epicardium,
myocardium,andendocardium,"valvulitisisbyfarthemostconsistentfeatureofARF,andisolatedpericarditisormyocarditisshould
rarely,ifever,beconsideredrheumaticinorigin"(Gewitzetal.,2015,p.1809).Itisdiagnosedasaneworchangedmurmur,recent
orworseningcardiomegaly,pericardialfrictionrub,oreffusionwithorwithoutevidenceofcongestiveheartfailure.Intheabsenceof
auscultatoryfindings,becauseoflackofrecognitiononexaminationorbecausefindingsarenotpresent,subclinicalcarditiscanbe
diagnosedbyechocardiographyandDopplerstudies(Gewitzetal.,2015).

Polyarthritis

Polyarthritisisusuallytheearliestmanifestation,presentingwithin21daysofGASinfection.Seenin50%to70%ofpatients(Gewitz
etal.,2015),itismorecommonlyfoundinteenagersandyoungadults(Gibofsky,2016).Typically,theinflammationaffectsseveral
jointsinquicksuccession,andeachjointisinflamedfor24to48hours,lastinguptoaweek.Aminimumoftwojointsmustbe
involved.Heat,erythema,tenderness,edema,andpainareusuallyallpresent.Arthralgiaaloneisinsufficienttomeetthiscriterion.
Thejointsmostfrequentlyaffectedarethekneesandankles,butinvolvementmayalsoincludetheelbows,hips,shoulders,and
wrists.Themanifestationofarthritisinvariousjointssynchronizeinanoverlappingsequence,thusgivingtheappearancethatthe
diseaseismigratoryortransitoryinpattern.Thearthritisresolveswithouttreatmentinapproximately4weeks,andthereisnolong
termjointdeformityorresidualdeficits(WHO,2004).

Sydenham'sChorea

Sydenham'schorea(SC),alsoknownasSt.Vitusdance,isseenin10%to30%ofpatients(Gewitzetal.,2015)."SCisapediatric
hyperkineticmovementdisorderpresentingweeksaftergroupAstreptococcalinfectionandisthemostcommonformofacquired
choreainchildhood"(BenPazi,Kroyzer,&Hashkes,2012,p.211).Itisaneurologicsequelamanifestingasmyastheniaemotional
instabilityandspontaneous,involuntarymovementslackingaconsistentrhythm.Choreahasamoredormantperiodthantheother
manifestations,typicallypresenting1to6monthsafteraGASinfection(Meyers,Kane,Porter,&Mazzaccaro,2014).Femalesare
morefrequentlyaffectedthanmalesbya2:1ratio(Gilbert,2016).Althoughnotinitiallyapparent,thesefindingsbecome
progressivelymoresevereandoftenresultinthedevelopmentofataxiaandslurringofspeech.Muscularweaknessappearsafter
thepresentationofinvoluntarymovementsandmaypresentinaunilateralfashion.Completerecoveryoftentakesseveralmonths,
andsomepatientsmaysufferpermanentneurologicsequelae(Meyersetal.,2014).

ErythemaMarginatum

Erythemamarginatum(Figure1),whichisseeninlessthan6%ofpatients(Gewitzetal.,2015),usuallyoccursearlyinthecourseof
ARF.Itisanonpruritic,transient,macularerythematousrashwithcircinatebordersandpalecenters.Itinvolvesthetrunkand
sometimesthelimbsbutsparestheface(Gibofsky,2016).Insomepatients,lesionswillbenotedlateintheprogressionofthe
illnessorevenduringtheconvalescentperiod.Erythemamarginatumextendsinaserpiginousandcentrifugallikemanner,with
returnofthecenterskintothepatient'sbaselinecolor(WHO,2004).Theoutermarginsarewelldefined,andtheinneraremore
diffuse.Individuallesionsaretransientinnaturethus,appearance,disappearance,andreappearanceisacommonpatternnoted.If
exposedtowarmwaterofabathorshower,theymaybecomemorepronounced.

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Figure1.

Imageoferythemamarginatum.Thephotoontheleftisoferythemamarginatumshowingerythematouslesionswithpale
centersandroundedorserpiginousmargins.Thephotoontherightisacloserviewoferythemamarginatuminthesame
patient.
AdaptedfromBinotto,M.A.,Guilherme,L.,andTanka,A.(2002)).Rheumaticfever.ImagesinPaediatricCardiology,4(2),1231.
Reprintedwithpermission.Thisfigureappearsincoloronlineatwww.jpedhc.org.

SubcutaneousNodules

Subcutaneousnodules(Figure2),seenin0%to10%ofpatients(Gewitzetal.,2015),arefirm,nontender,freelymobilenodulesin
thesubcutaneoustissuerangingfromafewmillimetersto2cminsize."Theyrarelyoccurasanisolatedmanifestation,andtheyare
associatedwithcarditisinmostcases"(Singhi,Bobhate,&Kappanayil,2010,p.946).Theappearanceofthesenodulesisusually
seeninpatientswithmoreseverecasesandarelocatedoverabonyprominenceorneartendons.Rarelyaretheynotedtopersist
indurationformorethanamonth(Gibofsky,2016).Theyaregenerallysymmetric,withamarkedincreaseininvolvementofthe
elbows.Thevarianceinthenumberoflesionsisfromonetoafewdozen,averagingbetweenthreetofour(WHO,2004).

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Figure2.

Imageofasubcutaneousnodule.Subcutaneousnoduleontheextensorsurfaceoftheelbowofapatientwithacute
rheumaticfever.
AdaptedfromBinotto,M.A.,Guilherme,L.,andTanka,A.(2002).Rheumaticfever.ImagesinPaediatricCardiology,4(2),1231.
Reprintedwithpermission.Thisfigureappearsincoloronlineatwww.jpedhc.org.

MinorManifestations
Fever

Feverof38.5Corhigherisconsideredaminorcriterion.

Polyarthralgia

Polyarthralgiaisamorenonspecificmanifestationintheminorcategory.Thepresentationcanincludeinvolvementoftwoormore
jointswithoutheat,edema,erythema,ortendernessatthesites.

ElevatedAcutePhaseReactants

ElevatedacutephasereactantsarealmostalwaysseeninpatientswithARF,exceptinsomepatientswithisolatedchoreaorin
thosetreatedwithantirheumaticdrugs.Leukocytosis,acceleratedsedimentationrate,and,morespecifically,anelevatedCreactive
proteinlevelarenoted.

ProlongedPRInterval

ProlongedPRinterval,afteraccountingforvariabilityseeninage,maybeseenonanelectrocardiogram.Itisconsideredaminor
manifestation,unlesscarditisisnotedasamajorcriterion.

OthersuggestiveclinicalfeaturesseeninpatientswithARFincludeabdominalpain,precordialpain,malaise,epistaxis,elevationof
pulseatrest,andtachycardiaoutofproportiontofever.

WhatIsAskedWhenTakingtheHistory?
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Whenobtainingthehistory,thepractitionershouldinquireaboutaprecedingsorethroatwithinthepast3months.Ifpresent,onset,
duration,andseverityshouldbediscussed.Iftreatmentwasrendered,thepractitionershouldaskaboutthemedicationgiven,the
durationoftreatment,andifadherencewascomplete.Thepractitionermustalsoinvestigateintothepresence,onset,andduration
ofallmajorandminormanifestationsofARF,aswellasnonspecificcomplaintssuchasweightloss,epistaxis,andabdominalpain.
Practitionersshouldalsoinquireaboutmedicationsusedintherecentpast,includingoverthecountermedicationssuchasaspirin
andnonsteroidalantiinflammatorydrugs,andtherationalefortheiruses.Drugsofthisnaturecanactasantipyreticsandmask
signsofinflammation,therebyskewingclinicalfindings.Practitionersshouldaskaboutpastmedicalhistory,familyhistory,andabout
familymemberswithcardiacdisease.PatientswithahistoryofARFareatriskforsubsequentepisodes,includingGASreinfection.
Suchrepeatepisodesareassociatedwithagreaterprobabilityofseverecardiacinvolvement.PreviousdiagnosisofARForthe
presenceofinactiverheumaticheartdiseaseshouldalertthepractitioner,becauserecurrencesarequitecommon.Inaddition,family
historyofARFshouldheightenthesuspicionforARFinthesettingofsuggestivefeatures.

WhatShouldBeLookedforonPhysicalExamination?

Whenperformingthephysicalexamination,thepractitionershouldobtainvitalsigns,notinginparticularanelevationintemperature
and/orheartrate.Skinshouldbeinspectedforexanthems,nodules,andlesions.Acompleteexaminationofearsandnoseshould
beperformed,asshouldathoroughinspectionoftheoropharynx,withcloseobservationforinjection,exudate,andpetechiae.
Practitionersshouldpalpateforlymphadenopathyandperformacompletecardiacexamination,specificallylisteningformurmursor
otherabnormalfindings.ElectrocardiographyshouldbeperformedtolookforprolongedPRintervals.Practitionersshouldauscultate
thelungs,assessingforadventitioussounds,suchasrales,thatcouldindicatecardiacinsufficiency.Acompleteneuromuscular
examinationshouldbeperformedtolookspecificallyforsignsandsymptomsofchorea.Alljointsshouldbeassessedforedema,
erythema,warmth,andtenderness.

WheretoGoFromHere?

IfARFisatthetopofthedifferentialdiagnosislist,thefollowinglaboratorytestsshouldbeperformed.Arapidantigendetectiontest
and/orthroatculturemustbeperformedtoassessforGAScolonization.Acompletebloodcountwithdifferentialmustbeperformed,
specificallytolookforleukocytosis.Erythrocytesedimentationrate(ESR)andCreactiveproteinlevelmustalsobeobtained.An
erythrocytesedimentationrateof60mmorgreaterand/orCreactiveproteinlevelof3.0mg/dlorgreaterisconsideredindicativein
lowriskpopulations.Formoderateandhighriskpopulations,anerythrocytesedimentationrateof30mmorgreaterand/orC
reactiveproteinlevelof3.0mg/dlorgreaterwouldbesuggestiveofcriteriamet(Bach,2015).Inaddition,antistreptolysinO(ASO)
titerandantideoxyribonucleaseBantibodyarehelpfulmarkersindeterminingprecedingGASpharyngitisinpatientswhoareno
longermanifestingthesymptomatology.ASOisthemeasurementoftheantibodytothestreptolysinOenzyme,whichisproduced
bystreptococci.ElevatedtitersofASOareseeninatleast80%ofpatientswithacuterheumaticfever(Gibofsky,2016).Levelspeak
atapproximately4weeksafterstreptococcalinfection,whichusuallyisduringthesecondorthirdweekofARF.Therefore,collection
ofserumASOtiterswhenthediagnosisofARFisfirstsuspectedmayprovehelpfulwithconfirmationofthediagnosis.Anelevated
antideoxyribonucleaseBantibodylevelisanotherindicatorofpriorGASinfectionthatisparticularlyusefulforpatientsduringthe
convalescentstage(Gilbert,2016).ObtaininganelectrocardiogramtoassessforprolongedPRintervalorotherelectrocardiographic
changeswilldeterminewhetherthisminormanifestationhasbeenmetaccordingtotheJonescriteria.Finally,achestradiograph
needstobedonetoassessforcardiomegalyandincreasedpulmonarymarkings.Ifacardiologydepartmentconsultationis
indicatedtoassesstheextentofanycardiacinvolvement,anechocardiogramandDopplerstudieswouldbedoneatthattime.

HowShouldaPatientWithNewlyDiagnosedARFBeManaged?

Whenthelaboratoryandtestresultsarebackandthediagnosisismade,itistimeformanagement.TreatmentofARFconsistsof
antibioticandantiinflammatorytherapy,aswellassupportivecare,whichmayincludemanagementofheartfailure(Webbetal.,
2015).PatientswithARFreceiveantibiotictherapynotonlytoeradicatecolonizationofGASbuttopreventfutureGASinfectionby
implementinglongtermprophylacticantimicrobials.Treatmentshouldproceedasdescribedformanagementofstreptococcal
pharyngitis(),regardlessofwhetherGASpharyngitisispresentatthetimeofdiagnosis(Gibofsky,2016).

Table1.TreatmentofpharyngitisduetogroupAStreptococcus

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Note.ReproducedwithpermissionfromGibofskyA.Acuterheumaticfever:Treatmentandprevention.In:UpToDate,PostTW(Ed),
UpToDate,Waltham,MA.(Accessedon[Date].)Copyrightq2016UpToDate,Inc.Formoreinformationvisitwww.uptodate.com.
Datafrom:Gerberetal.(2009),AmericanAcademyofPediatrics(2015),Shulmanetal.(2012),andCohenetal.(2002).

AntiinflammatorytherapyisparamountinthesymptomaticmanagementofARF,yetdiagnosismustbeconfirmedbeforeitsinitiation
toavoidmaskingclinicalmanifestations.ThefirstattempttomanageARFsymptomswithsalicylatetherapywasmadein1876bya
Dr.ThomasMacLagan,whogaveittohispatientafterafailedattemptwithalkalis(Doyle,2011Seckeler&Hoke,2011).Although
attemptshavebeenmadewithseveralvariedmedicationsthroughouttheyears,aspirinhasremainedthemainstay(Seckeler&
Hoke,2011).TheWHO(2004)recommends100mg/kg/dayofaspirindividedequallyintofourorfivedosesasthefirstlineof
therapy.Inthepediatricpopulation,dosingmaybeupto125mg/kg/dayandinadults,6to8g/day.Dosesareadjustedtorelieve
symptomswhileavoidingtoxicity.Afterattainingtheinitialdesiredconcentrationfor2weekswithsymptomaticimprovementnoted,
dosageshouldbedecreasedto50to60mg/kg/day.Aspirintherapyshouldbediscontinuedafterthepatientremainsasymptomatic
forapproximately2weeks(Watsonetal.,2015).Inthepresenceofsensitivitiesorallergiestoaspirin,ibuprofenat30mg/kg/dayin
threeequallydivideddosesornaproxenat10to20mg/kg/daydividedintotwodoseshavebeenused(Watsonetal.,2015).
"Patientswithpericarditisorheartfailurerespondfavorablytocorticosteroidscorticosteroidsarealsoadvisableinpatientswhodo
notrespondtosalicylatesandwhocontinuetoworsenanddevelopheartfailuredespiteantiinflammatorytherapy"(WHO,2004,p.
70).Prednisoneat1to2mg/kg/day(maximum=80mg/day),givenoncedailyorintwoequallydivideddoses,isusuallythedrugof
choice(WHO,2004).
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Supportivemodalityoptionsforpatientswithsignificantcarditisincludeoxygen,restrictingsaltandfluidintake,diuretics,digoxin,or
angiotensinconvertingenzymeinhibitors.Ifthelesionsofrheumaticheartdisease(RHD)aregreatlyaffectingapatient's
hemodynamicstatus,catheterizationorsurgerymayberequiredtorepairorreplacethedefectivevalves(WHO,2004).

PatientswithSCshouldbetreatedwhenmarkedimpairmentofmotorfunctionandthepotentialforselfinjuryarepresent.
Managementincludeseliminationofemotionalandphysicalstressandimplementationofsafetyprecautions.Pharmacological
agentsusedinpatientswithseverecasesarelistedinthe.AttentionmustbetakentonotconfuseSCwithpostinfectious
autoimmuneneurologicaldiseases(e.g.,pediatricautoimmuneneuropsychiatricdisordersassociatedwithstreptococcalinfections,
orPANDAS).PostinfectiousautoimmuneneurologicaldiseasesarenowknowntobeoftenassociatedwithantecedentGAS
infection.ThemanifestationsofPANDASincludetics,Tourette'ssyndrome,andobsessivecompulsivebehavior,allofwhichhave
beenobservedinsomepatientsduringorafteranattackofrheumaticchorea(Williams&Swedo,2015).

Box.PharmacologicmanagementofSydenham'schorea

Corticosteroids

ValproicAcid

Haloperidol

Phenobarbital

Pimozide

Carbamazepine

Diazepam

Chlorpromazine

DatafromGilbert(2016).

Bedrest,althoughcontributingtothereductionofrheumaticactivity(Cilliers,Adler,&Saloojee,2015)andonceamainstayof
treatmentseveralyearsago,hasnowelicitedcontroversy.Therefore,restrictionofactivitiesthroughtheacuteillness,especiallyfor
thosemanifestingfindingsofcarditis,isrecommended,withagradualincreaseinactivityastolerated.Commonly,physicalactivity
restrictionoccursuntiltheacutephasereactantsarewithinnormallimits.Hence,itisnecessarytofollowlaboratorytestresult
values.

HowCanRecurrentAttacksBePrevented?

PreventionoffutureepisodesofGASpharyngitisisthemosteffectivemethodforprohibitingprogressionandseverityofRHD,with
whichdisabilityordeathcouldensue(Gibofsky,2016Wyber,Zhlke,&Carapetis,2014)..Providersmustbeawarethatan
asymptomaticrecurrentGASinfectioncantriggerarecurrentattackandthatanasymptomaticGASpharyngitiscangoundetected.
Inaddition,arecurrenceofrheumaticfevercanstilloccureventhoughaGASpharyngitisinfectionwastreatedappropriately
(Gerberetal.,2009Gibofsky,2016).Hence,usingsecondaryprophylaxisrequiresacontinuousantimicrobialregimenratherthan
recognitionandtreatmentoftheacuteepisodicnature.Intheeventthat,duringprophylaxistreatment,ahouseholdorclosecontact
shoulddevelopanacuteepisodeofGASpharyngitis,thepatientandthecontactshouldbeevaluatedandtreatedpromptlyas
outlinedin(Gibofsky,2016).Providersmusttakeintoconsiderationeachpatient'sindividualsituationwhenselectingtheappropriate
prophylaxistreatmentanditsduration.Antibioticoptionsforsecondaryprophylaxisareillustratedin,anddurationofprophylaxisare
describedin.

Table1.TreatmentofpharyngitisduetogroupAStreptococcus

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Note.ReproducedwithpermissionfromGibofskyA.Acuterheumaticfever:Treatmentandprevention.In:UpToDate,PostTW(Ed),
UpToDate,Waltham,MA.(Accessedon[Date].)Copyrightq2016UpToDate,Inc.Formoreinformationvisitwww.uptodate.com.
Datafrom:Gerberetal.(2009),AmericanAcademyofPediatrics(2015),Shulmanetal.(2012),andCohenetal.(2002).

Table2.Secondaryrheumaticfeverprophylaxis

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Note.ReproducedwithpermissionfromGibofsky,A.(2016).Acuterheumaticfever:Clinicalmanifestationsanddiagnosis.InT.Post,
R.Sundel,D.Sexton,&E.TePas(Eds.),UpToDate.Waltham,MA:UpToDate.Retrievedfrom
https://www.uptodate.com/contents/acuterheumaticfeverclinicalmanifestationsanddiagnosis.

Table3.Durationofsecondaryrheumaticfeverprophylaxis

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Note.Reprintedwithpermission.Circulation.2009119:15411551q2009AmericanHeartAssociation,Inc.

Dependingontheirclinicalconditions,followupforpatientswithARFtypicallycanbeperformedduringtheirreturnforprophylaxis
every3to4weeksifreceivingintramuscularantibioticsotherwise,visitscanbescheduledforevery4to6weeks.Acutephase
reactantsneedtobemonitoreduntilnormalvaluesarenotedoncenormal,valuesshouldbemonitoredperiodicallyforanadditional
6to8weeks.

Infectiveendocarditis,alsoknownasbacterialendocarditis,isanuncommoninfectioncausingvegetationsoftheendocardiumwith
typicalvalveinvolvement.Patientswithsomeheartconditionsareatgreaterrisk(Nishimuraetal.,2014).TheAHApublished
updatedrecommendationsregardingtheuseofprophylacticantibioticstopreventinfectiveendocarditis.Becauseofalackof
publishedevidenceindicatingthatprophylaxisdefinitivelypreventsinfectiveendocarditis,thevalueofprophylaxishasbeencalled
intoquestionbytheAHAandotherinternationalscientificbodies.TheAHAnolongerrecommendsprophylaxisforpatientswith
RHD,althoughexceptionsdoexist(Gerberetal.,2009Nishimuraetal.,2014Wilsonetal.,2007).Inthoseparticularpatientsof
exception,currentAHArecommendationsshouldbefollowed.

Patientandparentteachingiscrucialtoobtaincompliancewithsecondaryprevention.Thoroughexplanationsofdiagnosis,and
management,aswellascomplicationsthatcanariseifadherenceiscompromised,areparamounttosuccess.Informingthe
patient'scommunitycenter,childcarefacility,school,and/orteacherisessentialinmaintaininghighqualityandseamlesscare.In
addition,makinginterdisciplinarymembersawareofcomplicationsshouldtheyoccuranduseofacollaborativeapproachto
managementimprovesthepatient'schancesforsuccess.

WhatDoestheFutureHold?

ARFandRHDcontinuetobeanundesirableglobalburden.Occurringatayoungage,ARFresultsinmorbidityandmortalityin
adolescentsandyoungadults.Withthecurrentidentificationofsubclinicalcarditis,versusthatofyearsprior,thetotalburdenofRHD
ishigherthanpreviouslyestimated.AlthoughitisknownthatARFfollowsaGASinfection,theexactpathogenesisremainsa
mystery.Thefocusofprimarypreventionmayneedtoberedirectedtowardanantistreptococcalvaccine,whichwouldeliminatethe
rootcauseofARF.

ConsideringthelargenumberofglobalGASinfectionsandpotentialsequelae,controlstrategiesthatareabletopreventinfection
andcolonization,suchasthedevelopmentofanantistreptococcalvaccine,areextremelyimportant(Guilherme,Ferreira,Khler,
Postol,&Kalil,2013).BecauseGASpharyngitiscanoccurasymptomatically,lackofrecognitionbythepatientortheparentis
unavoidable.Shouldavaccinebeavailable,worldwideeradicationwouldultimatelyleadtoARF'sdemise.SeveralGASvaccine
formulationsprovokingprotectiveimmunityinanimalmodelshaveshownpromise.Presentlyahumanvaccineisnotavailable,
althoughworkonitsdevelopmentisongoing(KumarandTandon,2013Walkeretal.,2014)."TheWorldHeartFederationhasa
goaltoreduceprematuredeathsfromrheumaticfeverandrheumaticheartdiseaseamongindividualsagedlessthan25yearsby
2025"(Wyberetal.,2014,p.768).Planstobesuccessfulincludeglobalaccesstopenicillinandsupportforvaccineresearch
(Casey,2013).WeareoptimisticthatARFwillbemerelyadocumenteddiseasethatwasknowntooccurafterGASinfection,but
untilthen,cognizance,diligence,andeducationremainpriorities.

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