Rheumatic Fever Guideline 1(2)

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Rheumatic Fever

New Zealand

Evidence-based, best practice Guidelines on:

1. Diagnosis, Management and Secondary Prevention

2. Sore Throat Management

3. Proposed Rheumatic Fever

Primary Prevention Programme

1. Diagnosis, Management and Secondary Prevention

NHF0239 80pp Cover 4C.indd 2 21/6/06 12:12:39

Endorsed by:

NHF0239 80pp Cover 4C.indd 3-4NHF0239 80pp Cover 4C.indd 3-4 21/6/06 12:34:5821/6/06 12:34:58

Table of Contents

1. Scope and Purpose of Guideline 5

2. About the Guideline 5

Disclaimer 5

Outlineofgradingmethodologyused 6

Endorsingorganisations 6

Organisationsconsulted 6

NewZealandguidelines:Writinggroup 7

Otherreviewersandcontributors 8

Secreteriatsupport 8

3. Introduction 9

Keypoints 9

Pathogenesis 9

Epidemiology 9

CosttoNewZealand 11

Populationprojections 11

PreventionofARFandRHD 11

DIAGNOSIS AND MANAGEMENT 13

4. Diagnosis of Acute Rheumatic Fever 14

Importanceofaccuratediagnosis 14

Currentapproachestodiagnosis 14

Clinicalfeaturesofacuterheumaticfever-majormanifestations 16

Clinicalfeaturesofacuterheumaticfever-minormanifestations 18

EvidenceofaprecedinggroupAstreptococcalinfection 19

Otherlesscommonclinicalfeatures 19

Echocardiography 19

Differentialdiagnosis 22

Investigations 23

5. Management of ARF 24

Observationandgeneralhospitalcare 28

Discharge 29

SECONDARY PREVENTION 31

6. Prophylaxis Regimes 32

Penicillin 32

Dose 32

Frequency 32

Secondaryprophylaxiswhilebreastfeeding,inpregnancyandwhileonoralcontraceptives 33

Secondaryprophylaxisinanti-coagulatedcases 34

7. Duration of Secondary Prophylaxis 34

8. Protocol for Secondary Prophylaxis Delivery 36

9. Anaphylaxis 37

10.Improving Adherence to Secondary Prophylaxis 37

ReducingthepainofBPGinjections 37

Education 38

ARFRegisters 38

KeydataelementsofARF/RHDregisters 40

Outreachandout-of-town 42

Non-compliance 42

11. Routine Review and Structured Care Planning 43

NHF0239 80pp Inside.indd 2 21/6/06 12:11:52

1Evidence-based, best practice New Zealand Guidelines for Rheumatic Fever

1. DIAGNOSIS, MANAGEMENT AND SECONDARY PREVENTION

He korokoro ora he manawa ora,Mo tatou katoa

( A healthy throat, a healthy heart for us all)

JUNE 2006

NHF0239 80pp Inside.indd 1NHF0239 80pp Inside.indd 1 3/7/06 11:13:263/7/06 11:13:26Process CyanProcess CyanProcess MagentaProcess MagentaProcess YellowProcess YellowProcess BlackProcess BlackPANTONE 186 CVCPANTONE 186 CVC

312. Prevention of Infective Endocarditis 44

13. Case Finding: Surveillance and Screening 44

Surveillance 44

Screeningforrheumaticheartdisease 45

Suggestedindicatorsforevaluation 46

14. Implementation 47

15. Algorithms 50

Algorithm1.GuideforthediagnosisofARF 50

Algorithm2.GuidefortheuseofechocardiographyinARF 52

Algorithm3.Guideforthedurationofsecondaryprophylaxis 53

16. References 54

17. Appendices 63

AppendixA:Guidelinedevelopmentprocess 63

AppendixB:JonescriteriaforthediagnosisofARF 64

AppendixC:UseofechocardiographyinARF 66

AppendixD:MedicationsusedinARF 68

AppendixE:Comparisonofintramuscularpenicillinandoralpenicillinforsecondaryprevention 70

AppendixF:Anaphylaxisrecognitionandmanagement 71

AppendixG:Protocolforfollow-upofnon-compliantcases 72

AppendixH:Walletcardforinfectiveendocarditisprevention 73

18. Glossary 74

19. Notes 75


List of Tables

Table1. Levelsofevidenceforclinicalinterventionsandgradesofrecommendation 6

Table2. NewZealandguidelinesforthediagnosisofARF 15

Table3. MajormanifestationsofARF 16

Table4. MinormanifestationsofARF 18

Table5. UpperlimitsofnormalforserumstreptococcalantibodytitresusedinNewZealandforARFdiagnosis 19

Table6. Minimalechocardiographiccriteriatoallowadiagnosisofpathologicalvalvularregurgitation 20

Table7. SeverityofARFcarditis 21

Table8. DifferentialdiagnosesofcommonmajormanifestationsofARF 22

Table9. InvestigationsinsuspectedARF 23

Table10. Prioritiesinmanagingacuterheumaticfever 24

Table11. Guidelinesforgeneralin-hospitalcare 28

Table12. Recommendedantibioticregimensforsecondarypreventionofacuterheumaticfever/rheumaticheartdisease 33

Table13. NewZealandrecommendationsforthedurationofsecondaryprophylaxis 34

Table14. Suggestedprotocolforthedeliveryofsecondaryprophylaxisbycommunitynurses 36

Table15. MeasuresthatmayreducethepainofbenzathinepenicillinGinjections 37

Table16. PrimaryaimsofARFregistersystems 39

Table17. Recommendedelementsofregister-basedcontrolprogramme 39

Table18. Datasetforacuterheumaticfeverregister 40

Table19. RecommendedroutinereviewandmanagementplanforARFandRHD 43

Table20. RecommendedelementsofascreeningprogrammeinNewZealand 45

Table21. ProposedindicatorsforevaluatingARF/RHDcontrolprogrammes 46

Table22. UsesofechocardiographyinARF 66

Table23. DiagnosticandclinicalutilityofsubclinicalrheumaticvalvedamageinARF 67

Table24. MedicationsusedinARF 68

Table25. Recommendeddoseofadrenalineinanaphylaxis 71


5[1. Scope and Purpose of Guideline]

[2. About the Guideline]

ThisguidelinehasbeendevelopedbyTheNationalHeartFoundationofNewZealandand theCardiacSocietyofAustraliaandNew Zealand. This guideline will be complemented by further guidelines on appropriate sore throat management and primarypreventionofacuterheumaticfever(ARF).

Theobjectivesofthisguidelineare:

toidentifyandpresenttheevidenceforbestpracticeinARFdiagnosis

toidentifythestandardofcarethatshouldbeavailabletoallpeopleinNewZealand

toidentifyareaswherecurrentmanagementstrategiesmaynotbeinlinewithavailableevidence

toensurethathigh-riskpopulationsreceivethesamestandardofcareasthatavailabletoother NewZealanders.

Thisguidelinewasdevelopedbyawritinggroupcomprisedofexpertsinrheumaticfever.SelectedindividualswithexperienceinARFandrelevantstakeholderswerealsoinvolved.Theseincludedarangeofgeneralandspecialistclinicians,alliedhealthprofessionals,Ma-oriandPacificprofessionals,andlayrepresentativegroups.

ThisguidelinehasbeenproducedforNewZealandandisendorsedbyNewZealandorganisations.

ThechairsoftheguidelinewritingcommitteewereinvolvedinthedevelopmentofasimilardocumentfortheAustralianpopulation,withtheunderstandingthattheAustralianguidelineswouldbeadaptedfortheNewZealandsetting.WearegratefulforthecontributionofourAustraliancolleagues.

ThedevelopmentprocessisdescribedinAppendixA.

Disclaimer ThisdocumenthasbeenproducedbyTheNationalHeartFoundationofNewZealandand theCardiacSocietyofAustraliaandNewZealand forhealthprofessionals.Thestatementsandrecommendations itcontainsare,unless labelledasexpertopinion,basedonindependentreviewoftheavailableevidence.Interpretationofthisdocumentbythosewithoutappropriatehealthtrainingisnotrecommended,otherthanattherequestof,orinconsultationwith,arelevanthealthprofessional.

Inaddition,therecommendationsinthisguidelinearenotintendedtoreplaceclinicaljudgmentofeachindividualcase.Treatmentshould take into account comorbidities, drug tolerance, lifestyle, living circumstances, cultural sensibilities and wishes. Whenprescribingmedication, clinicians shouldobserveusual contra-indications,bemindful ofpotential adversedrug interactionsandallergies,monitorresponsesandensureregularreview.


Endorsing organisations TheCardiacSocietyofAustraliaandNewZealand

TheNationalHeartFoundationofNewZealand,alongwith:

TeHotuManawaMa-ori

PacificIslandsHeartbeat

PaediatricSocietyofNewZealand

TheRheumaticFeverTrust.

Organisations consulted AustralasianSocietyforInfectiousDiseases

AustralasianFacultyofPublicHealthMedicine

NationalHeartFoundationofAustralia

NewZealandNursesOrganisation

NewZealandMinistryofHealth

PasifikaMedicalAssociationofNewZealand

RoyalAustralasianCollegeofPhysicians

TeOhuRataoAotearoa-Ma-oriMedicalPractitionersAssociation.

Richbodyofhigh-qualityRCTdata

LimitedbodyofRCTdataorhigh-qualitynon-RCTdata

Noevidenceavailablepanelconsensusjudgment

LEVEL OFEVIDENCE STuDY DESIGN GRADE OF RECOMMENDATION

I A

II B

III-I B

III-2 B

III-3 C

IV C

D/I

Table 1. Levels of Evidence for Clinical Interventions and Grades of Recommendation

Outline of grading methodology usedThereviewincludeslevelsofevidenceandaccompanyinggradesofrecommendation(Table1).

Evidenceobtainedfromasystematicreviewofallrelevantrandomisedcontrolledtrials(RCT)

Evidenceobtainedfromatleastoneproperlydesignedrandomisedcontrolledtrial

Evidenceobtainedfromwell-designedpseudo-randomisedcontrolledtrials(alternateallocation orsomeothermethod)

Evidenceobtainedfromcomparativestudieswith concurrentcontrolsandallocationnotrandomised(cohortstudies),case-controlstudies,orinterruptedtimeserieswithacontrolgroup

Evidenceobtainedfromcomparativestudieswithhistoricalcontrol,2ormoresingle-armstudies,orinterruptedtimeserieswithaparallelcontrolgroup

Evidenceobtainedfromcaseseries,eitherpost-testorpre-testandpost-test

Insufficientevidenceavailableexpertopinionor panelconsensusjudgment

Note: The levels of evidence and grades of recommendations are adapted from the National Heart Foundation of Australia Rheumatic Fever guidelines. (Details can be found at www.nhf.com.au)


7New Zealand guidelines: Writing group

Professor Diana Lennon (Co-chair)ProfessorofPopulationChild&YouthHealth,UniversityofAuckland

Dr Nigel Wilson (Co-chair)PaediatricCardiologist,StarshipChildrensHospital

Dr Polly Atatoa-CarrPublicHealthMedicineRegistrar

Dr Bruce ArrollAssociateProfessorofGeneralPractice,UniversityofAuckland

Ms Elizabeth FarrellPublicHealthNurse,CountiesManukauDistrictHealthBoard

Dr Jonathan JarmanMedicalOfficerofHealth,NorthlandDistrictHealthBoard

Dr Melissa KerdemelidisRheumaticFeverTrustResearchFellow

Mr Henare MasonProjectManager,CountiesManukauDistrictHealthBoard

Dr Johan MorreauPaediatrician,RotoruaHospital

Dr Ross NicholsonPaediatrician,KidzFirstHospital,MiddlemoreHospital

Dr Briar PeatSeniorLecturerinGeneralMedicine,UniversityofAuckland

Ms Heather SpinettoSpecialistCardiacNurse,StarshipChildrensHospital

Dr Lesley VossPaediatricianinInfectiousDiseases,StarshipChildrensHospital.


Other Reviewers and ContributorsDrRohanAmeratunga MsEricaAmon DrJeremyArmishaw MsCatherineAtkinson

DrAnitaBell DrCatherineBremner MsJosephineCottrell ProfessorBartCurrie

DrAlanFarrell DrTomGentles DrDavidGraham MsMichelleHooker

DrDavidJamison ProfessorEdwardKaplan DrAndrewKerr MsTraceyKunac

DrGraemeLear MsLindsayLowe MsMaoiteleLowen MrJohnKristiansen

DrChrisMansell DrFraserMaxwell DrMalcolmMcDonald DrMargotMcLean

MsAndreaMockford DrPhilipMoore DrChrisMoyes MsMarthaNgawaka

MsMaureenOHalloran DrTeuilaPercival DrNeilPoskitt MsKathyRennie

DrJanSinclair DrWarrenSmith MsReneeStreatfield DrRichardTalbot

DrCraigThornley MsLupeToilolo DrWendyWalker MsJoannaWilliams

DrElizabethWilson MsIsabelleTeokotaiWhite

AucklandDistrictNursingGroup.

Secretariat SupportMrsShaelynnSchaumkel.

Australian Guidelines Writing GroupDrAlexBrown;AssociateProfessorJonathanCarapetis(Chair);DrKeithEdwards;DrCliveHadfield;ProfessorDianaLennon;MsLynettePurton;DrAndrewTonkin;DrWarrenWalsh;DrGavinWheatonandDrNigelWilson.

Australian Guidelines reviewers and contributorsDrLeslieEBolitho;DrAndrewBoyden;DrChristianBrizard;DrRichardChard;MsEleanorClune;DrArthurCoverdale;DrSophieCouzos;ProfessorBartCurrie;DrJamesEdward;DrTomGentles;ProfessorMarciaGeorge;DrJefferyHanna;DrNoelHayman;DrAnaHerceg;DrMarcusIlton;DrJenniferJohns;DrJohnKnight;DrJohnMcBride;DrMalcolmMcDonald;DrJohanMorreau;DrMichaelNicholson;DrRossNicholson;MsSaraNoonan;DrBriarPeat;DrPeterPohlner;Dr JimRamsey;Dr JennyReath;MsEmmaRooney;DrWarrenSmith;DrLesleyVoss;DrMarkWenitong;MrChrisWilson;DrElizabethWilsonandDrKeithWollard.

DeclarationNoconflictsofinterestwereinvolvedinthedevelopmentofthisguideline.DrPollyAtatoa-CarrwhocoordinatedthewritingofthisguidelinewasfundedbyTheNationalHeartFoundationofNewZealandandtheAustralasianFacultyofPublicHealthMedicine.


9Key points

Acute rheumatic fever, an auto-immune response to group A streptococcus infection of the upper respiratory tract, may result in damage to the mitral and/or aortic valves and therefore rheumatic heart disease. Recurrences are likely in the absence of preventative measures and may cause further cardiac valve damage

Although acute rheumatic fever is rare in industrialised countries, it is a signi cant cause of disease among Ma-ori and Paci c children in New Zealand. The incidence of rheumatic heart disease is also high among these populations, with signi cant rates of procedures and death among young adults

Appropriate treatment of sore throats in high risk populations will eliminate group A streptococcus in most cases, and prevent individual cases of acute rheumatic fever

Prevention of recurrences, and therefore rheumatic heart disease prevention, with intramuscular penicillin is both effective and highly cost-effective.

Acuterheumaticfever(ARF)isanauto-immuneconsequenceofinfectionwiththebacteriumgroupAstreptococcus(GAS).Itcausesanacutegeneralisedinflammatoryresponseandanillnessthataffectsonlycertainpartsofthebody,mainlytheheart,joints,brainandskin.IndividualswithARFareoftenseverelyunwell,ingreatpainandrequirehospitalisation.Despitethedramaticnatureoftheacuteepisode,ARFleavesnolastingdamagetothebrain,jointsorskin.1

However, thedamage to theheart,ormorespecifically themitraland/oraorticvalves,may remainonce theacuteepisodehasresolved.Thisisknownasrheumaticheartdisease(RHD).PeoplewhohavehadARFpreviouslyaremuchmorelikelythanthewidercommunitytohavesubsequentepisodes.2TheserecurrencesofARFmaycausefurthercardiacvalvedamage.HenceRHDsteadilyworsensinpeoplewhohavemultipleepisodesofARF.

Becauseofitshighprevalenceindevelopingcountries,RHDisthemostcommonformofpaediatricheartdiseaseintheworld.Inmanycountriesitisthemostcommoncauseofcardiacmortalityinchildrenandadultsagedlessthan40years.3

PathogenesisARFhasbeenshown todevelop inapproximatelyone to threepercentof those inanepidemicsituationofuntreatedexudativepharyngitisand/oraculturepositiveforGAS.Despitethehighincidenceinsomeethnicgroups(suchasMa-oriandPacificpeopleinNewZealand),ageneticpredispositiontoARFremainsunproven.1SomestrainsofGAShavebeenrepeatedlyidentifiedascausativeinARF (and therefore labelledrheumatogenic)andother rheumatogenicstrainscontinue toappear. The roleofskin infectionsremainsuncertain.4,5

FollowingGASinfection,thereisalatentperiodaveragingthreeweeksbeforethesymptomsofARFbegin.Bythetimethesymptomsdevelop,theinfectingstrainofGAShasusuallybeeneradicatedbythehostimmuneresponse.

EpidemiologyTheburdenofARFin industrialisedcountriesdeclineddramaticallyduringthe20thCentury,duemainlyto improvements in livingstandards(andhencereducedtransmissionofGAS)andbetteravailabilityofmedicalcare.6,7InmostaffluentpopulationsARFisnowrare.RHDisalsorareinyoungerpeopleinindustrialisedcountries,althoughitisstillseeninsomeelderlypatients,alegacyofARFhalfacenturyearlier.

Bycontrast,ARFandRHDremaincommoninmanydevelopingcountries. ArecentreviewoftheglobalburdenofGAS-relateddiseaseestimatedthatthereisaminimumof15.6millionpeoplewithRHD,another1.9millionwithahistoryofARFbutnocarditiswhostillrequirepreventivetreatment,470,000newcasesofARFeachyearandover230,000deathsduetoRHDannually.8Almostallcasesanddeathsoccurindevelopingcountries.Thesefiguresarealllikelytobeunderestimatesofthetrueburdenofthedisease.

[3. Introduction]Key points

Acute rheumatic fever, an auto-immune response to group A streptococcus infection of the upper respiratory tract, may result in damage to the mitral and/or aortic valves and therefore rheumatic heart disease. Recurrences are likely in the absence of preventative measures and may cause further cardiac valve damage

Although acute rheumatic fever is rare in industrialised countries, it is a signi cant cause of disease among Ma-ori and Paci c children in New Zealand. The incidence of rheumatic heart disease is also high among these populations, with signi cant rates of procedures and death among young adults

Appropriate treatment of sore throats in high risk populations will eliminate group A streptococcus in most cases, and prevent individual cases of acute rheumatic fever

Prevention of recurrences, and therefore rheumatic heart disease prevention, with intramuscular penicillin is both effective and highly cost-effective.


10

ThereissubstantialregionalvariationintheburdenofARFandRHD.ThehighestdocumentedratesintheworldhavebeenfoundinMa-oriandPacificpeopleinNewZealand,AboriginalAustraliansandthoseinPacificIslandnations.9,10,11TheprevalenceofRHDisalsohighinSub-SaharanAfrica,LatinAmerica,theIndiansubcontinent,theMiddleEastandNorthernAfrica.8

NewZealandhashadsustainedhighratesofARFandRHDformanydecadeswithRHDbeingasignificantcauseofprematuredeathinthiscountry.12,13,14AnumberofsurveysofARFandRHDincidencehavebeenconductedsincetheearly1900sinNewZealand.Inthe1920s,surveysofschoolrecordsinNewZealanddeterminedanapproximateannualtotalpopulationincidenceofARFof65per100,000.9From1956to1973,theWairoaCollegeStudydeterminedthatthedeclineinincidenceofARFseeninotherdevelopedcountries was not evident in New Zealand and those pockets of the country which experienced isolation and socio-economicdeprivationhadsignificantlyhigherratesofbothARFandRHD.15

From 1995 to 2000, around 100 cases of ARFwere notified annually inNewZealand,with an incidence of 13.8 per 100,000populationin5to14yearolds.14From1993to1999,theAucklandRegisterrecordedanincidenceof21.9per100,000populationin5to14yearolds.Aucklandaccountsfor60%oftheactivecasesonNewZealandregisters.16,17

ARFispredominantlyadiseaseofchildrenagedbetween5to14years,withapeakataroundeightyears.ItisraretodiagnoseARFundertheageofthree(beforefullmaturationoftheimmunesystem).18,19AsRHDrepresentsthecumulativeheartdamageofpreviousARFepisodes,theprevalenceofRHDpeaksinthethirdandfourthdecadesoflife.20,21Therefore,althoughARFisadiseasewithitsrootsinchildhood,itseffectsarefeltthroughoutadulthood,especiallyintheyoungadultyearswhenpeoplemightotherwisebeattheirmostproductive.

The disparity of ethnicity in rheumatic fever populations has been described in many world centers where population groupsexperiencinglowsocio-economicstatusandlivinginovercrowdedsituationspresentwithahighincidenceofARF.19InNewZealand,Ma-oriandPacificpeopleshavethehighestburdenofbothARFandRHD.Despitethesignificantissuesregardingtheaccuracyofethnicitydatainpastmorbidityandmortalitystatistics,theratesofARFinMa-orihavealwaysbeenreportedassignificantlygreaterthanthoseseeninnon-Ma-ori.Forexample,from1949to1953thereportedincidenceofARFinMa-orichildren(ratesofgreaterthan1000per100,000)was11timesthatofthenon-Ma-oripopulation.9Theage-specificannualnotificationratesforARFbetween1990to1995forchildrenaged10to14yearswas77.7per100,000forPacificchildren,30.4per100,000forMa-orichildrenand1per100,000forEuropeanchildren.14Aucklandalsodisplaysthispattern:theannualincidenceofARFin5to14yearoldMa-orichildrenfrom1993to1999was41.2per100,000population,Pacificchildren83.7per100,000population/yearandtherestofthepopulation1.4per100,000population/year.21Dependingontheyearanalysed,thePacifichospitalisationratesareatleastninetimesthatofEuropeans/others.TheMa-orihospitalisationrateisjustoverfivetimesthatofEuropeans/others.23

Aswell ashigher ratesof initialARF incidence,Ma-ori andPacificpeoplealsohave thehighest ratesofARF recurrence. From1973to1982(priortotheintroductionofsystematicprophylaxisdelivery)recurrenceratesinMa-oriwere40%comparedto22%innon-Ma-ori.24AreviewofcasesintheAucklandrheumaticfeverregisterfrom1993to1999foundthatalthoughthetotalrecurrencerateshaddroppedsignificantlyfromthe1980s(22%to5.5%),alloftherecurrencesfoundwereinMa-oriandPacificpeople.16,17ItisthereforenotsurprisingthatMa-oriandPacificpeoplehavemuchhigherratesofcarditis,RHDandconsequentheartfailure,astheriskofthesecomplicationsincreaseswitheachattackofARF.

IthasnotbeenproventhatMa-oriandPacificpeoplehaveincreasedgeneticsusceptibilitytorheumaticfever.Itismorelikelythattheover-representationofthesesectorsofthepopulationreflectsacombinationofovercrowdedconditions,socio-economicdeprivation,anincreasedincidenceofupperrespiratoryinfectionswithGAS,anddifferenttreatmentoptionsoropportunitiesforappropriateandeffectivehealthcare.11,19,22


11

Cost to New ZealandThere are significant personal, community and national costs associatedwith ARF andRHD. These result from repeated andprolonged hospitalisation, the resources required formedical prophylaxis and treatment, surgical intervention, negative physicaland psychological experience, disruption of the lives of cases and their families and often premature death.25 In 1991, it wasestimatedthatthetotalcostofARFandRHDtotheAucklandhealthservicealonewas$3.6million,withchronicRHDaccountingfor71%ofthecosts.CostsinvolvedwerethedirectcostsofGPandoutpatientvisits,prescriptioncharges,travel,radiologyandthecostsofinformalcaregivenbyhouseholdmembers.20Inadditiontothesedirectcosts,thereareanumberofindirectcostsofARFandRHD,whichareoftendifficulttomeasure.Theseincludenotonlythelossofquantityoflife(ithasbeenestimatedthatfivetotenyoungpeopledieeachyearasadirectresultofARForRHD),butalsothelossofqualityoflife.Thisoccursthroughtimeawayfromeducationandoccupation,impactsonphysicaldevelopmentandfamilyrelationships,psychologicaleffectsandthelossofabilityforchildrenandyoungadultstorealisetheirfullpotential.12,20

Population projectionsCurrentlyMa-oriandPacificpeopleinNewZealandmakeupasizeablepercentageofthechildhoodpopulation.In2001,approximately37%ofMa-oriand40%ofPacificpeopleinNewZealandwereundertheageof15(comparedto23%European).ThemedianageofEuropeanswas36.8years,whilefortheMa-oriandPacificethnicgroupsthecomparablefigureswere21.9and21.0yearsrespectively.26It is reasonable topredict that theNewZealandpopulation in the futurewill representhighgrowthandasustainedyouthfulagestructureintheMa-oriandPacificpopulationswithmany(particularlychildren)livinginpoorsocio-economiccircumstance.26AllthesefeatureshavesignificantimplicationsforARFincidence,prevalenceandprevention.

Prevention of ARF and RHDPrimary prevention

In the future, a cost-effective vaccine for group A streptococcimay be the ideal solution for the primary prevention of ARF.27,28Scientificproblemshavesofarpreventedthedevelopmentofsuchavaccine,28andcurrentlypreventionofaninitialattackofARFrequiresthepromptandaccuratediagnosisandadequateantibiotictreatmentofGASthroatinfections.28,29,30ARFcanbepreventediftheprecedingthroatinfectionistreatedinatimelyandeffectiveway.3,31,32Recommendedtreatmentofstreptococcalthroatinfectionisintramuscular(IM)benzathinepenicillinoraten-daycourseoforalphenoxymethylpenicillin,bothofwhicheradicatethestreptococcifromthepharynx.Theoraltreatmentisoftenusedbecauseitissafe,inexpensiveandlesspainful.

Secondary prevention

Overthelast30yearsoneofthemajorsuccessesinARFmanagementhasbeenthemarkeddeclineinrecurrent(andoftendisabling)attacksofrheumaticfever,duetotheavailabilityofeffectiveantibioticsforsecondaryprophylaxis.32SecondarypreventionofARFisdefinedasthecontinuousadministrationofantibiotics(usuallyparenteralbenzathinepenicillinevery28days)tocaseswithpreviousARForwell-documentedRHD.28TheaimofsecondarypreventionistostoprecolonisationorreinfectionofthethroatwithgroupAstreptococciandtherebypreventingrecurrenceofARF.3TheriskofARFafterthefirstattackofgroupAstreptoccociisapproximately0.3-3%,butwithsubsequentinfectionthisriskrisesto25-75%.2Inaddition,thosewhosuffercarditisduringtheirinitialattackaresignificantlymorelikelytodevelopfurthercarditiswithsubsequentstreptococcalthroatinfections.33ThesystematicuseofregularantibioticprophylaxisinknownARFcaseshasbeenshowntoreducetheincidenceofrecurrentrheumaticfever,reducetheneedforhospitalisationandsurgery,decreasetherapidityandseverityofRHDandimprovequalityoflife.28,34Furthermore,nationalpreventionprogrammesbasedonsecondarypreventionhavethepotentialforconsiderablecostsavings,andhavebeenfoundtobeacost-effectivemethodofreducingmortalityandmorbidityfromARFinternationallyandinNewZealand.20,22,28,35


1


13

Diagnosis andManagement][


1

[4. Diagnosis of Acute Rheumatic Fever (ARF)]Importance of accurate diagnosisItisimportantthatanaccuratediagnosisofARFismadeas:

over-diagnosiswillresultintheindividualreceivingbenzathinepenicillinG(BPG)injectionsunnecessarily everyfourweeksforaminimumoftenyears

under-diagnosisofARFmayleadtotheindividualsufferingafurtherattackofARF,cardiacdamageand prematuredeath.

ThediagnosisofARFreliesonhealthprofessionalsbeingawareofthediagnosticfeatures,particularlywhenpresentationisdelayedoratypical.InAucklandforexample,between1993and1999,fourpatientsdiagnosedwithsepticarthritisbygeneralmedicineandorthopaedicphysicians,subsequentlydevelopedacuterheumaticfever.16,17

Currently,thereisnolaboratorytestdiagnosticforARF,sodiagnosisremainsaclinicaldecision.Thepre-testprobabilityfordiagnosisofARFvariesaccordingtolocationandethnicity.Forexample,inaregionwithhighincidenceofARF(suchastheNorthernhalfoftheNorthIsland),apersonwithfeverandarthritisismorelikelytohaveARFthanoneinalowincidenceregion(suchastheSouthIsland).Ma-oriandPacificpeoplearealsomorelikelythannon-Ma-oriandPacificpeopletohaveARF.

Current approaches to diagnosisTheJonescriteriaforthediagnosisofARFwereintroducedin1944.36ThecriteriadividetheclinicalfeaturesofARFintomajorandminormanifestations,basedontheirprevalenceandspecificity.Majormanifestationsarethosethatmakethediagnosismorelikely,whereasminormanifestationsareconsideredtobesuggestive,butinsufficientontheirown,foradiagnosisofARF.TheexceptiontothisisinthediagnosisofrecurrentARF.

TheJonescriteriahavebeenperiodicallymodifiedandupdated.The1992updateiscurrentlythemostwidelyusedandquotedversion.37

ThereareimportantcircumstanceswhereARFcanbediagnosedwithoutstrictlyadheringtotheJonescriteriaandtheseinclude:

choreaastheonlymanifestationofARF

indolentcarditis(carditisofinsidiousonsetandslowprogression)astheonlymanifestationofARF.37

Boththesetypesofpatientsmayhaveinsufficientsupportinghistorical,clinicalorlaboratoryfindingstofulfiltheJonescriteria.

The1992Jonescriteriaare intendedonly for the initial attackofARF. Furtherdiscussionof theJonescriteriacanbe found inAppendixB.

EachchangetotheJonescriteriawasmadetoimprovespecificityattheexpenseofsensitivity,largelyinresponsetothefallingincidenceofARFinAmerica.Asaresult,thecriteriamaynotbesensitiveenoughtopickupdiseaseinhighincidencepopulations,suchasMa-oriandPacificpeople.Insuchpopulations,theconsequencesofunder-diagnosisarelikelytobegreaterthanthoseofover-diagnosis.

AllcasesofsuspectedARFshouldbejudgedagainstthemostrecentversionoftheJonescriteria,butthecriterianeednotberigidlyadheredtowhenARFisthemostlikelydiagnosis.

AnexpertgroupconvenedbytheWorldHealthOrganisation(WHO)hasrecentlyprovidedadditionalguidelinesastohowtheJonescriteriashouldbeappliedinprimaryandrecurrentepisodes.38

The main modification made to the Jones 1992 criteria for these New Zealand guidelines is the acceptance of echocardiographic evidence of carditis as a major manifestation. In addition there is greater emphasis that monoarthritis may be a presenting feature if there is a history of non-steroidal anti-inflammatory drug (NSAID) use that is likely to have aborted classical ARF migratory polyarthritis.


15

DIAGNOSTIC REQuIREMENTS

InitialepisodeofARF

InitialepisodeofARF

InitialepisodeofARF

RecurrentattackofARF inacasewithknownpastARForRHD

2majoror1majorand2minormanifestationsplusevidenceofaprecedingGASinfection*

StrongclinicalsuspicionofARF,butinsufficientsignsandsymptomstofulfildiagnosisofdefiniteorprobableARF

Minormanifestations(seeTable4forkeypointsinidentifyingminormanifestations)

FeverRaisedESRorCRPPolyarthralgia

ProlongedP-RintervalonECG.

CATEGORY

DefiniteARF

ProbableARF

PossibleARF

Categoriesofdefinite,probableandpossibleARFcanbedeterminedbytheapplicationoftheNewZealandcriteriatoeachcase(Table2).

Table 2. New Zealand Guidelines for the Diagnosis of ARF

All categories assume that other more likely diagnoses have been excluded. Please see additional tables for details about specifi c manifestations.CRP = C-reactive protein; ECG = electrocardiogram; ESR = erythrocyte sedimentation rate; GAS = group A streptococcus; RHD = rheumatic heart disease

*

**

***

#

Elevated or rising antistreptolysin O or other streptococcal antibody (Table 5), is suffi cient for a diagnosis of defi nite ARF. A positive throat culture or rapid antigen test for GAS alone is less secure as 50% of those with a positive throat culture will be carriers only. Therefore, a positive culture alone demotes a case to probable or possible ARF

Most cases of recurrence fulfi l the Jones criteria. However in some cases (such as new carditis on previous RHD) it may not be clear. Therefore in order to avoid under-diagnosis, a presumptive diagnosis of rheumatic recurrence may be made where there are several minor manifestations and evidence of a preceding GAS infection in a person with a reliable history of previous ARF or established RHD. In addition, WHO (2004) recommendations state that where there is established RHD, a recurrent attack can be diagnosed by the presence of two minor manifestations plus evidence of a preceding group A streptococcal infection28

Acceptance of echocardiographic evidence of carditis as a major criterion is a modifi cation to the Jones (1992) update

When carditis is present as a major manifestation (clinical and/or echocardiographic), a prolonged P-R interval cannot be considered an additional minor manifestation in the same person

Other causes of arthritis/arthralgia should be carefully excluded, particularly in the case of monoarthritis e.g. septic arthritis (including disseminated gonococcal infection), infective or reactive arthritis and auto-immune arthropathy (e.g. juvenile chronic arthritis, infl ammatory bowel disease, systemic lupus erythematosus, systemic vasculitis and sarcoidosis. Note that if polyarthritis is present as a major manifestation, polyarthralgia cannot be considered an additional minor manifestation in the same person.

1majorand2minorwiththeinclusionofevidenceofaprecedingGASinfection*asaminormanifestation(Jones,1956)39

2majoror1majorand2minororseveral**minorplusevidenceofaprecedingGASinfection*(Jones,1992)37

Carditis(includingevidenceofsubclinicalrheumaticvalvediseaseonechocardiogram)#

Polyarthritis(orasepticmonoarthritiswithhistoryofNSAIDuse)Chorea(canbestand-aloneforARFdiagnosis)ErythemamarginatumSubcutaneousnodules

Majormanifestationsmodified*** fromJones1992(seeTable3forkeypointsinidentifyingmajormanifestations)

Special consideration should be given to high-risk population groups such as Ma-ori and Pacifi c people, and those residing in poor socio-economic circumstances. In these cases, it may be important to err on the side of diagnosis and prophylaxis.


1

Clinical features of acute rheumatic fever - major manifestationsThemajormanifestationsofARFandfeaturesfortheirdiagnosisarepresentedinTable3.

Table 3. Major Manifestations of ARF

Arthritis* MostcommonpresentingsymptomofARF(occurringinupto75%offirstattacks)

Classifiedasswellingofthejointinthepresenceoftwoormoreofthefollowing: limitationofmovement,hotnessofthejointandpaininthejointand/ortenderness.42Typically,thearthritisof ARFisextremelypainful

Largejointsareusuallyaffected,especiallykneesandankles

Polyarthritisisusuallyasymmetricalandmigratory(onejointbecominginflamedasanothersubsides)butcanbeadditive(multiplejointsprogressivelybecominginflamedwithoutwarning)

HighlyresponsivetosalicylateandNSAIDtherapy-usuallyrespondswithin3days

MonoarthritismaybeapresentingfeatureifthereisahistoryofNSAIDuseearlyinthecourseoftheillness(prematurelyabortingthemanifestationofpolyarthritis).**ThisdiagnosisisbestmadebyaphysicianexperiencedinARF

Thediagnosisofarthritisofthehipisacceptedbyhistoryofpainprecludingweightbearingand/orlimitationofmovementonexamination

Inordertosatisfypolyarthritisasamanifestation,atleastonejointshouldhavebeenobservedinaclinicalsettingaccompaniedbyadefinitehistoryofarthritisinotherjoints(GradeD)

Carditis Valvulitisusuallypresentsclinicallyasanapicalholosystolicmurmurwithorwithoutamid-diastolicflowmurmur(Carey-Coombsmurmur)oranearlydiastolicmurmuratthebaseoftheheart(aorticregurgitation)

Althoughpericarditisandmyocarditismayoccur,cardiacinflammationinARFalmostalwaysaffectsthevalves,especiallythemitralandaorticvalves43,44

Earlydisease leadstovalvularregurgitation,whereasprolongedorrecurrentdiseasemay leadto increasedvalvularregurgitationorstenoticlesions43

Therheumaticaetiologycanusuallybeconfirmedbyatypicalappearanceonechocardiography(seeTables6and7)

InNewZealand,echocardiographicevidenceofsubclinicalcarditiscanalsobeacceptedasamajormanifestation

CongestiveheartfailureinARFresultsfromvalvulardysfunctionsecondarytovalvulitisandisnotduetoprimarymyocarditis45

Thenaturalhistoryofvalveregurgitationisa25-50%improvementbyoneyear46

Ifpericarditisispresent,thefrictionrubmayobscurevalvularmurmurs.

POINTS FOR DIAGNOSISMAJOR MANIFESTATION

Patientswhodonot fulfil thesecriteria,but inwhomtheclinician remainssuspicious that thediagnosismaybeARF,shouldbemaintainedonoralpenicillinand reviewed in two to fourweekswitha repeatechocardiogramtodetect theappearanceofnewlesions.40,41Ifthereisevidenceofrheumaticvalvediseaseclinicallyoronechocardiogram,thediagnosisisconfirmedandlong-termsecondaryprophylaxiscanbecommenced.IfthereisnoevidenceofcarditisandnoalternativediagnosishasbeenfoundthenARFmaybethediagnosisbyexclusion.Thosewithepidemiologicalriskfactors(Ma-ori,Pacificandlowsocio-economicstatus)shouldbecommencedonsecondaryprophylaxiswithdueconsiderationofanalternativediagnosis(suchasrheumatological)andtheneedforongoingreview.


17

Sydenhamschorea Consists of jerky, uncoordinated movements, especially affecting the hands, feet, tongue and face. Themovementsdisappearduringsleep.Theymayaffectonesideonly(hemichorea)

Usefulsignsinclude:47

themilkmaidsgrip(rhythmicsqueezingwhenthepatientgraspstheexaminersfingers) spooning(flexionofthewristsandextensionofthefingerswhenthehandsareextended) thepronatorsign(turningoutwardsofthearmsandpalmswhenheldabovethehead) inabilitytomaintainprotrusionofthetongue

Thismanifestationaffectsfemalespredominantly,particularlyinadolescence48,49

Chorea may occur after a prolonged latent period following GAS infection50,51,52 therefore no additionalmanifestations(includingraisedantibodytitres)arerequiredinordertomakeadiagnosisofARF

Choreahasastrongassociationwithcarditis,***henceechocardiographyisessential forassessmentofallpatientswithchorea,regardlessofthepresenceofcardiacmurmurs(LevelIV,GradeC).Afindingofsubclinicalcarditisbyechowill furthersupport thediagnosisofchoreaasamanifestationofARF (GradeD). Even intheabsenceofechocardiographicevidenceofcarditis,patientswithchoreashouldbeconsideredatriskofsubsequent cardiac damage.53 Therefore, they should all receive secondary prophylaxis, and be carefullyfollowedupforsubsequentdevelopmentofRHD

ChoreaistheARFmanifestationmostlikelytorecurandisoftenassociatedwithpregnancyororalcontraceptiveuse.Thevastmajorityofcasesresolvewithin6months(usuallywithin6weeks)althoughrarecaseslastingaslongas3yearshavebeendocumented47

Subcutaneousnodules

Rare(lessthan2%ofcases)buthighlyspecificmanifestationofARF54

Theyare0.5-2.0cmindiameter,round,firm,freelymobileandpainlessnodulesthatoccurincropsofupto12overtheelbows,wrists,knees,ankles,Achillestendon,occiputandposteriorspinalprocessesofvertebrae

Tendtoappear1-2weeksaftertheonsetofothersymptoms,lastonly1-2weeks(rarelymorethan1month)

Stronglyassociatedwithcarditis

Subcutaneousnodulesare rarely seenas the solemajor criterion inARFandshouldbeaccompaniedbyadditionalmajorcriteriainordertomakethediagnosis

Erythemamarginatum

Rareaswellasdifficulttodetect(especiallyindark-skinnedpeople)

Occursascircularpatternsofbrightpinkmaculesorpapulesthatblanchunderpressureandspreadoutwardsinacircularorserpiginouspatternonthetrunkandproximalextremities(almostneveronface).Therashmaybemoreapparentaftershowering

Notitchyorpainfulandnotaffectedbyanti-inflammatorymedication

Mayrecurforweeksormonths,despiteresolutionoftheotherfeaturesofARF

ErythemamarginatumisrarelyseenasthesolemajorcriterioninARFandshouldbeaccompaniedbyadditionalmajorcriteriainordertomakethediagnosis.

ARF should always be considered in the differential diagnosis of patients presenting with arthritis in high-risk populations. In the hospital setting, physicians and surgeons should collaborate when the diagnosis of arthritis is unclear. Patients with sterile joint aspirates in the absence of previous antibiotic exposure should never be treated speculatively for septic arthritis without further investigation, particularly in areas with high ARF/RHD prevalence

Note that in New Zealand, NSAIDs are now readily available over the counter and have therefore often been used prior to presentation

During recent outbreaks of ARF in the USA, up to 71% of patients with chorea had carditis.55 Even though clinically evident carditis increases the risk of later development of RHD, prior to cardiac echocardiography approximately 25% of patients with pure chorea also eventually developed RHD.53,56 This is explained by the fi nding that over 50% of patients with chorea, but without cardiac murmurs, have echocardiographic evidence of mitral regurgitation.5

*

POINTS FOR DIAGNOSISMAJORMANIFESTATION

***

**


1

Clinical features of acute rheumatic fever - minor manifestations

TheminormanifestationsofARFandfeaturesfortheirdiagnosisarepresentedinTable4.

POINTS FOR DIAGNOSISMINOR MANIFESTATION

Arthralgia MaysuggestARFifthearthralgiaoccursinthesamepatternasrheumaticpolyarthritis(migratory,asymmetricalandaffectinglargejoints)

If polyarthritis ispresentasamajormanifestation,polyarthralgiacannotbeconsideredanadditionalminormanifestationinthesameperson

Alternativediagnoses(assuggested inTable8)shouldbeconsidered inapatientwitharthralgiathat isnottypicalofARF

Fever MostmanifestationsofARFareaccompaniedbyfever(withtheexceptionofchorea)

InNew Zealand, an oral, tympanic or rectal temperature greater than or equal to 38C on admission, ordocumentedduringthecurrentillness,shouldbeconsideredasfever(LevelIV,GradeC)

Fever,likearthritisandarthralgia,isusuallyquicklyresponsivetosalicylate/NSAIDtherapy

Elevatedacutephasereactants

InNewZealand,aserumCRPlevelof30mg/LorESRof50mm/hmeetsthisdiagnosticcriterion(GradeD)

TheESRinARFistypically>80mm/hr,usuallyremainselevatedfor>4weeks,andmayremainelevatedfor3-6monthsdespiteamuchshorterdurationofsymptoms

TheserumCRPconcentrationrisesmorerapidlythantheESRandalsofallsmorerapidlywithresolutionoftheattack

ProlongedP-Rinterval Anelectrocardiogram(ECG)shouldbeperformedinallcasesofsuspectedARF(LevelIV,GradeC)

TheP-Rintervalincreasesnormallywithagethereforeneedstobeage-adjusted.ThefollowingupperlimitsofnormalareusedinNewZealand:*

Age3-12years:0.16seconds Age12-16years:0.18seconds Age17+years:0.20seconds

AprolongedP-Rintervalisoccasionallyanormalvariant,butonethatresolvesovertheensuingdaystoweeksmaybeausefuldiagnosticfeatureofARFincaseswheretheclinicalfeaturesarenotdefinitive.**Inthesecases,arepeatECGafter1-2monthsmaybeuseful

Extremefirstdegreeblocksometimesleadstoajunctionalrhythm,usuallywithaheartratesimilartothesinusrate

Seconddegree,andevencompleteheartblock,canoccurand,ifassociatedwithaslowventricularrate,maygivethefalseimpressionthatcarditisisnotsignificant

Intheabsenceofclinicalorechocardiographiccarditis,asecondorthirddegreeblockaccompaniedbyothermanifestationsofARFishighlysupportiveofthediagnosis(GradeD)

Whencarditisispresentasamajormanifestation(clinicaland/orechocardiographic),prolongedP-Rintervalcannotbeconsideredanadditionalminormanifestationinthesameperson.

Table 4. Minor Manifestations of ARF

*

**

Adapted from Park M K. 58 p42.

In a recent resurgence of ARF in the USA, 32% of patients had abnormal AV conduction, usually a prolonged P-R interval. A small proportion had more severe conduction abnormalities, which were sometimes found by auscultation or echocardiography in the absence of evidence of valvulitis.57


19

Evidence of a preceding group A streptococcal infectionGASareisolatedfromthroatswabsinlessthantenpercentofARFcasesinNewZealand5andlessthanfivepercentofcasesinAboriginalAustralians.54ThislatterfiguremaybearesultoflaterpresentationofARF,as28%ofAboriginalAustralianshavebeenfoundtopresentaschorea59comparedtosixpercentofARFcasesinAuckland(1993-1999).22,23Apositiveculturewithoutsupportiveantibodyelevationmaybecarriageinupto50%ofcases.37Streptococcalantibodytitresarethereforecrucialinconfirmingthediagnosis.ThemostcommonlyusedtestsaretheplasmaantistreptolysinO(ASO)andtheantideoxyribonucleaseB(anti-DNaseB)titres.TheserumASOtitrereachesamaximumataboutthreetosixweeksafterinfectionandtheserumanti-DNaseBtitrecantakeuptosixtoeightweekstoreachamaximum.60Therateofdeclineoftheseantibodiesvariesenormously,withtheASOtitrestartingtofallsixtoeightweeksandtheanti-DNaseBtitrethreemonthsafterinfection.61Intheabsenceofreinfection,theASOtitreusuallyapproachespre-infectionlevelsaftersixto12months,whereastheanti-DNaseBtitretendstoremainelevatedforlonger.62Thereferencerangefortheseantibodytitresvarieswithageandgeographicallocation.Inapopulationwithahighrateofstreptococcalinfections,manychildrenwillhavehighbackgroundstreptococcaltitres.Theupperlimitofnormalapproachattemptstodeterminearaisedtitreoverandabovethisbackground,andthereforeselectoutthosechildrenwhohavehadarecentstreptococcalinfection.63InNewZealand,anASOtitreofgreaterthanorequalto480and/oranantiDNaseBtitreofgreaterthanorequalto680isacceptedassignificant(GradeD)Table5.

Established from residual sera from children (under 15 years) hospitalised in Auckland in 1982. Lower levels may be acceptable in the very young or those over the age of 15 years. A two-tube (two-fold) rise or fall in antibody titres after 10 -14 days would also be diagnostic.Note that evidence of a preceding GAS infection is not necessary for the diagnosis of chorea as ARF.

TITRE (Iu/ML)

480

ANTIBODY TEST

ASO(anti-streptolysinO)

Anti-DNaseB 680

AllcasesofsuspectedARF(choreaisanexception)shouldhaveelevatedserumstreptococcalserologydemonstrated.Iftheinitialtitreisbelowtheupperlimitofnormal,testingshouldberepeated10to14dayslater(GradeD).

Other less common clinical featuresTheseincludeepistaxis,abdominalpain,rheumaticpneumonia(pulmonaryinfiltratesinpatientswithacutecarditis),mildelevationsofplasmatransaminaselevelsandmicroscopichaematuria,pyuriaorproteinuria.NoneisspecificforARFbutepistaxisandabdominalpainoccurcommonly.

EchocardiographyPrior to the introduction of echocardiography, the diagnosis of rheumatic carditis relied on clinical evidence of valvulitis orpericarditis,supportedbyradiographicevidenceofcardiomegaly.Today,allpatientswithsuspectedordefiniteARFshouldundergoechocardiography,ifpossible,toidentifyevidenceofcarditis(GradeC).

InNewZealand,echocardiologyfacilitiesarereadilyavailableinthelargercentersforpopulationsathigh-riskofARF.Theuseofechocardiographyasamajorcriterion forARFdiagnosis requiresexpert interpretationadhering toechocardiographicdiagnosticstandards.ThesestandardsconcurwithrecentWHOechocardiographiccriteriaforARFandaresummarisedinTable6(LevelIV).ThesecriteriacanreadilydistinguishasmallcolourjetofphysiologicalregurgitationinanormalchildfrompathologicalregurgitationinachildwithRHD.

Table 5. Upper Limits of Normal for Serum Streptococcal Antibody Titres Used in New Zealand for ARF Diagnosis


0

Table 6. Minimal Echocardiographic Criteria to Allow a Diagnosis of Pathological Valvular Regurgitation

Echocardiography allows the operator to comment on the appearance of valves that are affected by rheumatic inflammation. The degree of thickening gives some insight into the duration of valvulitis, no significant thickening occurs in the first weeks of acute rheumatic carditis (Level IV)

Only after several months is immobility of the subchordal apparatus and posterior leaflet observed. Several other findings have also been reported, including acute nodules, seen as a beaded appearance of the mitral valve leaflets.64 Although none of these morphological features is unique to ARF, the experienced echocardiographic operator can use their presence as supportive evidence of a rheumatic aetiology of valvulitis.

InNewZealand,ARFcarditisisclassifiedmild,moderateorsevere(Table7)andthesecategoriesareusedtoguidethedurationofsecondaryprophylaxis(seeSection7andTable13).

MITRAL REGuRGITATION

Colour:

Substantialcolourjetseenin2planesextendinggreaterthanorequalto2cmbeyondthevalveleaflets

ContinuouswaveorpulsedDoppler:

Holosystolicwithwell-definedhighvelocityspectralenvelope

Bothmitralandaorticvalveshavepathologicalregurgitation

Themitralregurgitantjetisdirectedposteriorly,asanteriormitralvalveprolapseismorecommonthanposteriorvalveprolapse

Multiplejetsofmitralregurgitation

ThepresenceofmorphologicaloranatomicalchangesconsistentwithRHD(seetext),butexcludingslightthickeningofvalveleaflets:

Definitethickeningofmitralvalveleaflets,indicativeofchronicRHD*

Elbowordoglegdeformity**ofanteriormitralvalveleaflet.

If theaetiologyof aorticormitral regurgitationonDopplerechocardiography isnotclear, the following featuressupportadiagnosisofrheumaticvalvedamage:

AORTIC REGuRGITATION

*

**

Colour:

Substantialcolourjetseenin2planesextendinggreaterthanorequalto1cmbeyondthevalveleaflets

ContinuouswaveorpulsedDoppler:

Holodiastolicwithwell-definedhighvelocityspectralenvelope

Source: Adapted with permission from Wilson N J. & Neutze J M.65 These criteria further evolved as part of the development of the Australian guidelines on rheumatic fever diagnosis and the WHO working groups on echocardiography.66


1

MODERATE CARDITIS

Anyvalvelesionofmoderateseverityclinically(e.g.mildormoderatecardiomegaly),or

Anyechocardiographicevidenceofcardiacchamberenlargementor

Anymoderateseverityvalvelesiononecho**

Mitralregurgitationisconsideredmoderateifthereisabroadhigh-intensityproximaljetfillinghalftheleftatriumora lesservolumehigh-intensityjetproducingprominentbluntingofpulmonaryvenousinflow41

Aorticregurgitationisconsideredmoderateifthediameteroftheregurgitantjetis15%to30%ofthediameterofthe leftventricularoutflowtractwithflowreversalinupperdescendingaorta41

SEVERE CARDITIS

AnyimpendingorpreviouscardiacsurgeryforRHD,or

Anyseverevalvelesionclinically(significantcardiomegalyexpected,and/orheartfailure),or

Anyseverevalvelesiononecho:

AbnormalregurgitantcolourandDopplerflowpatternsinpulmonaryveinsareaprerequisiteforseveremitral regurgitation41

Dopplerreversalinlowerdescendingaortaisrequiredforsevereaorticregurgitation.41

Valvular regurgitation is usually relatively mild in the absence of pre-existing disease; in fi rst episodes of ARF, severe mitral and aortic regurgitation occurred in less than 10% of patients in New Zealand41

When there is both mitral and aortic regurgitation, one must be moderate by echo criteria in order for the carditis to be classifi ed of moderate severity.

Ifvalvulitisisnotfoundatpresentation,itmayappearwithintwoweeks,40oroccasionallywithinonemonth41butnolonger.ThusanequivocalinitialechocardiographshouldbefollowedupintwotofourweeksifthefindingswouldalterthediagnosisofARF.

Usuallyitisnotpossibletoconfidentlydistinguishbetweenacutecarditisandpre-existingrheumaticvalvediseasebyechocardiography.InapatientwithknownpreviousRHD,thediagnosisofacutecarditisduringarecurrenceofARFreliesonaccuratedocumentationofthecardiacfindingsbeforetherecurrence,sothatnewclinicalorechocardiographicfeaturescanbeconfirmed.But,inapatientwithnopriorhistoryofARForRHD,itmakeslittledifferencewhetherechocardiographicchangesareneworold.

FurtherdetailsontheuseofechoinARFcanbefoundinAppendixC.

MILD CARDITIS*

Mildmitraloraorticregurgitationclinicallyand/oronecho(fulfillingtheminimalechostandardsinTable6)withnoclinicalevidence ofheartfailureandnoevidenceofcardiacchamberenlargementonCXR,ECGorechocardiography

Table 7. Severity of ARF Carditis

*

**

Tricuspid and pulmonary regurgitation graded mild or greater may be seen in people with normal hearts who have fever, volume overload or pulmonary hypertension. For this reason a diagnosis of carditis should not be based on right-side regurgitation alone. Although pulmonary and tricuspid regurgitation are often seen in association with left-sided lesions in ARF, pressure and volume overload must be excluded before attributing even moderate tricuspid regurgitation to valvulitis. If both left and right-sided lesions coexist in ARF carditis, then the predominant infl uence for diagnosis is the severity of the left-sided lesion.


Table 8. Differential Diagnoses of Common Major Manifestations of ARF

Source: Adapted from Lennon D. 2004,32 and Carapetis J et al. 2005.67

POLYARTHRITIS AND FEVER

Otherinfections*(includinggonococcal)

Connectivetissueandotherauto-immunedisease**

Reactivearthropathy

Sicklecellanaemia

Infectiveendocarditis

Leukaemiaorlymphoma

Goutandpseudogout

Henoch-Schonleinpurpura

Post-streptococcalreactivearthritis***

Other,e.g.HIV/AIDS,leukaemia

CARDITIS CHOREA

DIF

FER

EN

TIA

L D

IAG

NO

SE

S

Innocentmurmur

Mitralvalveprolapse

Congenitalheartdisease

Infectiveendocarditis

Hypertrophiccardiomyopathy

Myocarditisviraloridiopathic

Pericarditisviraloridiopathic

Systemiclupuserythematosus

Drugingestion(extrapyramidalsyndrome)#

Wilsonsdisease(usuallyadultonset)

Ticdisorder

Congenital,e.g.hyperbilirubinaemia

Choreoathetoidcerebralpalsy

Encephalitis

Familialchorea(includingHuntingtons)

Intracranialtumour

Hormonal

Metabolic,e.g.Lesch-Nyhan, hyperalanaemia,ataxia,telangiectasia

Antiphospholipidantibody

Differential diagnosisManyoftheclinicalfeaturesofARFarenon-specific,soawiderangeofdifferentialdiagnosesshouldbeconsideredasshowninTable8.32,67

Includes bacterial arthritis (e.g. Staphylococcus aureus, Neisseria gonorrhea), influenza b, cytomegalovirus, Epstein-Barr Virus, mycoplasma, rubella (also post-vaccination), hepatitis B, parvovirus, Yersinia spp and other gastrointestinal pathogens

Includes rheumatoid arthritis, juvenile chronic arthritis, inflammatory bowel disease, systemic lupus erythematosus, systemic vasculitis, sarcoidosis and others

Some patients present with arthritis not typical of ARF, but with evidence of recent streptococcal infection and are said to have post-streptococcal reactive arthritis. In these cases the arthritis may affect joints that are not commonly affected in ARF (such as the small joints of the hand), and is less responsive to anti-inflammatory treatment. These patients are said not to be at risk of carditis, and therefore do not require secondary prophylaxis. However, some patients diagnosed with post-streptococcal reactive arthritis have developed later episodes of ARF, indicating that the initial diagnosis should have been atypical ARF (Level IV)68,69

It is recommended that the diagnosis of post-streptococcal reactive arthritis should rarely, if ever, be made in high-risk populations, and with caution in low-risk populations (Grade C). Patients so diagnosed should receive secondary prophylaxis for at least 5 years (Grade D). Echocardiography (see algorithm 2) should be used to confirm the absence of valvular damage in all of these cases before discontinuing secondary prophylaxis (Grade D)

Drugs and toxins include anticonvulsants, antidepressants, lithium, scopolamine, calcium channel blockers, methylphenidate, theophylline and antihistamines

Some cases of chorea are mild or atypical and may be confused with motor tics or the involuntary jerks of Tourettes syndrome. There may therefore be confusion between Sydenhams chorea and these conditions. The term PANDAS (Pediatric Auto-immune Neuropsychiatric Disorder Associated with Streptococcal infection) refers to a subgroup of children with tic or obsessive-compulsive disorders (OCD), whose symptoms may develop or worsen following GAS infection.

Five criteria have been used to define the PANDAS subgroup:70,71

The presence of a Tic disorder and/or OCD Pre-pubertal age of onset (usually between 3 and 12 years of age) Abrupt symptom onset and/or episodic course of symptom severity Temporal association between symptom exacerbations and streptococcal infection (approx 7-14 days) Presence of neurologic abnormalities during periods of symptom exacerbation (typically adventitious movements or motoric hyperactivity)

*

**

***

#


3

Investigations Therecommendedinvestigations inARFare listed inTable9.Other investigationsmaybeappropriatedependingontheclinicalpictureandpotentialdifferentialdiagnoses.

Whitebloodcellcount

Erythrocytesedimentationrate(repeatweeklyoncediagnosisconfirmed)

C-reactiveprotein

Bloodculturesiffebrile

Electrocardiogram(repeatasnecessaryifconductionabnormalitymorethanfirstdegree)

Chestx-rayifclinicalorechocardiographicevidenceofcarditis

Echocardiogram(repeatasnecessaryin2-4weeksifequivocalorifseriouscarditis)

Throatswab(preferablybeforegivingantibiotics)cultureforgroupAstreptococcus

Anti-streptococcalserology:bothanti-streptolysinOandanti-DNaseBtitres,ifavailable(repeat10-14dayslaterif1sttestnot confirmatory)

Serologyandauto-immunemarkersforauto-immuneorreactivearthritis(includingANA-AntiNuclearAntibody)

Repeatedbloodculturesifpossibleendocarditisorsepticarthritis

Jointaspirate(microscopyandculture)forpossiblesepticarthritis*

JointX-ray

Copper,caeruloplasmin,anti-nuclearantibody,drugscreen,andconsiderCT/MRIheadforchoreiformmovements.**

RECOMMENDED FOR ALL CASES

TESTS FOR ALTERNATIVE DIAGNOSES, DEPENDING ON CLINICAL FEATuRES

Typically, the synovial fl uid in joints affected by ARF contains 10,000 to 100,000 white blood cells/mm3 (predominantly neutrophils). The protein concentration is approximately 4g/dL, glucose levels are normal, gram stain negative and a good mucin clot is present72

The chorea of ARF can be readily diagnosed on the basis of history, physical examination and laboratory evaluation. Neuroimaging is seldom necessary and should be reserved for cases who have an atypical presentation such as hemichorea.73

Table 9. Investigations in Suspected ARF

However, the evidence supporting PANDAS as a distinct disease entity has been questioned.71 Hence, in New Zealand populations with a high prevalence of ARF, clinicians should rarely (if ever) make a diagnosis of PANDAS, and should rather err on the side of over-diagnosis of ARF and secondary prophylaxis (Grade D). If ARF is excluded, secondary prophylaxis is not needed, but such cases should be carefully followed up to ensure that they do not develop carditis in the long term

Includes oral contraceptives, pregnancy (chorea gravidarum), hyperthyroidism and hypoparathyroidism.

*

**


Ideally,allthosewithsuspectedARF(firstepisodeorrecurrence)shouldbehospitalisedassoonaspossibleafteronsetofsymptoms(GradeD).Thisensuresthatallinvestigationsareperformedand,ifnecessary,observationscompletedforaperiodpriortocommencingtreatmenttoconfirmthediagnosis.Hospitalisationalsoprovidesanidealopportunityforeducation

Observationpriortoanti-inflammatorytreatment(paracetamol[1stline]forfeverorjointpain)

Investigations(asperTable9)

TREATMENT

All cases

CONFIRMATION OF THE DIAGNOSIS

ADMISSION TO HOSPITAL*

[5. Management of ARF]

Antibiotics**

OralpenicillinV(250mgtwicedaily)shouldbecommencedinallcaseswhilethediagnosisisbeingestablished.Toreliablyeradicate GAS,oralpenicillinshouldbegivenforthefull10days

Oralerythromycinusedincaseswithreliablydocumentedpenicillinallergy***(10daysoferythromycinethylsuccinate(EES)40mg/kg perdayin2-4divideddoses,maximum1g/dayinchildrenor400mgtwicedailyinadolescentsandadults).76EESiscurrentlythe onlyfullysubsidisedoralerythromycininNewZealand

Intravenousantibioticsarenotindicated.Roxithromycinisnotrecommendedbecauseofthelimitedavailableevidencethatitisnot aseffectiveaserythromycinineradicatingGASfromtheupperrespiratorytract77

ThefirstdoseofintramuscularbenzathinepenicillinG(BPG1,200,000Uor600,000Uiflessthan20kg)shouldalsobegiveninhospitalinassociationwitheducationabouttheimportanceofsecondaryprophylaxis.OncethefirstdoseofBPGisgiven,theoralpenicillinisstopped

Arthritis/arthralgia

Salicylates/NSAIDS

ThearthritisofARFhasbeenshownincontrolledtrialstoresponddramaticallytosalicylatesandhasalsobeennotedtorespondto otherNSAIDtherapy,78,79,80oftenwithinhoursandalmostalwayswithin3days (LevelII)

SalicylatesarerecommendedasfirstlinetreatmentbecauseoftheextensiveexperiencewiththeiruseinARF.38,81,82Theyshould becommencedincaseswitharthritisorseverearthralgiaassoonasthediagnosisofARFhasbeenconfirmed(GradeB),butthey shouldbewithheldifthediagnosisisnotcertain.Insuchcases,paracetamolorcodeineshouldbeusedinsteadforpainrelief

Aspirinshouldbestartedatadoseof60-100mg/kg/day(4-8g/dayinadults)in4-5divideddoses.Ifthereisanincompleteresponse within2weeks, thedosemaybe increased to125mg/kg/day,butwith thesehigherdosescareful observation for featuresof salicylatetoxicityisadvised.Iffacilitiesareavailable,bloodlevelsmaybemonitoredeveryfewdays,andthedoseincreaseduntil serumlevelsof20-30mg/100dLarereached.Toxiceffects(tinnitus,headache,hyperpnoea)arelikelyabove20mg/100dLbutoften resolveafterafewdays

Mostcasesrequire10daysorlessofaspirintherapyandthereforebloodlevelmonitoringisseldomnecessary.Manyneedaspirin foronly1-2weeks,althoughsomeneeditforupto6weeks.Insuchcases,thedosecanoftenbereducedto60-70mg/kg/day

ThemajorpriorityinthefirstfewdaysafterpresentationinARFisconfirmationofthediagnosis.Exceptinthecaseofheartfailuremanagement,noneofthetreatmentsofferedtocaseswithARFhavebeenproventoaltertheoutcomeoftheacuteepisodeortheamountofdamagetoheartvalves.74,75Thus,thereisnourgencytobegindefinitivetreatment.TheprioritiesinmanagingARFareoutlinedinTable10.

Table 10. Priorities in Managing Acute Rheumatic Fever


5

TREATMENT CONTINuED

Activity Arthritisalone

Mild carditis Moderate carditis Severe carditis

In hospital 1-2weeks 2-3weeks 4-6weeks 2-4months

Mobilisefreelyastolerated

House arrest (activity and school work at home)

1-2weeksafterdischarge

2-3weeks 4-6weeks 2-4months

School 2weeks 2-4weeks 1-3months 2-3months

Gradualreturntofullactivity Avoidsportandphysicaleducation

Full activity(sport)

After6weeks

After3months After3-6months Variable

Urgentechocardiogram

Anurgentechocardiogramandcardiologyassessmentarerecommendedforallcaseswithheartfailure

aftertheinitial1-2weeks.32Asthedoseisreduced,orwithin3weeksofdiscontinuingaspirin,jointsymptomsmayrecur.Thisdoes notindicaterecurrence,andcanbetreatedwithanotherbriefcourseofhigh-doseaspirin.MostARFepisodessubsidewithin6 weeks,and90%resolvewithin12weeks.Approximately5%ofcasesrequire6monthsormoreofsalicylatetherapy83

ThereisalsotheriskofReyessyndromeinchildrenreceivingsalicylateswhodevelopcertainviralinfections,particularlyinfluenza.Itisrecommendedthatchildrenreceivingaspirinduringtheinfluenzaseason(autumn/winter)alsoreceiveaninfluenzavaccine(GradeD)

Naproxenhasbeenused (10-20mg/kg/day)successfully in thosewithARF, includingonesmall randomised trial,andhasbeen advocatedasasaferalternativetoaspirin(LevelIII-I).84,85Ithastheadvantageofonlytwice-dailydosing,lesshepatotoxicity,anditis alsoavailableinanelixirforyoungchildren.Theexperiencewiththismedicationislimited,sotherecommendationcurrentlyisto restrictittothoseintoleranttoaspirin,ortouseitasastep-downtreatmentoncecasesaredischargedfromhospital(GradeD)

Paracetamol

Mildarthralgiaandfevermayrespondtoparacetamolalone

Fever

Low-gradefeverdoesnotrequirespecifictreatment.Feverwillusuallyresponddramaticallytosalicylatetherapy.Feveralone,orfeverwithmildarthralgiaorarthritis,maynotrequiresalicylates,butcaninsteadbetreatedwithparacetamol

Carditis/heart failure

Bedrest

In thepre-penicillinera,prolongedbedrest in thosewithrheumaticcarditiswasassociatedwithshorterdurationofcarditis, fewerrelapsesandlesscardiomegaly.86Ambulationshouldbegradualandastoleratedincaseswithheartfailure,orsevereacutevalvedisease,especiallyduringthefirst4weeks,oruntiltheserumCRPlevelhasnormalisedandtheESRhasnormalisedordramaticallyreduced.Thosewithmilderornocarditisshouldremaininbedonlyaslongasnecessarytomanageothersymptoms,suchasjointpain(GradeD).

Aguideforactivitylevelsisshownbelow(AdaptedfromLennonD.200432)(GradeD).


Anti-failuremedication

Diuretics/fluidrestrictionformild-moderatefailure

ACEinhibitorsformoreseverefailure,particularlyifaorticregurgitationpresent

Glucocorticoidsoptionalforseverecarditis74

Digoxinifatrialfibrillationpresent

Thereislittleexperiencewithbeta-blockersinheartfailureduetoacutecarditis,andtheiruseisnotrecommended (GradeD)

Detailed recommendations for themanagement of heart failure can be found in a separate Heart Foundation clinical guideline(availableathttp://www.nzgg.org.nz)

Valvesurgery

Surgeryisusuallydeferreduntilactiveinflammationhassubsided.Rarely,valveleafletorchordaetendinaeruptureleadstosevereregurgitationwhichrequiresemergencysurgery.Thiscanbesafelyperformedbyexperiencedsurgeons,althoughtheriskappearstobeslightlyhigherthanwhensurgeryisperformedafteractiveinflammationhasresolved.87Valvereplacement,ratherthanrepair,isusuallyperformedduringtheacuteepisode,becauseofthetechnicaldifficultiesofrepairingfriable,inflamedtissue.Nevertheless,veryexperiencedsurgeonsmayachievegoodresultswithrepairinthissituation

Chorea

Sydenhamschoreaisself-limited.Mostcaseswillresolvewithinweeksandalmostallcaseswithin6months,88althoughrarecasesmaylastaslongas2-3years.59,89,90Mildormoderatechoreadoesnotrequireanyspecifictreatment,asidefromrestandacalmenvironment.Over-stimulationorstresscanexacerbatethesymptoms.Sometimeshospitalisationisusefultoreducethestressthatfamiliesfaceindealingwithabnormalmovementsandemotionallability

Becausechoreaisbenignandself-limiting,andanti-choreamedicationsarepotentiallytoxic,treatmentshouldonlybeconsideredifthemovementsinterferesubstantiallywithnormalactivities,placethepersonatriskofinjuryorareextremelydistressingtothepatient,familyandfriends.Aspirinandglucocorticoidtherapydonothaveasignificanteffectonrheumaticchorea47

Smallstudiesof intravenousimmunoglobulin(IVIG)havesuggestedmorerapidrecoveryfromchorea,buthavenotdemonstratedreduced incidenceof long-termvalvedisease innon-choreaARF.41,91 Untilmoreevidence isavailable, IVIG isnotrecommended,exceptforseverechorearefractorytoothertreatments(LevelII/IV,GradeC)

Carbamazepineandvalproicacid+arenowpreferredtohaloperidol,whichwaspreviouslyconsideredthefirst-linemedicaltreatmentforchorea.92,93Asmall,prospectivecomparisonofthese3agentsrecentlyconcludedthatvalproicacidwasthemosteffective94

Otheranti-choreamedicationsshouldbeavoidedbecauseofpotentialtoxicity.Becauseofthesmallpotentialforlivertoxicitywithvalproicacid,itisrecommendedthatcarbamazepinebeusedinitiallyforseverechorearequiringtreatment,andthatvalproicacidbeconsideredforrefractorycases(LevelIII2,GradeB).Aresponsemaynotbeseenfor1-2weeks,andsuccessfulmedicationmayonlyreduce,butnoteliminate,thesymptoms.Medicationshouldbecontinuedfor2-4weeksafterchoreahassubsidedandthenwithdrawn.Recurrencesofchoreaareusuallymildandcanbemanagedconservativelybut,insevererecurrences,themedicationcanbere-startedifnecessary

CLINICAL FOLLOW-uP

Allcasesshouldreceiveregularreviewandoutpatientfollow-upshouldbeinitiatedpriortodischarge

Thefrequencyanddurationofreviewisdependentontheindividualclinicalneedsandlocalcapacityandshouldbecomemorefrequentintheeventofsymptomonset,symptomaticdeteriorationorachangeinclinicalfindings

Particularcareshouldbetakenwhencasesaretransferredfrompaediatrictoadultservices.Acasecanbemadeformaintaininglessseverecasesinthepaediatricservicesuntildischargeatage21inordertoensurecontinuityoffollow-up

Jointcardiologyandgeneralpaediatric/physicianmanagementforcaseswithseverecarditisarerecommended

FurtherinformationregardingfrequencyandnatureofroutinereviewcanbefoundinSection11


7

COMMENCEMENT OF LONG-TERM PREVENTIVE MEASuRES

*

**

***

#

Secondaryprophylaxis

Obtainconsentfromcaregiver/caseforIMpenicillintreatment

Firstdoseofsecondaryprophylaxisshouldbedeliveredinhospital

Notification

Caseshouldbenotifiedtoa localARFregister ifavailable(seeSection10.3).Thereshouldbeaneasymeanstodothis,viaa standardnotificationform,telephonecallorotherwise

Inaddition,asARFisanotifiablediseaseinNewZealand,eachcaseshouldbenotifiedtothelocalpublichealthunitfornational infectiousdiseasesurveillance

Contactcommunityservicestoensurefollow-up

Theregistercoordinator (ifavailable)shouldnotifycommunityhealthstaffaboutARFcases in theirarea. Thenotifyingmedical practitionershouldalsomakedirectcontactwiththoseinthecommunityresponsibleforprophylaxisdeliveryinordertoensurethat theyareawareofthediagnosis,theneedforsecondaryprophylaxisandanyotherspecificfollow-uprequirements.Thismayinclude districtnurses,publichealthnurses,medicalofficerofhealthandotherpublichealthstaff

Acommunitynurseand/orcommunityhealthworkerfortheareawherethecaseresidesshouldalsodoawardand/orfamilyvisitif possiblebeforedischarge

Whererelevant,itisalsoimportantforconsenttobeobtainedfromthecase(orcaregiver)fortheirlocalMa-oriorPacificproviderto knowabouttheillness

Education

Atthetimeofdiagnosis,itisessentialthatthediseaseprocessbeexplainedtothepatientandtheirfamilyinaculturallyappropriate way,usingavailableeducationalmaterialsandinteractivediscussion.Furthereducation,usingculturallyappropriateeducational materialsshouldfollowoncethecasehasreturnedhome

ForfurtherinformationregardingeducationseeSection10.2

Organisedentalcheckandongoingdentalcare

Thisiscriticalinthepreventionofendocarditis.Asthosewithoutrheumaticvalvedamagemaystillbeatlong-termriskofdeveloping RHD,particularlyintheeventofrecurrentepisodesofARF,dentalcareisessential,regardlessofthepresenceorabsenceofcarditis

Eachcaseshouldbenotifiedtotheappropriateschooldentalserviceordentist

Contactmanagement

Allsymptomaticandasymptomatichouseholdcontactsoftheindexcaseaged3yearsandoldershouldhaveathroatswabifthe contactwasnolongerthanonemonthbeforetheonsetofARFintheindexcase.Thisshouldbeorganisedthroughtheappropriate publichealthunitandallcontactswithpositiveGASculturesshouldbeofferedantibiotictreatment.Streptococcalacquisitionrates of25%orgreaterhavebeenrecordedinfamilycontactsofstreptococcalpharyngitis78,95,96

Opportunisticcare

ItisimportanttonotethisopportunitytoprovideinformationandotherservicesforARFcases,whomfrequentlyhaveotherchallenges to theirgeneralwellbeing. Thismay includepromotingahealthydiet,exerciseandhygiene,aswellasassistancewithsocio- economicstressors,andtheopportunityforongoingsupport.

Occasionally, when the diagnosis has already been confi rmed and the case is not unwell (e.g. mild recurrent chorea in a child with no other symptoms or signs), outpatient management may be appropriate. In such cases health staff must ensure that investigations, treatment, health education, registration (where available) and notifi cation are all completed and prophylaxis commenced

Controlled studies have failed to show that treating ARF with large doses of penicillin affects the outcome of rheumatic valvular lesions 1 year later.97,98 Despite this, most authorities recommend a course of penicillin, even if throat cultures are negative, to ensure eradication of streptococci that may persist in the upper respiratory tract (Grade D)

Most people labelled as being allergic to penicillin are not. Because penicillin is the best antibiotic choice for secondary prophylaxis it is recommended that those with stated penicillin allergy be investigated carefully, preferably with the help of an allergist, before being accepted as truly allergic (Grade D) (Section 6)

If the symptoms and signs do not remit substantially within 3 days of commencing anti-infl ammatory medications, a diagnosis other than ARF should be considered


The use of glucocorticoids and other anti-inflammatory medications in rheumatic carditis has been studied in two meta-analyses.74,75 All of these studies of glucocorticoids were performed more than 40 years ago, and did not use drugs in common use today. These meta-analyses failed to suggest any benefit of glucocorticoids or IVIG over placebo, or of glucocorticoids over salicylates, in reducing the risk of long-term heart disease (Level I). The available evidence suggests that salicylates do not decrease the incidence of residual RHD (Level IV).78,79,80 Therefore, salicylates are not recommended to treat carditis (Grade C). Glucocorticoids may be considered for those with heart failure in whom acute cardiac surgery is not indicated (Grade D). This recommendation is not supported by evidence, but is made because many clinicians believe that glucocorticoids may lead to more rapid resolution of cardiac compromise, and even be life-saving in severe acute carditis.75,99 The potential major adverse effects of short courses of glucocorticoids, including gastrointestinal bleeding and worsening of heart failure as a result of fluid retention, should be considered before they are used. If glucocorticoids are used, the drug of choice is oral prednisone or prednisolone (1-2mg/kg/day, to a maximum of 80mg once daily or in divided doses). Intravenous methyl prednisolone may be given in very severe cases. If a week or less of treatment is required, the medication can be ceased when heart failure is controlled, and inflammatory markers are improving. For longer courses (usually no more than 3 weeks is required), the dose may be decreased by 20-25% each week. Treatment should be given in addition to the other anti-failure treatments outlined below. Mild to moderate carditis does not warrant any specific treatment. As glucocorticoids will control joint pain and fever, salicylates can usually be discontinued, or the dose reduced, during glucocorticoid administration. Salicylates may need to be recommenced after glucocorticoids are discontinued to avoid rebound joint symptoms or fever

Side effects of carbamazepine include CNS adverse reactions (dizziness, headache, ataxia, drowsiness, fatigue and diplopia); gastrointestinal disturbances (nausea and vomiting), as well as allergic skin reactions. Uncommon side effects include abnormal involuntary movements (e.g. tremor, asterixis, dystonia and tics) and nystagmus. Rarely carbemazapine can cause orofacial dyskinesia, oculomotor disturbances, speech disorders (e.g. dysarthria and slurred speech), choreoathetotic disorders, peripheral neuritis, paresthesia, muscle weakness and paretic symptoms100

Side effects of valproic acid include pancreatitis, hepatic toxicity, hyperammonaemia and thrombocytopaenia.100

Observation and general hospital careGuidelinesforgeneralin-hospitalcareareprovidedinTable11(GradeD).

Table 11. Guidelines For General In-Hospital Care

NuRSING RECORDINGS

Temperature,pulse,RR,BP4timesdailySleepingpulse(e.g.0200hrs)Ifpulse>100bpm,recordapicalHR

DIET

Freefluids(ifnoheartfailure)Normaldiet(limitextras)Earlydietaryadviceifoverweightandinfailure,toavoidfurtherweightgainWeeklyweight

BED REST AND GENERAL CARE

Examinedailyforthepatternofarthritisandthepresenceofheartmurmur,choreiformmovements,skinrashandsubcutaneous nodulesIfclinicalcarditispresent: Documentcardiacsymptomsandsigns Dailyweightandfluidbalancechart Medicationsasappropriate(seeTable10andAppendixD) Seegeneralguidelinesforbedrest(Table10) CardiologyopinionRepeatinvestigationsasnecessaryProvideculturalsupport(asrelevant)PlancaretoproviderestperiodsProvideage-appropriateactivitiesNotifyschoolteacherInvolvefamilyincare.

+

Source: Adapted from Lennon D. 2004.32

Note: RR = respiratory rate; BP = blood pressure; HR = heart rate.


9

DischargeTiming of discharge

Thedurationoftreatmentisdictatedbytheclinicalresponseandimprovementininflammatorymarkers(ESRandCRP).MostcasesofARFwithoutseverecarditiscanbedischargedfromhospitalafterapproximatelytwoweeks.Thelengthofadmissionwillpartlydependonthesocialandhomecircumstances.Ifcasescomefromremotecommunitiesorothersettingswithlimitedaccesstohighqualitymedicalcare,itisadvisabletodiscussdischargetimingwiththeperson,familyandthelocalprimaryhealthcareteam(particularlyMa-oriorPacifichealthproviderswherepossible).Insomecases,itmaybeadvisabletoprolongthehospitalstayuntilrecoveryiswelladvanced.

Advice on discharge

Allcasesshouldhaveagoodunderstandingofthecauseofrheumaticfeverandtheneedtohavesorethroatstreatedearlyinotherfamilymembers.Contactmanagement(asperTable10)shouldbediscussed.

CasesandtheirfamiliesshouldunderstandthereasonforsecondaryprophylaxisandtheconsequencesofmissingaBPGinjection.ThefirstdoseofBPGisusuallygiveninahospitalsetting.Arrangementsforthefirstinjectionpostdischargeshouldbemade.Theyshouldbegivenclearinformationaboutwheretogoforsecondaryprophylaxisoncedischarged,knowwhotocontactwithquestionsconcerningtheirfollow-uporsecondaryprophylaxis,andbegivenwritteninformationonappointmentsforfollow-upwiththeirlocalmedicalpractitioner,physician/paediatricianandcardiologist(ifneeded).Theyshouldbeadvisedoftheappropriateactivityleveluntiltheirnextclinicappointment.

Casesandtheirfamiliesshouldalsoberemindedoftheimportanceofadditionalantibioticprophylaxisfordentalandotherprocedurestoprotectagainstendocarditis(AppendixH).

Copiesofthedischargesummaryshouldgotothefollowingservices:communitynursingstaffresponsibleforprophylaxisdelivery(suchasdistrictnurse,publichealthnurse),rheumaticfeversecretaryorstaffresponsiblefortheregister(whereapplicable),primarycareproviderandthefamily.


30


31

[SecondaryPrevention]


3

[6. Prophylaxis Regimes]

SECONDARY PREVENTIONSecondarypreventionofrheumaticfeverisdefinedasthecontinuousadministrationofantibioticstocaseswhohavehadapreviousattack of ARF orwell-documented RHD. The purpose is to prevent infection of the upper respiratory tract withGAS and thedevelopmentofrecurrentrheumaticfever.28

TheregularadministrationofantibioticstopreventinfectionwithgroupAstreptococcal(GAS)andrecurrentARFisrecommendedforallpeoplewithahistoryofARForRHD.3ThisstrategyhasbeenproveninrandomisedcontrolledtrialstopreventstreptococcalpharyngitisandrecurrentARF.

PenicillinInearlystudiesofARFprophylaxisusingsulphonamides,1.5%oftreatedcasesdevelopedARFrecurrences,comparedto20%ofuntreatedcases.Subsequently,penicillinwasfoundtobemoreefficaciousthansulphonamides(LevelI).35,83

ArecentCochranemeta-analysis101concludedthattheuseofpenicillin(comparedtonotherapy) isbeneficial inthepreventionofrecurrentARF,andthatintramuscularbenzathinepenicillinG(BPG)issuperiortooralpenicillininthereductionofbothrecurrentARF(8796%reduction)andstreptococcalpharyngitis(71-91%reduction)(LevelI)(AppendixE).

SecondaryprophylaxisalsoreducestheseverityofRHD.Itisassociatedwithregressionofheartdiseaseinapproximately50-70%ofthosewithadequateadherenceoveradecade(LevelIII2),56,102,103andreducesmortality(LevelIII2).104

DoseTheinternationallyacceptedstandarddoseofBPGforthesecondarypreventionofARFinadultsis1,200,000U.3,38,105Thedoseforchildrenislessclear.InNewZealand,itisrecommendedthat1,200,000UofBPGshouldbeusedforsecondaryprophylaxisforallpersonsweighing20kgormore(LevelIII-2,GradeB),and600,000Uforthoseweighinglessthan20kg(GradeD).106

FrequencyWhile BPG is usually administered every four weeks (28 days), serum penicillin levels may be low or undetectable 28 daysfollowingadoseof1,200,000U.107 Fewerstreptococcal infectionsandARFrecurrencesoccurredamongthosereceiving three-weeklyBPG(LevelI).101,108,109Moreover,thethree-weeklyregimenresultedingreaterresolutionofmitralregurgitationinalong-termrandomised study in Taiwan (66%vs46%) (Level II).110 Prospective data fromNewZealandhowever, showed that recurrenceswere rare among people who were fully adherent to a four-weekly BPG regimen. In Auckland (1993 to 1999), the rate ofrecurrenceinfullyadherentindividualsona28dayregimewas0.07per100patientyears.Failureontheprophylaxisprogramme(i.e. including thosewhowere less than fully adherent) was 1.4 per patient years.16,17 This compares favourably to prophylaxisfailure reported in Taiwan of 0.25 (21-day programme) and 1.29 (28-day programme) per 100 patient years.111 Furthermore, afour-weekly regime is preferable to a three-weekly regime because of the resource and compliance implications (Grade D).InNewZealand,threeweekly(21-day)BPGisrecommendedonlyforthosewhohaveconfirmedrecurrentARFdespitefulladherencetofour-weekly(28-day)BPGdelivery(GradeC).16,17

AnalternativestrategyistheadministrationoflargerdosesofBPG,leadingtoahigherproportionofpeoplewithdetectableserumpenicillinlevelsfourweeksafterinjection.112However,untilmoredataareavailable,thisstrategycannotberecommended.


33

Table 12. Recommended Antibiotic Regimens for Secondary Prevention of Acute Rheumatic Fever/Rheumatic Heart Disease

ANTIBIOTIC

First line

DOSE ROuTE FREQuENCY

BenzathinepenicillinG(BPG)

1,200,000U20kg600,000U20kg450mg(600,000U)20kg

REGIMEN

Singledose

DuRATION

PenicillinVPO

or

250mgbd 10days

ErythromycinethylsuccinatePO

40mg/kgperdayin2-4divideddosesmaximum1g/day(children)

400mgbd(adolescentsandadults)

10days

ParacetamolPO Arthritisorarthralgia-mildoruntildiagnosisconfirmed

60mg/kg/day(max4g)givenin4-6doses/day.Mayincreaseto90mg/kg/dayifneeded,undermedicalsupervision

UntilsymptomsrelievedorNSAIDstarted

CodeinePO Arthritisorarthralgiauntildiagnosisconfirmed

0.5-1.0mg/kg/dose(adults15-60mg/dose)4-6h

AspirinPO Arthritisorseverearthralgia(whenARFdiagnosisconfirmed)

80-100mg/kg/day(4-8g/dinadults)givenin4-5doses/day

Reduceto60-70mg/kg/daywhensymptomsimprove

Considerceasinginthepresenceofacuteviralillness,andconsiderinfluenzavaccineifadministeredduringautumn/winter

Untiljointsymptomsrelieved

NaproxenPO Arthritis(ifaspirin-intolerant)

10-20mg/kg/day (max1250mg)givenbd

Asforaspirin

PrednisoneorPrednisolonePO

Severecarditis,heartfailure,pericarditiswitheffusion

1-2mg/kg/day(max80mg).Ifused>1week,taperby20-25%perweek

Usually1to3weeks

FrusemidePO/IV(canalsobegivenIM)

Heartfailure Children:1-2mg/kgstat,then0.5-1mg/kg/dose6-24hrly(max6mg/kg/dose)

Adults:20-40mg/dose12-24hrlyupto250-500mg/day

Untilfailurecontrolledandcarditisimproved


9

SpironolactonePO

MEDICATION

Heartfailure

INDICATION

1-3mg/kg/day(max100-200mg/day)in1-3doses.Rounddosetomultipleof6.25mg(quarterofatab)

REGIMEN

Asforfrusemide

DuRATION

EnalaprilPO Children:0.1mg/kg/dayin1-2dosesincreasedgraduallyover2wkstomaxof1mg/kg/dayin1-2doses

AdultsInitial:2.5mgdailyMaintenance:10-20mgdaily(max40mg)

Asforfrusemide

LisinoprilPO Heartfailure Children:0.1-0.2mg/kgoncedailyupto1mg/kg/dose

Adults:2.5-20mgoncedaily(max40mg/day)

Asforfrusemide

DigoxinPO/IV Heartfailure/atrialfibrillation

Children:15mcg/kgstatandthen5mcg/kgafter6hrs,then3-5mcg/kg/dose(max125mcg)12-hourly.

Adults:125-250mcgdaily

Checkserumlevels

CarbamazepinePO Severechorea 7-20mg/kg/day(7-10mg/kg/dayusuallysufficient)giventds.

Untilchoreacontrolledforseveralweeks,thentrialoffmedication

Heartfailure

Seekadvicefromspecialist

ValproicacidPO Severechorea(mayaffectsalicylatemetabolism)

Usually15-20mg/kg/day(canincreaseto30mg/kg/day)giventds

Asforcarbamazepine.


70

Appendix E: Comparison of intramuscular penicillin and oral penicillin for secondary prevention

AsearchwasconductedbyManyembaandMayosi(2002).101ThesearchstrategyincludedtheControlledTrialsRegister(CochraneLibrary Issue2,2001),Medline (January1996toJuly2000),Embase (January1985toJuly2000), reference listsofarticlesandconsulatationwithexperts.

Randomisedandquasi-randomisedstudiescomparing:(i)oralwithintramuscularpenicillin;and(ii)two-weeklyorthree-weeklywithfour-weeklyintramuscularpenicillininpatientswithpreviousARF.Tworeviewersindependentlyassessedthetrialqualityandextractedthedataofsixincludedstudies(1,707patients).

Fourtrials(1,098patients)comparedIMwithoralpenicillinandallshowedthatIMpenicillinwasmoreeffectivethanoralinreducingrecurrenceofARFandstreptococcalthroatinfections.

Onetrialcomparedtwo-weeklywithfour-weeklyIMpenicillin.PenicillingiveneverytwoweekswasbetteratreducingARFrecurrence(relativerisk(RR)0.52,95%confidenceinterval(CI)0.33-0.83)andstreptococcalthroatinfections(RR0.60,95%CI0.42-0.85).

Onetrial(249patients)showedthatthree-weeklyIMpenicillininjectionsweremoreeffectivethanfour-weeklyIMpenicillinatreducingstreptococcalthroatinfections(RR0.67,95%CI0.48-0.92).

TheconclusionsmadethereforewerethatIMpenicillinseemedtobemoreeffectivethanoralpenicillininpreventingARFrecurrenceandstreptococcalthroatinfections.Two-weeklyorthree-weeklyinjectionsappearedtobemoreeffectivethanfour-weeklyinjections.However,theevidencewasbasedonpoor-qualitytrialsandtheuseofoutdatedformulationsoforalpenicillin.101


71

Appendix F: Anaphylaxis recognition and management

Thesignsandsymptomsofananaphylacticreactioninclude:rapidweakpulse,wheeze,tightnessinchest,pruritis,urticaria,giddinessorheadache,flushingand/orperiorbitaloedema.

Response procedure: donotleavethepatientalone

callforassistance

liepatientinrecoveryposition(maybebettersittingupifsevererespiratorydistress)

ensureairwayisclear,applyoxygenifavailable

giveadrenaline(Table25)

ring111forambulance

checkvitalsigns,notecolour,toneandperfusion

ifsignsoffurtherdeterioration,repeatadrenalineafter10minutes

upto3dosesofadrenalinecanbegiven.

Adrenaline dosage:Table 25. Recommended Dose of Adrenaline in Anaphylaxis*

0.5mlof1:1000adrenaline,deepIMinjection

12 YEARS OF AGE AND OVER

Approximately0.01ml/kgof1:1000adrenaline,deepIMinjection

Age0-3years:0.1ml

Age4-6years:0.2ml

Age6-8years:0.3ml

Age9-12years:0.4ml

uNDER 12 YEARS OF AGE

Source: StarshipHospitalClinicalPracticeManual,AucklandDistrictHealthBoard(2006).

* Up to 3 doses of adrenaline can be given


7

Appendix G: Protocol for follow-up of non-compliant cases

Case is non-compliant with injections on 3-4 concurrent occasions. All attempts at contact are clearly documented in the patients fi le. These attempts should include the use of multiple modalities for contact including telephone

calls, visits, texting and the use of the local knowledge of community health workers

Discuss with primary nurse and refer to community health worker, public health nurse, or other community staff as fi tting in the area for follow up. Note also opportunity to involve staff from Ma-ori or Pacifi c

primary health providers, if appropriate

Community health worker (or other community staff responsible) follows up with case (and family) to determine reason for non-

compliance. Where necessary and appropriate, provides on going support, education, and arranges appointments for review at

outpatient clinic

If compliance is no longer a problem, continue routine

secondary prophylaxis

At the end of the holding period, the primary nurse and community health

worker review the case and ifconsidered appropriate a discharge

letter is to be sent to the case, with a copy to the patient fi le, GP, and rheumatic

fever register (if available)

If non-compliance continues, letter of planning to discharge is copied to the

case, case fi le, and GP after discussion with primary nurse and community

health worker

Case fi le goes on holdfor up to six months

(local area policy may suggest regular attempts at contact while

case is on hold)


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Appendix H: Wallet card for infective endocarditis prevention

Information For Patient/Parents/Guardians

has a heart disorder and therefore needs antibiotic protection to be given before some of the procedures that dentists and doctors may need to do.

YOU MUST SHOW THIS CARD TO ANY DENTIST/DENTAL THERAPIST OR DOCTOR BEFORE TREATMENT IS STARTED.

General Advice

1 Regular teeth cleaning and avoiding sugary foods and drinks will reduce the need for dental surgery.2 Regular dental check ups will help keep teeth healthy.

HOSPITAL CHECK UPS DO NOT REPLACE VISITS TO YOUR LOCAL DENTIST/ DENTALTHERAPIST.

3

Rheumatic Fever Guideline 1(2)

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