Juvenile Idiopathic Arthritis Uveitis

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JuvenileIdiopathicArthritisUveitisAuthor:ManoletteRRoque,MD,MBAChiefEditor:HamptonRoySr,MDmore...

Updated:Jun19,2012

Background

Approximately6%ofallcasesofuveitisariseinchildren.[1]Themostfrequentcauseofchronicintraocularinflammationamongchildrenisjuvenileidiopathicarthritis(JIA)associateduveitis.[2]Aunifyingclassification,juvenileidiopathicarthritis(JIA),encompassingjuvenilechronicarthritisandjuvenilerheumatoidarthritis(JRA),hasbeendevelopedbyconsensus.

Acuteanterioruveitiswithhypopyoninachild.CourtesyofManoletteRoque,MD,OphthalmicConsultantsPhilippinesCo,EYEREPUBLICOphthalmologyClinic.

Chroniciridocyclitisoccursin1020%ofallpatientswithJIA.[3,4,5,6]ChronicuveitischaracteristicallyisasymptomaticinchildrenwithJIA,leadingtoinsidiousbutprogressivemorbidityandpossibleblindness.Theinvolvedeyesoftenarewhiteandquietappearing,yet3040%ofpatientswithJIAassociateduveitisexperienceseverelossofvisionasaconsequenceoftheircondition.

JIA,asdefinedbytheAmericanRheumatismAssociation(ARA),isthepresenceofarthritis(chronic,seronegative,andperipheral)beforeage16years,ofatleast3monthsduration,whenothercauseshavebeenexcluded.Itisclassifiedby1of3typesofonset.[7]

Oligoarticular(pauciarticular)onsetJIA(4060%)iscommoningirls(5:1).Peakageofonsetisatage2years.Fourorfewerjointsareinvolvedduringthefirst6monthsofthedisease(oftenasymmetric).Oligoarticularonsetcommonlyinvolvesthekneesand,lessfrequently,theanklesandwrists.Thearthritismaybeevanescent,rarelydestructive,andradiologicallyinsignificant.Approximately75%ofthesepatientstestpositiveforantinuclearantibody(ANA).Thismodeofonsetrarelyisassociatedwithsystemicsigns.Ahighriskforuveitisexists.[8]

PolyarticularonsetJIA(2040%)iscommoningirls(3:1).Peakageofonsetisatage3years.Itinvolves5ormorejointsduringthefirst6monthsofthedisease.PolyarticularonsetJIAcommonlyinvolvesthesmalljointsofthehandand,lessfrequently,thelargerjointsoftheknee,ankle,orwrist.Asymmetricarthritismaybeacuteorchronicandmaybedestructivein15%ofpatients.ImmunoglobulinM(IgM)rheumatoidfactor(RF)ispresentin10%ofchildrenwiththisJIAsubgroup.Itisassociatedwithsubcutaneousnodules,erosions,andapoorprognosis.Approximately40%ofthesepatientstestpositiveforANA.Systemicsymptoms,includinganorexia,anemia,andgrowthretardation,aremoderate.Anintermediateriskforuveitisexists.

SystemiconsetJIA(1020%)isequalfrequencyinboysandgirlsandcanappearatanyage.Symmetricpolyarthritisispresentandmaybedestructivein25%ofpatients.Hands,wrists,feet,ankles,elbows,knees,hips,shoulders,cervicalspine,andjawmaybeinvolved.ANAispositiveinonly10%ofthepatients.Systemiconsetisassociatedwithfever(highineveningandnormalinmorning),macularrash,leukocytosis,lymphadenopathy,andhepatomegaly.Pericarditis,pleuritis,splenomegaly,andabdominalpainlesscommonlyareobserved.Alowriskforuveitisexists.

Pathophysiology

ThecauseofuveitisandarthritisinJIAremainsunknown.[9]Akintomanyotherautoimmunediseases,thetargetantigenisunidentified.Immunereactionstoocularantigens(Santigenoririsantigen)havebeenstudiedhowever,theiractualrole(activeorpassive)isunknown.Thecourseofthediseasemaybeshortandlimitedorprogressiveandsevere.

Epidemiology

Frequency

UnitedStates

JIAhasanestimatedprevalenceofabout113casesper100,000children.ItisestimatedthatJIAafflicts60,00070,000children,butonlyaminoritydevelopeyedisease.IncidenceofeyediseaseintheJIApopulationisuncertain,butitisbelievedtobearound10%.[7]

Mortality/Morbidity

MorbidityinJIAassociateduveitismayresulteitherfromlackoftreatmentorfromoverzealoustreatment.Mortalitymayresultfromthelatter.

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Race

Noknownracialpredilectionexists.

Sex

Astrongpredilectionexistsforgirls.Thegirltoboyratiois4:1.

Age

JIAisachildhooddisease.

Bydefinition,JIAoccursinchildrenyoungerthanage16years.Inviewofthefactthattheoculardiseasecanfollowthesystemicdiseasebynumerousyears,alotofpatientsarewellbeyondtheirteenswhentheyareexaminedandtreatedforuveitis.[10]

ContributorInformationandDisclosuresAuthorManoletteRRoque,MD,MBAGeneralManager,FullPartner,OphthalmicConsultantsPhilippinesCo.PresidentandCEO,ChiefRefractiveSurgeon,EYEREPUBLICOphthalmologyClinicSectionChief,OcularImmunologyandUveitis,DepartmentofOphthalmology,AsianHospitalandMedicalCenterSectionChief,OcularImmunologyandUveitis,InternationalEyeInstitute,StLuke'sMedicalCenterGlobalCitySeniorEyeSurgeon,TheLASIKSurgeryClinicDirector,AMCEyeCenter,AlabangMedicalCenterPresident,PhilippineOcularInflammationSociety

ManoletteRRoque,MD,MBAisamemberofthefollowingmedicalsocieties:AmericanAcademyofOphthalmicExecutives,AmericanAcademyofOphthalmology,AmericanSocietyofCataractandRefractiveSurgery,AmericanSocietyofOphthalmicAdministrators,AmericanUveitisSociety,InternationalOcularInflammationSociety,PhilippineMedicalAssociation,PhilippineOcularInflammationSociety,andPhilippineSocietyofCataractandRefractiveSurgery

Disclosure:Nothingtodisclose.

Coauthor(s)BarbaraLRoque,MDFullPartner,OphthalmicConsultantsPhilippinesCoServiceChief,PediatricOphthalmologyandStrabismus,DepartmentofOphthalmology,AsianHospitalandMedicalCenterActiveStaff,InternationalEyeInstitute,StLuke'sMedicalCenterGlobalCityVisitingOphthalmologist,AMCEyeCenter,AlabangMedicalCenter

BarbaraLRoque,MDisamemberofthefollowingmedicalsocieties:AmericanAcademyofOphthalmology,AmericanAssociationforPediatricOphthalmologyandStrabismus,AmericanSocietyofCataractandRefractiveSurgery,PhilippineAcademyofOphthalmology,PhilippineSocietyofCataractandRefractiveSurgery,andPhilippineSocietyofPediatricOphthalmolo

Disclosure:Nothingtodisclose.

ElisabettaMiserocchi,MDFellowinImmunologyandUveitisService,DepartmentofOphthalmology,HarvardMedicalSchool

Disclosure:Nothingtodisclose.

CStephenFoster,MD,FACS,FACR,FAAOClinicalProfessorofOphthalmology,HarvardMedicalSchoolConsultingStaff,DepartmentofOphthalmology,MassachusettsEyeandEarInfirmaryFounderandPresident,OcularImmunologyandUveitisFoundation,MassachusettsEyeResearchandSurgeryInstitution

CStephenFoster,MD,FACS,FACR,FAAOisamemberofthefollowingmedicalsocieties:AlphaOmegaAlpha,AmericanAcademyofOphthalmology,AmericanAssociationofImmunologists,AmericanCollegeofRheumatology,AmericanCollegeofSurgeons,AmericanFederationforClinicalResearch,AmericanMedicalAssociation,AmericanSocietyforMicrobiology,AmericanUveitisSociety,AssociationforResearchinVisionandOphthalmology,MassachusettsMedicalSociety,RoyalSocietyofMedicine,andSigmaXi

Disclosure:Nothingtodisclose.

SpecialtyEditorBoardAndrewADahl,MDDirectorofOphthalmologyTeaching,MidHudsonFamilyPracticeInstitute,TheInstituteforFamilyHealthAssistantProfessorofSurgery(Ophthalmology),NewYorkCollegeofMedicine

AndrewADahl,MDisamemberofthefollowingmedicalsocieties:AlphaOmegaAlpha,AmericanAcademyofOphthalmology,AmericanCollegeofSurgeons,AmericanMedicalAssociation,AmericanSocietyofCataractandRefractiveSurgery,andWildernessMedicalSociety

Disclosure:Nothingtodisclose.

SimonKLaw,MD,PharmDClinicalProfessorofHealthSciences,DepartmentofOphthalmology,JulesSteinEyeInstitute,UniversityofCalifornia,LosAngeles,DavidGeffenSchoolofMedicine

SimonKLaw,MD,PharmDisamemberofthefollowingmedicalsocieties:AmericanAcademyofOphthalmology,AmericanGlaucomaSociety,andAssociationforResearchinVisionandOphthalmology

Disclosure:Nothingtodisclose.

RChristopherWalton,MDProfessor,DirectorofUveitisandOcularInflammatoryDiseaseService,DepartmentofOphthalmology,UniversityofTennesseeCollegeofMedicine

RChristopherWalton,MDisamemberofthefollowingmedicalsocieties:AmericanAcademyofOphthalmology,AmericanCollegeofHealthcareExecutives,AmericanUveitisSociety,AssociationforResearch

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inVisionandOphthalmology,andRetinaSociety

Disclosure:Nothingtodisclose.

LanceLBrown,OD,MDOphthalmologist,AffiliatedWithFreemanHospitalandStJohn'sHospital,RegionalEyeCenter,Joplin,Missouri

Disclosure:Nothingtodisclose.

ChiefEditorHamptonRoySr,MDAssociateClinicalProfessor,DepartmentofOphthalmology,UniversityofArkansasforMedicalSciences

HamptonRoySr,MDisamemberofthefollowingmedicalsocieties:AmericanAcademyofOphthalmology,AmericanCollegeofSurgeons,andPanAmericanAssociationofOphthalmology

Disclosure:Nothingtodisclose.

AdditionalContributorsTheauthorsandeditorsofMedscapeReferencegratefullyacknowledgetheassistanceofRyanIHuffman,MD,withtheliteraturereviewandreferencingforthisarticle.

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