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PediatricRubella
Author:EliasEzike,MDChiefEditor:RussellWSteele,MDmore...
Updated:Dec18,2014

Background
ThenamerubellaisderivedfromaLatintermmeaning"littlered."Rubellaisgenerallyabenigncommunicable
exanthematousdisease.Itiscausedbyrubellavirus,whichisamemberoftheRubivirusgenusofthefamily
Togaviridae.Nearlyonehalfofindividualsinfectedwiththisvirusareasymptomatic.Clinicalmanifestationsand
severityofillnessvarywithage.Forinstance,infectioninyoungerchildrenischaracterizedbymildconstitutional
symptoms,rash,andsuboccipitaladenopathyconversely,inolderchildren,adolescents,andadults,rubellamaybe
complicatedbyarthralgia,arthritis,andthrombocytopenicpurpura.Rarecasesofrubellaencephalitishavealso
beendescribedinchildren.
Themajorcomplicationofrubellaisitsteratogeniceffectswhenpregnantwomencontractthedisease,especiallyin
theearlyweeksofgestation.Theviruscanbetransmittedtothefetusthroughtheplacentaandiscapableof
causingseriouscongenitaldefects,abortions,andstillbirths.Fortunately,becauseofthesuccessfulimmunization
programinitiatedintheUnitedStatesin1969,rubellainfectionandcongenitalrubellasyndromerarelyareseen
today.Seetheimagesbelow.

Numberofrubellacasesperyear.

Numberofcongenitalrubellasyndromecasesperyear.

Deathsfromrubellaperyear.

Thefewcasesofrubellarecordedinrecentyearsinvolvesusceptibleindividualswhohavenotbeenimmunizedwith
rubellavaccineanddonothaveahistoryofpreviousrubellainfection.
AnindependentpanelconvenedbytheCentersforDiseaseControlandPrevention(CDC)in2004foundthatabout
91%oftheUSpopulationisimmunetorubella.Thisexplainsthedecreasednumberofoutbreaksofrubellaand
congenitalrubellasyndromereportedintherecentyears.

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Pathophysiology
Postnatalrubella
Theusualportalofentryofrubellavirusistherespiratoryepitheliumofthenasopharynx.Thevirusistransmittedvia
theaerosolizedparticlesfromtherespiratorytractsecretionsofinfectedindividuals.Thevirusattachestoand
invadestherespiratoryepithelium.Itthenspreadshematogenously(primaryviremia)toregionalanddistant
lymphaticsandreplicatesinthereticuloendothelialsystem.Thisisfollowedbyasecondaryviremiathatoccurs620
daysafterinfection.Duringthisviremicphase,rubellaviruscanberecoveredfromdifferentbodysitesincluding
lymphnodes,urine,cerebrospinalfluid(CSF),conjunctivalsac,breastmilk,synovialfluid,andlungs.Viremiapeaks
justbeforetheonsetofrashanddisappearsshortlythereafter.Aninfectedpersonbeginstoshedthevirusfromthe
nasopharynx38daysafterexposurefor614daysafteronsetoftherash.

Congenitalrubellasyndrome
Fetalinfectionoccurstransplacentallyduringthematernalviremicphase,butthemechanismsbywhichrubellavirus
causesfetaldamagearepoorlyunderstood.Thefetaldefectsobservedincongenitalrubellasyndromearelikely
secondarytovasculitisresultingintissuenecrosiswithoutinflammation.Anotherpossiblemechanismisdirectviral
damageofinfectedcells.Studieshavedemonstratedthatcellsinfectedwithrubellaintheearlyfetalperiodhave
reducedmitoticactivity.Thismaybetheresultofchromosomalbreakageorduetoproductionofaproteinthat
inhibitsmitosis.Regardlessofthemechanism,anyinjuryaffectingthefetusinthefirsttrimester(duringthephase
oforganogenesis)resultsincongenitalorgandefects.

Epidemiology
Frequency
UnitedStates
Duringthe19621965worldwideepidemic,anestimated12.5millionrubellacasesoccurredintheUnitedStates,
resultingin20,000casesofcongenitalrubellasyndrome.Sincethelicensingoftheliveattenuatedrubellavaccine
intheUnitedStatesin1969,asubstantialincreasehasbeennotedinthevaccinationcoverageamongschoolaged
childrenandthepopulationimmunity.In2004,theestimatedvaccinationcoverageamongschoolagedchildrenwas
about95%,andthepopulationimmunitywasabout91%.
Asaresultoftheprogressmadeinvaccinationagainstrubella,aremarkabledrophasoccurredinthenumberof
casesofrubellaandcongenitalrubellasyndrome.Forinstance,in1969,atotalof57,686casesofrubellaand31
casesofcongenitalrubellasyndromewererecorded.Subsequently,from19932000,thenumberofcasesofrubella
recordedannuallydecreasedtoarangeof128364,andcasesofcongenitalrubellasyndromealsodroppedto49
casesperyear.Since2001,theannualnumberofrubellacasesrangedfromarecordlowof7in2003to23in
2001,andcongenitalrubellasyndromecasesbetween03peryear(seetheimagesbelow).

Numberofrubellacasesperyear.

Numberofcongenitalrubellasyndromecasesperyear.

AnindependentpanelconvenedbytheCDCin2004toassessprogresstowardseliminationofrubellaand
congenitalrubellasyndromeintheUnitedStatesconcludedunanimouslythatrubellaisnolongerendemicinthe
UnitedStates.Infact,thepatternofvirusgenotypesisolatedinrecentyearswasconsistentwithvirusoriginatingin
otherpartsoftheworld.
FollowingthenearrecordlowlevelsinrubellaincidenceintheUnitedStates,theoccurrenceofisolatedoutbreaks
amongsusceptibleadultshasalsobecomerare.Infactnooutbreakofrubellawasreportedfrom20002005,in
contrasttotheprecedingyearinterval,19961999,when16outbreakswerereported.Themediannumberofcases
peroutbreakwas21.ThemostrecentcasesoccurredinNewYorkduring19971998,Kansasin1998,Nebraskain
1999,andArkansasin1999.Mostoftheseoutbreakswerereportedincollegecampuses,militaryinstallations,

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prisons,andworkplaces,includinghealthcareenvironments.Inmostinstances,theindividualsinvolvedinthese
outbreakshavenohistoryofrubellaimmunization.Inaddition,mostoftheoutbreakshavebeenreportedamong
personswhoemigratedfromcountrieswhererubellaisnotincludedintheroutineimmunizationschedule.
From2000to2012,risingnumbersofWHOmemberstatesbeganusingrubellacontainingvaccines(RCVs)intheir
immunizationprogramandbeganreportingrubellaandcongenitalrubellasyndrome(CRS)surveillancedata.Asof
December2012,132(68%)WHOmemberstateshadintroducedRCV,a33%increasefrom99memberstatesin
2000.Some43%ofinfantshadreceivedaRCVdosein2012,a96%increasefromthe22%ofinfantswhohad
beenvaccinatedagainstrubellain2000.Atotalof94,030rubellacaseswerereportedtoWHOin2012from174
memberstates,an86%decreasefromthe670,894casesreportedin2000from102memberstates. [1,2]
International
Rubellaoccursworldwide. [3]Thenumberofreportedcasesishighincountrieswhereroutinerubellaimmunizationis
eithernotavailableorwasrecentlyintroduced.Forinstance,inMexicoin1990,atotalof65,591casesofrubella
werereported.Aftertheintroductionofrubellavaccineintothechildhoodimmunizationschedulein1998,the
numberofreportedcasesdeclined68%to21,173.InEurope,theincidenceofrubellaremainshigh.Forinstance,in
2003,atotalof304,320caseswerereported41%ofthesewerefromtheRussianFederation,and40%werefrom
Romania.
Althoughtheburdenofcongenitalrubellasyndromeisnotwellcharacterizedinallcountries,morethan100,000
casesareestimatedtooccureachyearindevelopingcountriesalone.InEurope,atotalof47casesofcongenital
rubellasyndromewerereportedfrom2001200332%werefromtheRussianFederation,and36%werefrom
Romania.

Mortality/Morbidity
Themorbidityandmortalityratesofrubelladiseasedroppedremarkablysincethelicensingofliveattenuated
rubellavaccinein1969.Infact,in1969,complicatedrubellainfectioncaused29fatalitiesintheUnitedStates,
whereasfrom19922001,only02deathsperyearwererecorded(seetheimagebelow).

Deathsfromrubellaperyear.

Incontrasttopostnatalrubella,whichisnotadebilitatingdisease,congenitalrubellainfectionmayresultingrowth
delay,learningdisability,mentalretardation,hearingloss,congenitalheartdisease,andeye,endocrinologic,and
neurologicabnormalities.
Table1.ReportedCasesofRubella,DeathsFromRubella,andNumberofCasesofCongenitalRubellaSyndrome
intheUnitedStatesFrom19692007[4,5,6,7](OpenTableinanewwindow)
Year NumberofCases NumberofDeaths CasesofCongenitalRubellaSyndrome
1969 57,686

29

31

1970 56,552

31

77

1971 45,086

20

68

1972 25,507

14

42

1973 27,804

16

35

1974 11,917

15

45

1975 16,652

21

30

1976 12,491

12

30

1977 20,395

17

23

1978 18,269

10

30

1979 11,795

62

1980 3,904

50

1981 2,077

19

1982 2,325

1983 970

22

1984 752

1985 630

1986 551

1987 306

1988 225

1989 396

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1990 1,125

11

1991 1,401

47

1992 160

11

1993 192

1994 227

1995 128

1996 238

1997 181

1998 364

1999 267

2000 176

2001 23

2002 18

N/A

2003 7

N/A

2004 10

N/A

2005 11

N/A

2006 11

N/A

2007 12

N/A

Race
Noethnicdifferenceinincidencehasbeenclearlydemonstrated,althoughthecharacteristicrashismoredifficultto
diagnoseinpersonswithdarkskin.

Sex
Noappreciabledifferencesininfectionratesbysexareapparentinchildren,butinadults,morecasesarereported
inwomenthaninmen.Rubellaarthralgiaandarthritisaremorefrequentinwomenthaninmen.

Age
Beforelicensingoftheliveattenuatedvaccinein1969,rubellaintheUnitedStateswasprimarilyadiseaseof
schoolagedchildren,withapeakincidenceinchildrenaged59years.Followingwidespreaduseofrubellavaccine
inchildren,peakincidencehasshiftedtopersonsolderthan20years,whocomprise62%ofcasesofrubella
reportedintheUnitedStates.
ClinicalPresentation

ContributorInformationandDisclosures
Author
EliasEzike,MDConsultingStaff,BeaumontPediatricCenter,PLLC
EliasEzike,MDisamemberofthefollowingmedicalsocieties:AmericanAcademyofPediatrics
Disclosure:Nothingtodisclose.
Coauthor(s)
JocelynYAng,MD,FAAP,FIDSAAssociateProfessor,DepartmentofPediatrics,WayneStateUniversity
SchoolofMedicineConsultingStaff,DivisionofInfectiousDiseases,Children'sHospitalofMichigan
JocelynYAng,MD,FAAP,FIDSAisamemberofthefollowingmedicalsocieties:AmericanAcademyof
Pediatrics,InfectiousDiseasesSocietyofAmerica,PediatricInfectiousDiseasesSociety
Disclosure:Nothingtodisclose.
SpecialtyEditorBoard
MaryLWindle,PharmDAdjunctAssociateProfessor,UniversityofNebraskaMedicalCenterCollegeof
PharmacyEditorinChief,MedscapeDrugReference
Disclosure:Nothingtodisclose.
ChiefEditor
RussellWSteele,MDClinicalProfessor,TulaneUniversitySchoolofMedicineStaffPhysician,OchsnerClinic
Foundation
RussellWSteele,MDisamemberofthefollowingmedicalsocieties:AmericanAcademyofPediatrics,
AmericanAssociationofImmunologists,AmericanPediatricSociety,AmericanSocietyforMicrobiology,
InfectiousDiseasesSocietyofAmerica,LouisianaStateMedicalSociety,PediatricInfectiousDiseasesSociety,
SocietyforPediatricResearch,SouthernMedicalAssociation
Disclosure:Nothingtodisclose.
AdditionalContributors
LeonardRKrilov,MDChiefofPediatricInfectiousDiseasesandInternationalAdoption,ViceChair,
DepartmentofPediatrics,WinthropUniversityHospitalProfessorofPediatrics,StonyBrookUniversitySchoolof
Medicine
LeonardRKrilov,MDisamemberofthefollowingmedicalsocieties:AmericanAcademyofPediatrics,
AmericanPediatricSociety,InfectiousDiseasesSocietyofAmerica,PediatricInfectiousDiseasesSociety,

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SocietyforPediatricResearch
Disclosure:Nothingtodisclose.
Acknowledgements
LeslieLBarton,MDProfessorEmeritaofPediatrics,UniversityofArizonaCollegeofMedicine
LeslieLBarton,MDisamemberofthefollowingmedicalsocieties:AmericanAcademyofPediatrics,
AssociationofPediatricProgramDirectors,InfectiousDiseasesSocietyofAmerica,andPediatricInfectious
DiseasesSociety
Disclosure:Nothingtodisclose.

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