Current Approach in The Diagnosis and Management of Posterior Uveitis

5/25/2016
Current approach in the diagnosis and management of posterior uveitis
IndianJOphthalmol.2010JanFeb58(1):2943.
PMCID:PMC2841371
doi:10.4103/03014738.58470
Currentapproachinthediagnosisandmanagementofposterioruveitis
SSudharshan,1SudhaKGanesh,1andJyotrimayBiswas2
1
MedicalResearchFoundation,18,CollegeRoad,SankaraNethralaya,Chennai600006,India
2
MedicalandVisionResearchFoundation,18,CollegeRoad,SankaraNethralaya,Chennai600006,India
Correspondenceto:Dr.JyotirmayBiswas,MedicalandVisionResearchFoundation,18,CollegeRoad,SankaraNethralaya,Chennai600
006,India.Email:drjb@snmail.org
Received2008Sep4Accepted2009Aug3.
CopyrightIndianJournalofOphthalmology
ThisisanopenaccessarticledistributedunderthetermsoftheCreativeCommonsAttributionLicense,whichpermitsunrestricteduse,
distribution,andreproductioninanymedium,providedtheoriginalworkisproperlycited.
Abstract
Posterioruveiticentitiesarevariedentitiesthatareinfectiveornoninfectiveinetiology.Theycanaffect
theadjacentstructuressuchastheretina,vitreous,opticnerveheadandretinalbloodvessels.Thorough
clinicalevaluationgivesacluetothediagnosiswhileancillaryinvestigationsandlaboratorytestsassistin
confirmingthediagnosis.Newerevolvingtechniquesintheinvestigationsandmanagementhaveincreased
thediagnosticyield.Incaseofdiagnosticdilemma,intraocularfluidevaluationforpolymerasechain
testingforthegenomeandantibodytestingagainstthecausativeagentprovidegreaterdiagnosticability.
Keywords:GoldmanWitmercoefficient,immunosuppressives,infectiveposterioruveitis,ocular
toxoplasmosis,oculartoxocariasis,oculartuberculosis,polymerasechainreaction,systemicsteroids,white
dotsyndromes
Posterioruveitiscancommonlybeinsidiousinonsetalthoughitcanhaveanacutepresentation.This
sightthreateningconditionhaspathognomonicclinicalfeaturesidentifiableonclinicalexamination.
AccordingtotheStandardisationofUveitisNomenclatureworkinggroup[1]classification,posterior
uveitisisclassifiedasgiveninTable1.
Posterioruveitiscanhaveinflammationinvolvingadjacentstructuressuchastheretina,vitreous,optic
nervehead,retinalvessels,alongwithchoroidalinflammation.
Athoroughdiagnosticworkupdirectedbythehistoryofpresentingcomplaints,patient'ssymptomsand
signs,andclinicalexaminationismandatory.Ancillaryinvestigationssuchasfundusfluorescein
angiography(FFA),indocyaninegreenangiography(ICG),ultrasonography(USG),opticalcoherence
tomography(OCT),andselectivelaboratoryinvestigationshelpinconfirmingthediagnosis.Itisof
paramountimportancetoidentifythepossibleetiologyasposterioruveitiscanbeinfectiveornon
infective.Newerdiagnosticmodalitieshaveledtoearlyrecognitionoftheconditionanditspossible
etiology,whichhasapositiveinfluenceonthemanagementofthedisease.Thisarticlegivesanoverview
ofthecurrentapproachinthediagnosisandmanagementofcommonposterioruveiticentitiesfoundin
India.
Clinicalapproachtoapatientofposterioruveitis
History:Thoroughhistoryisacriticallyimportantstepinevaluatinganypatientwithuveitis.Itallowsthe
cliniciantogaincriticalevidenceregardinggeneralmedicalhistory,travelhistory,socialhistory,
associatedmedicationsandfamilyhistory.Theseoftenprovidecriticallyusefulinformation.
Criticalquestionsfordiagnosis:Answerstothefollowingsetofquestionspavesthewayforanaccurate
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC2841371/?report=printable
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diagnosisandmanagementofposterioruveiticentities.Itisalsohelpfulinidentifyingthepossible
etiologyenablingappropriatetreatment.
1.Isitposterioruveitisonlyorisitpartofapanuveitis?
2.Isitchoroiditis,retinitis,orretinochoroiditis?
3.Isthereassociatedinvolvementoftheopticnerveheadand/ortheretinalvessels?
4.Doestheclinicalfeaturefitintoanyknowninfectiveornoninfectiveentity?
5.Isthereassociatedanteriorsegmentinflammation,vitritis,orcomplications?
6.Isitassociatedwithothersystemicfeatures?
7.Isitrecurrent?Ifso,howhasitrespondedtoprevioustherapy?
8.Isitassociatedwithanimmunocompromisedstate?
9.Isitamasqueradesyndrome?
Posterioruveiticdiseasescanbeclassifiedbasedon
1.Etiology
1.Infectivecauses
Toxoplasmosis
Toxocariasis
Tuberculosis(TB)
Syphilis
Bartonella
Viral(Herpessimplex,Varicellazoster,cytomegalovirus[CMV])
Humanimmunodeficiencyvirus(HIV)relatedeyediseases
2.Noninfectivecauses
Acuteposteriormultifocalplacoidpigmentepitheliopathy(APMPPE)
Multipleevanescentwhitedotsyndrome(MEWDS)
Geographichelicoidperipapillarychoroidopathy(GHPC)
Multifocalchoroiditis(MFC)
Punctateinnerchoroidopathy(PIC)
Birdshotchoroidopathy
Presumedocularhistoplasmosissyndrome(POHS)
Subretinalfibrosisanduveitissyndrome(SFU)
Diffuseunilateralsubacuteneuroretinitis(DUSN)
Retinalpigmentepithelitis(Krill'sdisease)
Sarcoidosis
2.Clinicalcharacteristicsofalesion
Choroiditis
Retinochoroiditis/chorioretinitis
Retinitis
Neuroretinitis
Granuloma
Masslesions(masqueradingasuveitis)
Investigationsinthediagnosisofposterioruveitis
Ancillaryinvestigations:Aprovisionalclinicaldiagnosiscanbereachedinmostcasesofposterioruveitis.
Ancillaryinvestigationsassistinnotonlyconfirmingtheclinicaldiagnosisbutalsoincasesofdiagnostic
dilemma.
Colorfundus/FFA:Serialdocumentationoflesionswithcolorfundusphotographscanassistinthefollow
upofthediseasewithtreatment.FFAmaybeusefulinconfirmingtheactivityofachoroiditis/retinitis
denotedbyacharacteristicearlyhypofluorescenceandlatehyperfluorescenceincaseofactivechoroiditis.
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Itcanbeusedtodetectdiseasesequelaesuchasneovascularization,capillarynonperfusionareas,and
vascularstainingincasesofretinalvasculitis.Itcanrevealatypicalflowerpetalpatternincystoidmacular
edema(CME)oraspoolingofdyeinlatephaseinVKH.FFAismostusefultodetectthepresence,type,
andactivityofchoroidalneovascularization(CNV),whichisavisionthreateningcomplicationassociated
withmanyposterioruveiticentities.
ICG:ICGismorehelpfulincaseofdeeperchoroidallesions,CNV,andinthepresenceofhemorrhages.It
isespeciallyusefulindetection,identification,andfollowupofentitieswithdeeperchoroidallesionssuch
asWhitedotsyndromes,whereFFAmaynotbecompletelyconfirmatory.
OCT:OCTisanoncontactimagingtoolhelpfulindetectingandmonitoringmacularpathologiessuchas
CME,epiretinalmembrane,CNVmembrane(CNVM),andmacularhole.Theconventionaltimedomain
OCThasaresolutionof10micronswhilethenewerspectraldomainOCT(SDOCT)hasincreasedthe
resolutionto5micronsandgivesathreedimensionalviewenhancingitsdiagnosticpotential.
UltrasoundBscan(USG):Itisaveryusefultool,especiallywhenthemediaishazyandincaseswith
cataractorseverevitritisorvitreoushemorrhage.ThereisanindicationforUSGBscanevenwhenthere
isarelativelyclearmediainmanyposteriorandpanuveiticconditions.Ithelpsdifferentiate
rhegmatogenousandexudativeretinaldetachmentbasedontheshiftingoffluid,whichismore
characteristicofanexudativeretinaldetachment.Anincreasedchoroidalthickeningcanbeasignificant
finding.ItmaybeseeninVogtKoyanagiHarada'sdisease(VKH)withsignificantposterioruveitic
manifestationsandinposteriorscleritis.Normalchoroidalthicknessisaround1.1mm.PresenceofT
signand/orTenon'sspacewideningnotedinUSGBscanispathognomonicofposteriorsclerits.Itisalso
usefultodiagnoseintraoculartumorsmasqueradingasuveitisandelevatedmasslikelesionssuchasTB
subretinalabscess.
Laboratoryinvestigations
Tailoredlabinvestigationrelevanttotheclinicalentityinquestionistherightapproachinidentifyingthe
etiologyofaposterioruveiticentity.Laboratorytestsaremoreusefulininfectivethaninnoninfective
conditions.Specifictestsforeachentityhavebeendescribedlater.Incasesofdiagnosticdilemma,
intraocularfluidevaluationforpolymerasechainreaction(PCR)andantibodytitershelpsclinchthe
diagnosis.
Systemicexamination
Itisextremelyimportantthatthepatientbeevaluatedthoroughlybyaninternisttoruleoutpossible
associatedcausesofhis/heruveitisandalsotoevaluatethelaboratorytestfindings,asmostoftheuveitic
entitiescanhavesystemicassociations.Thetreatmentofthepatientisincompletewithoutsimultaneous
treatmentoftheunderlyingsystemiccondition.
Treatment
Localandsystemicsteroidsalongwithimmunosuppressivesinselectcasesarethemainstayoftreatment
ofnoninfectiveconditions.Infectiveconditionsneedtobetreatedprimarilywiththespecificanti
infectiveagentsalongwithantiinflammatorytherapyintheformoflowdosesteroids.Incaseofinfective
uveitis,systemicsteroidsneedtobeinitiatedatleast4872hafterstartofspecificantiinfectivetherapy
andthenstoppedatleast1weekpriortostoppageofspecifictreatment.
Infectiveposterioruveitis
Infectiveposterioruveitisisaclinicaldiagnosisbasedoncharacteristicfunduspictureandrelevant
positivehistory.Laboratoryinvestigationsarepredominantlybasedonantibodytestingagainstthespecific
antigenandPCRtestingfortheparticulargenome.Otherteststodetectassociatedsystemicconditionmay
berequiredtoclinchthediagnosis.Itisimportanttotreattheunderlyingsystemicdiseasealongwith
ophthalmictreatment.BasicmanagementapproachisgiveninTable2.
Acomprehensiveoverviewofthecharacteristicclinicalappearanceofcommoninfectiveposterioruveitic
entitiesandthecurrentmanagementapproachisbrieflydescribedbelow.
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Oculartoxoplasmosis
Clinicaldiagnosis:Oculartoxoplasmosisisthemostcommoninfectivecauseofposterioruveitisin
immunocompetentpatients.Mostcasesoftoxoplasmosisintheimmunocompetenthostaresubclinicalor
benign.Prevalenceishigherintropicalcountriesthaninaridorcoldareas.[3]Apositivehistoryofcontact
withpetssuchasdogsandcats,[2,3]ingestionofraw,undercookedmeat,orcontaminatedmunicipal
water[4]canidentifythesource.TransplacentaltransmissionofT.gondiiistheonlyformofhumanto
humantransmission.
Toxoplasmicretinochoroiditisisunilateralin7283%ofthecases.[3]Oculartoxoplasmosisoccursfrom
theactivationofcystsdepositedinorneartheretina.Focalnecrotizingretinitisisthecharacteristiclesion.
Peripheralretinochoroidalscarsarethemostcommonocularfinding,occurringin82%ofthepatients.
However,toxoplasmahasastrongpredilectionfortheposteriorpole,particularlythemacularregion,[2]
thislocationoccurringinmorethan50%ofthecases.[3]
Congenitaltoxoplasmosis
Presenceofanasymptomaticpunchedoutmacularcicatriciallesionwithacentralnecroticzoneinvolving
theretina,choroid,andvitreousisdiagnostic[Fig.1].Ocularinfectionmaybetheonlymanifestationof
congenitaltoxoplasmosis.Tenpercentofthepatientshaveocularlesionswithoutclearevidenceofdisease
inotherorgans.[2,3]
Recurrentlesionsfrequentlydevelopatthebordersoftheoldtoxoplasmascars,socalledsatellitelesions,
andarecalledreactivationofcongenitaltoxoplasmosis.
Acquiredtoxoplasmosis
Patientswithoculartoxoplasmosiswithmacularinvolvementusuallypresentwithdiminishedvision
and/orfloaters.Headlightinthefogappearanceofafocalnecrotizingretinochoroiditiswithoverlying
vitritisisthecharacteristic[Fig.2].Classically,theinitiallesionstartsinthesuperficialretina,gradually
involvingthefullthicknessretina,adjacentchoroid,vitreous,andevensclera.Ayellowishwhiteorgrey
exudativelesionisseenwithilldefinedbordersbecauseofthesurroundingareaofretinaledema.Thesize
ofthelesionvariesfromafractionofthedisctoabouttwoquadrantsoftheretina.Adjacentchoroiditis,
hemorrhage,andvitreitismaybeseen.Itisrelativelyasymptomaticinperipherallesions,[3]seenin70
90%,andpatientsmaypresentwithonlyfloaters.
Vascularinvolvementmaybenotedclosetotheactivelesionorinthedistantretinaandcanpresentasa
diffuseorsegmentalvasculitis.Thisisproducedbytheantigenantibodycomplexdepositionand/or
localizedmononuclearcellinfiltratesinthevesselwall.Althoughphlebitisiscommon,arterial
involvementisalsoseen.Kyrieleisarterialitis(exudatesorperiarterialplaques)canalsobeseen.[2]
Thehealedscarhaswelldefinedbordersaroundthecentralretinochoroidalatrophy.Uncommon
presentationsincludeassociatedserousmaculardetachment,[5]retinalvasculitis,neuroretinitis[6]with
papillitis,dischemorrhageswithvenousengorgement,andmacularstar.Anterioruveitisisacomplication
oftheretinochoroiditisandthepresenceoftheparasiteintheanteriorsegmenthasbeendemonstratedin
immunocompromisedpatients.[3]
Inimmunocompromisedindividuals,asinacquiredimmunedeficiencysyndrome(AIDS),punctateouter
retinaltoxoplasmosisornecrotizingretinitislesionsmimickingviralretinitismaybeseen.
Secondaryglaucomaisthemostcommoncomplication.Othersincludecataract,vitreoushemorrhage,
retinaldetachment,CNVM,CME,vascularocclusions,andopticatrophy.
Confirmationofdiagnosis:DiagnosisismainlyclinicalandancillaryinvestigationslikeFFA,ICG,[7]and
OCTarecomplimentary.[3]Althoughdetectionoftoxoplasmaspecificantibodiesinserumisusefulin
atypicalcases,hightitersofpositivetoxoplasmaantibodiesinthenormalhumanpopulationmay
complicateresults.Serumantitoxoplasmaantibodytiterscanbedeterminedbyseveraltechniquessuchas
SabinFeldmandyetest(Goldstandard),complementfixationtest,hemagglutinationtest,
immunofluorescenceantibodytest,enzymelinkedimmunosorbentassay(ELISA),immunoblotting,and
immunosorbentagglutinationassay,butELISAisthemostcommonserologicaltestemployed.
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Antibodydetectionandcharacterizationdifferentiatesrecentlyacquiredandchronicinfections[3]
(immunoglobulin[Ig]MandIgGrespectively).Acutesystemictoxoplasmosishastraditionallybeen
diagnosedbyseroconversion.AntiToxoplasmaIgGtiterspresenta4foldincreasethatpeak68weeks
followinginfection,thendeclineoverthenext2years,butremaindetectableforlife.
AntiToxoplasmaIgMappearsinthefirstweekoftheinfectionandthendeclinesinthenextfewmonths.
Antibodytitersintheserumdonotalwayscorrelatewithoculardiseaseandhencemanagementhastobe
basedontheclinicaldiagnosis.AntiToxoplasmaantibodiesmaybeverylowandshouldbetestedin
undiluted(1:1)samplesifpossible.ItistobenotedthatraisedIgGantibodytitres,whichmaybeseenin
otheruveiticconditions,shouldnotformthebasisfortreatmentwithantiToxoplasmadrugsifthereisno
clinicalevidencesuggestiveofactiveoculartoxoplasmosis.
PCRisanimportanttoolinthediagnosisofoculartoxoplasmosis,especiallyincasesofequivocal
serology.VariousgroupshavecomparedPCRandGoldmanWitmer(GW)coefficientanalysisandhave
beenfoundtobeofequalutility.
Incaseofdiagnosticdilemma,aqueousorvitreoussamplesmaybeevaluatedforthepresenceof
ToxoplasmaDNAsequences,usingthistechnique.Antibodiestitersaremeasuredinaqueoushumorand
serumandGWcoefficientiscalculated.
AcombinationofPCRtestingandGWcoefficientofantibodytitersinaqueousorvitreous[810]hasa
highdegreeofspecificityandsensitivity.AnalysisofIgG,IgM,andIgAincreasesthesensitivityofthe
aqueoushumorstudy[2,911]andhelpsruleoutviraletiology,especiallyinimmunocompromised
individuals.
Treatment:Anidealcombinationthatdestroystissuecystsandpreventsrecurrencehasnotbeen
found[2,1214]ascurrenttherapiesaretargetedonlyontrophozoites.Pyrimethamine(100mg1stday,75
mg2ndday,50mg3rdday,followedby25mgoncedaily)andSulfadiazine(4gdailydivided6thhourly)
for46weeks,isthemosteffectivecombinationthatworkssynergistically.Theotherdrugsusedinthe
treatmentoftoxoplasmosisaregiveninTable3.
Treatmentregimenconsistingofasulfonamideandanonsulfonamidewithsystemicsteroidsandfolic
acidsupplementsispreferred.Inpatientswithsulfaallergy,clindamycinandazithromycinaresuitable
alternatives.Topicalsteroidsandcycloplegicsareusedtotreatassociatedanterioruveitis.
Immunocompromisedindividualsrequirelongtermprophylaxistillimprovementinimmunestatusofthe
individualevenafterresolutionoflesions.[3]Itisimportanttoruleoutassociatedcentralnervoussystem
involvementinpatientswithAIDS.
Modificationsinthetreatmentregimeninvariousspecialsituationssuchaspregnancyandinneonatesis
giveninTable4.
ToxoplasmicCNVMhasbeentreatedeffectivelywithverteporfinphotodynamictherapy.[15,16]Pars
planavitrectomyisperformedtotreatpersistentvitreousopacitiesorvitreoretinaltraction.Anewer
fluoroquinolone,trovafloxacin,haspotentantiToxoplasmaactivityandappearspromising.Sobrinetal.
[17]reportedfavorableresponseinsixpatientsoftoxoplasmicretinochoroiditiswithintravitreal
clindamycinwith/withoutparsplanavitrectomy.
Oculartoxocariasis
Clinicaldiagnosis:Toxocariasisisaninfectioncausedbytheaccidentalingestionoflarvaeofthedog
roundwormToxocaracanisorthecatroundwormToxocaracati.Childrenwhohavepicaandareinclose
contactwithpuppiesareparticularlyvulnerable.Oculartoxocariasisisdiagnosedbasedonapositive
historyofcontactwithpetsandsuggestiveocularfindings.Riskofhumaninfectionishigherinchildren
withpicawhomayingestcontaminatedsoilormeat.Infectionsinhumans,anendhost,resultinafocal
granulomatousreactioninmanyorgans,includingtheeye.
Variousclinicalmanifestationsaccordingtothedecreasingpreferenceoftheparasiteare:
1.Granulomaintheperipheralretinaandvitreous
2.Posteriorpolegranuloma
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3.Chronicendophthalmitis
4.Opticnerveinvolvement
5.Anteriorsegmentinvolvement
Presenceofaposteriorpoleoraperipheralgranuloma[Fig.3]withtractionalretinaldetachmentora
chronicendophthalmitislikepicture[18]impairingvisualisationistypical.Earlylesionsmaybepoorly
visualizedbecauseofintensevitreoushaze.Longstandingmassesmayhavesubstantialsecondaryatrophy
andhyperplasiaoftheretinalpigmentepithelium(RPE).Whitish/grayishwhitegranulomasofvarious
sizesmayrarelybeseeninjuxtapapillaryandsubfoveallocations.Inferiorlylocatedlesionscan
sometimesbemistakenforparsplanitis.Deadlarvaearesometimesseenasadarkgreyareawithinthe
whitishmassattheposteriorpole.CNVMandasubretinaltoxocaragranulomahavetoberuledout.[19]
Confirmationofdiagnosis:Diagnosisismainlyclinical,althoughELISAwithToxocaraexcretory
secretoryantigen(TESAg)hasbeenshowntobehighlyspecificfortoxocarainfection.Anincreaseof
antiTESAgIgElevelindicatesacutetoxocarainfectionorprogressiveinflammation.Anincreaseinthe
IgGlevelconfirmsapastorpresentinfectionwithminimuminflammation.ToxocaraGWcoefficient
analysisfromaqueousandserumcanbeofvaluewhendiagnosingpatientswithposteriorfocallesionsor
vitritisofunknownetiology.[20]USG/computerizedtomography(CT)findingshaveadditionalvalue.
Differentialdiagnosis:
Retinoblastomamaymimictoxocariasiswithcalcification
Ruleoutendophthalmitis,severeparsplanitis,juvenileidiopathicarthritisassociateduveitis,Coats'
disease,primaryhypertrophicproliferativevitreopathy,familialexudativevitreoretinopathy,andlate
stagesofretinopathyofprematurity.
Treatment:Nolargecasecontroltrialasyethascomparedantihelminthictherapyforoculartoxocariasis
againstobservationalone.Systemicsteroidsisthemainstayanditisimportanttomonitoritssideeffects,
especiallygrowthretardationinchildren.Surgicaltreatmentsuchasparsplanavitrectomy,cryopexy,and
laserphotocoagulationhasbeenusedtotreatcomplications.Vitrectomymaybebeneficialforpatients
withendophthalmitis,unrelievedvitreoretinaltractionwithretinaldetachment,[1]andalsoasanoptical
indication.
Tubercularposterioruveitis
OcularTBcanbeprimary,wheretheeyeistheinitialsiteofentryorsecondary,whereorganismsspread
totheeyehematogenouslythistypeincludestuberculousuveitis.Hypersensitivityreactiontotuberculous
proteincanalsocauseretinalvasculitis.Astudyfromourcenternotedthatonly1.39%patientswith
systemicTBhadtubercularuveitis.
Clinicaldiagnosis:Themostcommonpresentationoftuberculousuveitisisofdisseminatedchoroiditis.
[21]Choroidaltuberclesmaybeoneoftheearliestsignsofdisseminateddisease.Thelesionsmayvary
fromfewnumberstoseveralhundred.Thelesionsrangefrom0.5to3.0mmindiameterandmayvaryin
sizeandelevationwithinthesameeye.Theyaredeepinthechoroid,appearyellow,white,orgray,andare
fairlywellcircumscribed.Inthevastmajorityofcases,thelesionspresentintheposteriorpole.
Thenextmostcommonpresentationisasingletubercle,alsotermedfocalchoroiditis,[22]whichcan
occurattheposteriorpole.Asinglechoroidalmassisthecharacteristicfeatureonpresentation,although
multiplechoroidaltuberclescanbeseenincasesofmiliarytuberclesandinimmunosuppressed
individuals.Alargetuberclemaymeasureupto4.0mmindiameterhowever,choroidalmassesupto14
mmindiameterhavebeenreported.Themassistypicallyelevatedandmaybeaccompaniedbyan
overlyingserousretinaldetachment[23][Fig.4].Subretinalabscessisformedprogressivelyfroma
choroidaltubercle,whichcanbesingleormultiple.Diagnosisisbasedonclinicalfeatures,suggestiveof
systemicfindingsandsupportiveinvestigations.
Solitarytubercularchoroidalgranuloma[24,25]canbemisdiagnosedaschoroidalmelanomadueto
multiplicityofclinicalfindings,whichalsocausesdiagnosticdelay.Otherpresentationsinclude
serpiginouslikechoroiditis,[26]retinalvasculitis,[27]intermediateuveitis,panuveitis,andneuroretinitis.
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Confirmationofdiagnosis:Angiographyconfirmsactivityinchoroiditis[28]andrevealsaclassicalring
offireappearanceinthesubretinalabscess/choroidalgranuloma.Retinalvasculitiscanpresentwith
neovascularizationandcapillarynonperfusionareasduetoinflammatoryvesselobstruction,whichmay
needprophylacticlaserpanretinalphotocoagulation.OCTscansthroughareasofsuspectedgranulomacan
behelpfulindifferentiatingchoroidalgranulomasfromothernoninflammatoryconditions,suchascentral
serouschorioretinopathy(CSR),exudativeagerelatedmaculardegeneration,andchoroidaltumors
mimickingchoroidalgranuloma.InTBgranuloma,OCT[29]revealsanareaoflocalizedadhesion
betweenthechoriocapillarisRPElayerandoverlyingtheneurosensoryretina(contactsign),possibly
duetoinflammatoryadhesionsoverlyingthegranulomathatcausetheneurosensoryretinatosticktothe
RPEatthatpoint.Inflammatorycellsappearasincreasedreflectivityinthedeeperretinallayersoverthe
granuloma.Thesefeatures,uniqueinTBandotherinflammatoryconditions,areunusualinnon
inflammatorylesions.USGBscanhelpsruleouttumorsinlargemasslikesubretinalabscesses.
Associatedsystemiclatent/manifestTBconfirmsdiagnosis.Mandatoryinvestigationsincludehemogram,
erythrocytesedimentationrate(ESR),Mantouxtest,andradiologicalimagingsuchaschestXray/CT
scan.
Itisimportanttonotethattuberculintestcanbefalsepositiveinpatientshailingfromendemiccountries
likeIndiaandisalsoaffectedbypreviousBCGvaccination.
NewertestsbasedongammainterferonassayssuchastheQuantiferonTBGoldtestarepromising.[30]
OthersincludeBACTECMGIT960system,MB/BacTsystem,andtheESPIIculturesystem.
Histopathologicaland/ormicrobiologicalconfirmationofmycobacteriuminfection,especiallybyPCR,
fromintraocularspecimensisdiagnostic.IS6II0primerbasedPCRiswidelyusedfordetectionoftheM.
tuberculosiscomplex.However,nestedPCRtechniqueemployingtheMPB64geneis10,000timesmore
sensitiveand100%specificandisusedincasesofdoubt.[3133]RealtimePCRtechnologycan
differentiatecommensalsandcontaminantsfrominfectingmicrobes.DotblothybridizationofthePCR
productby32Plabeledspecificprobesimprovessensitivity.[34]
NOTE:ELISAandPCRtestingforTBonserumarenotuseful,especiallyinendemicregionslikeIndia,
andshouldnotformthebasisofdiagnosisofintraocularTB.
Treatment:Itisimperativethatantituberculoustherapy(ATT)beinitiatedundercareofaninternistonce
tuberculousetiologyisconfirmed.Concomitantsystemicsteroidsfor46weekshaveaprotectiveeffect
againsttissuedamagefromdelayedtypeofhypersensitivity(DTH).Useofcorticosteroidsaloneshouldbe
avoidedasitpromotesmultiplicationofbacilliandcanleadtopanophthalmitis.Guidelinesdescribedby
Guptaetal.[35]canformthebasisfordiagnosisandmanagementofocularTB.Successfulmedical
managementofsubretinaltuberculargranulomahavealsobeendescribed,[36]whereauthorshave
concludedthatoncethediagnosisofpresumedorconfirmedTBisestablished,surgicalintervention
shouldbeavoidedandsuccessfulresolutionoflesionscanbenotedwithATTandsteroidsalone.Surgery
isanoptionmainlyforcomplications.[37]
What'snew?
NewerquinolonesandrifamycinslikerifabutinandmacrolidesarepartofATTregimes,especially
inmultidrugresistantTB.
UsefulnessofRetinalamineinpatientswithTBchorioretinitisisbeingevaluated.[38]
Viralretinitis
Aretinitisshouldevokeasuspicionofaviralinfection,whichisusuallycausedbyHerpessimplex,
Varicellazoster,andCMV.Otherrarecausesincludechikungunyaandrubellaviruses.
Herpeticeyediseaseisamongthemostcommoncausesofinfectiousuveitis.Itmayaffecthealthyaswell
asimmunocompromisedhosts,althoughitsclinicalpresentationvariesaccordingly.Posterioruveitis
causedbyherpesvirusesmayappearaspartofherpeticdiseaseelsewhere(skin,brain,anteriorsegmentof
theeye)orasanisolatedfinding.Mostformsofherpeticposterioruveitisareacuteandfulminant,often
resultinginseriouscomplicationssuchasretinaldetachmentandproliferativevitreoretinopathy.
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Presentationofherpeticinfectionsvariesbasedontheimmunestatusoftheindividual.Associated
systemicherpeticmanifestations,apasthistoryofchickenpox,orsignsandsymptomsofan
immunocompromisedstatemaybediagnosticclues.
Clinicaldiagnosis:Acuteretinalnecrosis(ARN)istheclassicalpresentationofherpeticviruses.
Characteristicclinicaltriadofmoderatetoseverevitritis,arteritis,andperiphlebitisandconfluent
peripheralretinalnecrosisisdiagnosticofARN,whichcanpresentasapanuveitis.Althoughtheposterior
poleisnottypicallyaffectedearlyinthediseaseprocess,itcanalsobeinvolvedprimarilyinnecrotizing
herpeticretinopathies.
Progressiveouterretinalnecrosis(PORN):Necrotizingretinitis,confluentareasofouterretinalwhitening
withminimalvitritisinvolvingtheposteriorpoleandsparingofretinalvesselsattheearlystage,the
typicalcrackedmudappearance[Fig.5]isvirtuallydiagnostic.[39]AlthoughARNandPORNare
consideredtobetwodifferententities,theyareconsideredessentiallyasdifferentmanifestationsofthe
samediseaseandthevariedpresentationsoccurringduetodifferencesintheimmunestatusofthehost.
PORNisfrequentlybilateral,occurringexclusivelyinimmunocompromisedstate,suchasinpatientswith
HIVinfection,[39]andisassociatedwithrapiddevelopmentofrhegmatogenousretinaldetachmentor
opticatrophy.
Viralposterioruveitiscanalsorarelypresentaspatchy,single,ormultipleretinitispatches,whichincludes
acutevaricellaretinitis/choroiditisseeninchildrenaswellaschronicchoroiditisornonnecrotizingretinal
vasculitisinadults.
Chikungunyaretinitis[4042]mimicsherpeticorCMVretinitisandisdiagnosedbasedonahistoryof
ailmentwithchikungunyadiseaseanddetectionofspecificantibodiestothevirusfromserumor
intraocularfluid.Itisoftenselflimitingand,althoughnotspecific,acyclovirorintravitrealganciclovir
havebeenfoundtobebeneficial.
CMVretinitis,usuallyseeninimmunocompromisedstatessuchasinAIDSorpostorgantransplant
patientsonimmunosuppressives,hasacharacteristicgranularorpizzapieappearance[Fig.6]anda
typicalbrushfirepatternofspreadalongthebloodvessels.
Althoughrubellavirus,whichispartoftheTORCHcomplex,canalsocauseocularlesions,rubella
retinopathycanbeclearlydifferentiatedfromretinitisduetoherpeticvirusesasithasacharacteristicsalt
pepperappearanceandcanmimicretinitispigmentosa.
Treatment:Longtermsystemicantiviraldrugssuchasacyclovirorvalaciclovirarethetreatmentof
choiceincaseofherpeticretinitis.AciclovirisveryeffectiveagainstHSVandVZV.Treatmentwith
acyclovirreducesinfectionofthefelloweyefrom70to13%inthefirstyear.[2]
Thedosageis15mg/kgbodyweightinthreedosesfor721days.Then,24gdailyisrecommendedfora
further46weeks.[2]Usually,intravenousacyclovir500mg8thhourlyfor12weeksfollowedbyoral
acyclovir800mg5timesdailyisrecommendedwithadditionallowdosesystemicsteroids.Alternatively,
oralvalaciclovir,whichhasabetterbioavailability,canbegivenas1gthreetimesdaily.Antiviralsmaybe
continuedforalmost36monthsinsomecases.Renalfunctionneedstobemonitoredifantiviralsare
administeredonalongtermbasis,especiallyinextremesofage.Prophylacticlaserbarrageadjacentto
retinitislesionsisknowntopreventretinaldetachment,althoughthereisnorandomizedstudythat
validatestheuseoflaserbarrageforpreventingasubsequentretinaldetachment.Earlyvitrectomyasan
optionhasbeenadvocatedbutalsoneedstobetestedinarandomizedfashion.Inresistantcases,
alternativesincludeacombinationofsystemicandintraocularantiviraltherapywithfoscarnetand
ganciclovir.[43]
Intravenousganciclovir57mg/kg/dayintwodivideddosesfor2weekinductiondosefollowedbyonce
dailymaintenancedosetillcompleteresolutionoflesionsandimprovementofimmunestatusisthe
treatmentofchoiceinCMVretinitis.Intravitrealinjectionsofeitherganciclovirorfoscarnetmaybe
considered,especiallyininitialcaseswherethemaculaisthreatened.Itcanbeawaytogetthegreatest
concentrationofdrugtotheaffectedareaimmediately.
Alternatively,oralvalganciclovir900mgBDasinductionand900mgODasmaintenancedosehasan
additionaladvantageofbeinganonparenteralmodeoftreatment,avoidingcomplicationsrelatedto
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indwellingcatheters,especiallyinimmunocompromisedindividuals.Appropriatemanagementof
underlyingsystemicdiseaseundercareofaninternistpromotesearlyresolutionofocularlesions.
NOTE:Necrotizingretinopathyduetotoxoplasmacloselymimicsherpeticretinitis,especiallyin
immunocompromisedpatients.
CMVretinitiscanalsoinvolvetheposteriorpoleinitiallyand,inearlystages,canmimiccottonwoolspots
ofHIVretinopathy.
Ocularsyphilis
Ocularsyphiliscanmimicanyoftheuveiticentities,andhastoberuledoutespeciallyinanycaseof
infectiveuveitis.Itisthemostcommonintraocularbacterialinfectionandisreemerginginvariedforms,
especiallywiththeadventofAIDS.[44]About12%ofHIVpositivepatientsarefoundtohaveocular
syphilis.
Clinicaldiagnosis:Syphilisiscalledthegreatimitatorasitcanpresentaschorioretinitis,neuroretinitis,
saltpepperretinopathy,opticneuritis,papilloedema,andopticperineuritis.1Syphiliscanpresentwith
placoidchoroidallesionsandcanmimicAPMPPE.Anunusualmanifestationofsyphilisisacute
necrotizingretinopathy,whichmimicsARN.Syphiliticuveitiscanbethefirstpresentationofthesystemic
disease[45]inbothimmunocompetentandimmunocompromisedindividuals.InHIVpositivepatients,
ocularsyphilisismorecloselyassociatedwithneurologicalabnormalities.
Confirmationofdiagnosis:Laboratoryinvestigationssuchasvenerealdiseaseresearchlaboratorytest,
rapidplasmareagintest,andtreponemapallidumhemagglutinationtestsareused.Confirmatorytests
includefluorescenttreponemaantibodyagglutination(FTAABs)testanddarkgroundmicroscopy.A
specifictreponemaltestsuchasFTAABscanbeusedasaninitialtestforasyphilisscreenasitismore
reliableandhaslowfalsepositivity.Patientsoftenshowabnormalcerebrospinalfluidfindings.Diagnosis
isverychallengingasupto38%ofHIVpositivepatientscanbeseronegativedespiteactivesyphilitic
disease.[46]
Treatment:Treatmentofocularsyphilisissimilartothatofneurosyphilis.Longactingpenicillinisthe
treatmentofchoice.
Neuroretinitis:Involvementoftheopticnerveheadalongwithretinitisandmacularstarconstitutesthe
typicalappearanceofaneuroretinitis[Fig.7].Itcanbecausedduetotoxoplasma,TB,syphilis,andother
noninfectiveconditionssuchascollagenvasculardiseases,andistreatedaccordingly.
Catscratchdisease(CSD)causedduetoBartonellaisanimportantcauseofneuroretinitis.Itcanalso
presentwithacutemacularneuronopathy.[47]Doxycycline100mgisthetreatmentofchoice.Multifocal
chorioretinallesionsassociatedwithB.hensalaecanbeatypicalophthalmicmanifestationsofCSD,which
mayoccurinimmunosuppressedpatients.[48]Recognitionandappropriatetreatmentofunderlying
diseasegivesbettervisualoutcomes.
DUSN
DUSN,arareentitycausedbyawormintheeye,isusuallyunilateral.Clinicalvisualizationofsubretinal
nematodemakesthediagnosisobvious.Liveworm,ifdetected,canbedestroyedbydirectlaser
application.Wormanditsbyproductscancausesevereinflammationaffectingtheopticnerveheadand
retinaandneedstobetreatedwithhighdosesystemicsteroids.Ifawormisnotfoundclinically,
appearanceofsubRPEserpiginoustractintheinferotemporalretina,peripheralRPEhypopigmentation,
goodclinicalresponsetoantihelminthics,andabnormalERGsupportsthediagnosis.[49]Treatmentwith
albendazoleisbeneficial.
Intraocularcysticercosis
Cysticercuscellulosecyst,foundinsidetheeye,cancausesevereinflammatoryreaction.[50,51]When
visualizationisimpaired,USGBscanisdiagnostic.Neurologicalassociationneedstoberuledout.
Treatmentiswithsteroidsandantihelminthictherapy.[50]Removalofthecystisnecessaryinmostcases.
Noninfectiveposterioruveitis
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CommonnoninfectiveposterioruveiticentitiesincludetheWDSandarenamedaccordingtotheir
clinicalappearanceandbehavior.FFAandICGarevirtuallydiagnosticinWDS.Laboratoryinvestigations
arenotroutinelynecessaryfortheirdiagnosisbutareessentialformonitoringtherapyrelatedsideeffects.
AsignificantpercentageofpatientswithWDShaveaprodromalvirallikeillnessthattriggerstheonsetof
theocularconditionandmaybeselflimiting,althoughtheyneedtobefollowedupcloselyfor
complicationssuchasscarringandCNVM.PatientswithWDSpresentwithsuddenblurringofvision
associatedwithphotopsia,floaters,scotomata,andmetamorphopsia.Anapproachtoacaseof
inflammatoryposterioruveitisisdipictedin[Table5].
ThecomparativeclinicalcharacteristicsandinvestigativefindingsofvariousWDSaregivenbelow
[Tables68].
OverviewofclinicalanddiagnosticfeaturesofWDS
APMPPE[Fig.8]:Itisaninflammatoryretinal/choroidaldiseasecharacterizedbysuddenlossofvision
causedbythesuddenappearanceofmultipleyellowwhite,flatinflammatorylesionslyingdeepwithinthe
sensoryretina,mostnotablyattheleveloftheRPEandthechoriocapillaries.[1]AtrophyandRPE
scarring[52]resultsinpoorvisualacuity.Newlesionsmaybeobservedintheperipheralfundusforupto3
weeksandtendtobemorelinear.Additionalfindingsincludediscedema,keraticprecipitates,retinal
vasculitis,neurosensorydetachments,andvenousocclusions.[1]OCTfindingsinconjunctionwithFFA
andmicroperimetryareuseful.[53]
APMPPEhasaselflimitedclinicalcourse,withspontaneousrecoveryofvisioninmostcases.Ofthose
eyesthatareaffected,90%ofthemtypicallyachieveavisualacuityofmorethan20/25.Inrarecases,
recurrencesmayoccurwithin6monthsoftheinitialepisode,therebygivingalessfavorableprognosis.
Corticosteroidsandcytotoxicdrugsareindicated,especiallyincasesofassociatedcerebralvasculitis.[54]
CNVisararecomplication.
NOTE:[52,54,55]
APMPPEmimicsnoninflammatoryconditionslikemultifocalchoriocapillaryinfarctsdueto
hypertension,toxemiaofpregnancy,anddisseminatedintravascularcoagulation.[52]
Ruleoutassociatedcerebralvasculitis.[55]
MEWDS:MEWDSisararedisorderofunknownetiologycharacterizedbythepresenceofwhitelesions
deepintheouterretinaorattheleveloftheRPE.Itcanalsopresentwithopticdiscedema,mildvitritis,
panuveitis,diffusechoroidalthickening,andarelativeafferentpupillarydefect.Newlyrecognized
angiographicfeaturestermeddotsandspots,whichvariedinsizeandlocationinthefundus,havebeen
reported.SmalldotswereintheinnerretinaorattheleveloftheRPE,andlargerspotsweremoreexternal
inthesubpigmentepithelialarea.Presenceofwhitedotsaroundthenerveisrareandfielddefectpersists
evenafterlesionsdisappear.[51]
Aviralprodromeisknownin50%ofthepatients,andthemultifocalnatureofthedisease,aviral
prodrome,isthoughtbymany.Patientsalsopresentwithacute,painless,unilaterallossofvision.
ItcanbedistinguishedfromotherWDSsbyitsdistinctmorphology,associatedmaculargranularity,
transientnature,characteristicangiographicappearance,unilaterality,selflimitingcourse,lackof
significantsequelea,absenceofassociatedsystemicinvolvement,rapidrecovery,andexcellentvisual
outcome.
MEWDSisaselflimiteddisease,withalmostallpatientsregaininggoodvisualacuitywithin39weeks.
Thelesionsdisappearwithoutscarringandphotopsiasandscotomatagraduallyresolve.Occasionally,
patientswithMEWDSmayhavepersistentblindspotenlargement.Althoughuncommon,recurrencescan
occur.However,theprognosisisfairlygoodforthesepatients.ArarecomplicationofMEWDSisCNV,
whichmayrequirelaserphotocoagulation.
NOTE:
Carefulevaluationbyslitlampbiomicroscopy[1]isamust.
CharacteristicfindingofMEWDSisfovealgranularity.[56,57]
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MultifocalERGhelpsdifferentiateMEWDSfromotherblindspotenlargingconditions.[58,59]
Serpiginouschoroiditis[60](GHPC)
Itisarare,chronic,progressive,andrecurrentbilateralinflammatorydiseaseinvolvingtheRPE,the
choriocapillaries,andthechoroid.Itischaracterizedacutelybyirregular,graywhiteorcreamyellow
subretinalinfiltratesatthelevelofthechoriocapillariesandtheRPE.Basedonclinicalpresentation,itcan
beclassifiedinto(1)peripapillary,(2)macular,and(3)ampiginoustypes.Theclinicalcourse,regardless
ofthepresentation,isprogressive,withmultiplerecurrencesleadingtopotentiallysignificantvisualloss.
Patientspresentwithunilateralorbilateralvisuallosswhenthemaculaisinvolvedandtheymayalso
noticephotopsiasandscotomata.Theanteriorsegmentusuallyappearsquiet,althoughanon
granulomatousanterioruveitishasbeendescribed.GraywhitelesionsarenotedattheleveloftheRPE.
Activelesionsareusuallyfoundattheborderofinactivelesionsandappearinaninterlockingpolygonal
patternthatspreadsouttheperipheryfromtheopticnerve[Fig.9].Macularinvolvementiscommon.Mild
vitreousandanteriorchamberinflammationisobservedinonethirdofthecases.
AmpigenouschoroiditismimicsplacoidlesionsofAPMPPEandcoalescedlesionsofGHPC.Persistent
placoidmaculopathyisaresistantformofserpiginouschoroidopathyandresemblesmacularGHPC,but
differsinitsclinicalcourseandeffectonvisualacuityasamajorityoftheeyesdevelopCNVM,[61,62]
resultingincentralvisionloss.Tento12%canhavemacularinvolvementalone.[63]Mildvitreousand
anteriorchamberinflammationisobservedinonethirdofthecases.Histopathologyrevealslymphocytic
infiltrationintheaffectedchoroidandpresenceoffibroglialtissuesurroundingtheBruchmembrane.
Note:
Toruleouttuberculousetiologyinpatientswithserpiginouslikechoroiditis.
PCRprovenviraletiologyhasalsobeenimplicated.[63]
Toxoplasmainfectionpresentinglikeserpiginouschoroiditishasalsobeenreported.[64]
MFC
Bilateralinvolvementispresentinapproximately6679%ofthepatients.[65]Opticdiscedema,rarely,
peripapillaryscarring,andprominentlinearchorioretinalstreaksmayalsobepresent.Patientmayalso
presentwithCMEandCNVM.[1]
PIC[53,66,67]
PICisaninflammatorymultifocalchorioretinopathyofunknownetiology.Itpresentswithanacute
bilaterallossofvision,photopsias,andscotomata.Theanteriorsegmentisquiet.Thevitreousisclear
withoutinflammatorycells.ThelackofvitreousinflammationisahallmarkofPICandthepresenceof
vitritisshouldsuggestadifferentdiagnosis.[53]
NotreatmentisadvisedforthemajorityofpatientswithoutCNVMorsubretinalfibrosis,whichusually
occurswithinthefirstyear.[66]PatientswithoutCNVMhaveexcellentvisualoutcomes.[1]
Note:
PICoccursinmyopicwomen.
AbsenceofvitritisHallmarkofPIC
PresenceofvitritisSuggestsadifferentialdiagnosis
SFU
ProgressivesubretinalfibrosiswithmultifocallesionsoftheRPEandchoroidcanbeseeninassociation
withPICandrecurrentMFC.Althoughsteroidsmaybenefitinitially,progressivefibroticsubretinal
lesionsleadstosevereandpermanentvisualloss.[67]Fibrosisispredominantlyatareasofprevious
inflammatorylesionsandaturbidSRFthatoverliesthelesionsisalsonoted.SFUisalsoseeninother
inflammatoryandnoninflammatoryconditions,[68]suchaslatestageofserpiginouschoroiditis,SLE
associatedCSR,andonchocerciasis.[1]Infliximabhasbeentriedwithgoodresults.[69]
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UncommonWDSinIndia
Birdshotchorioretinopathy(BCR)vitiliginouschoroiditis:Researchcriteriaforitsdiagnosishavebeen
formulated[70]andmorethan90%ofthepatientswithBCRareHLAA29positive.[71,72]Associated
nongranulomatousuveitisisseeninabout25%cases.Thetermbirdshotisgivenbecausethepatternof
lesionsinthefundusresemblestheshotgunscatterofabirdshot.Arecentlongitudinalcohortstudyhas
describedthebaselineclinicalcharacteristicsandhasconcludedthatlesionpigmentationmaybeamarker
ofdecreasedvisualfunctionthatisnotreflectedincentralvisualacuity.[73]
POHS:POHSisusuallyseeninendemicareasofHistoplasmacapsulatumandrarelyinothernon
endemicareas.[1,74]Itpresentsasymptomaticallyorwithcentralscotoma.H.capsulatumhasneverbeen
isolatedfromthechoroid.CNVMismanagedbyargonlaserphotocoagulationforextrafovealtype,while
kryptonlaserphotocoagulationisbeneficialforjuxtafovealtype.Dabiletal.[75]reportedasignificant
associationbetweentheHLADR15/HLADQ6haplotypeanddevelopmentofCNVMinPOHS
Acuteretinalpigmentepithelitis(ARPEKrill'sdisease)[76]:Itusuallypresentswithunilateralblurred
visionandmetamorphopsiainyoungadultswithoutsignificantprodromalflulikeillness.Roundmacular
lesions,hallmarkofthedisease,aremanifestedbytransientandsubtleRPEalterations.Discreteclustersof
small,hyperpigmented,darkgrayspotsattheRPElevelsurroundedbyayellowishwhitehaloorareaof
maculardepigmentationcanaffectvision.Graydotsandhalofadewithresolutionandbecomeclinically
undetectable.Vitritisisrare.VEPandERGarenormal,whileEOGmaybeabnormal.FFArulesout
CSR[51]andnotreatmentisneeded.
Treatmentofnoninfectiveposterioruveiticconditions
Systemicsteroidsarethemainstayoftherapyinnoninfectiveposterioruveitis.Invisionthreatening
lesions,suchasthosewithopticnerveheadormacular/fovealinvolvement,pulsetherapyofintravenous
methylprednisolone(IVMP)[77]1gdailyforthreeconsecutivedaysshouldbeadministeredonan
emergencybasisonlyundercareofaninternist,preferablyinplacewithanintensivecareunitbackup/set
up.Thisisfollowedbyoralprednisolone11.5mg/kg/day,preferablysinglemorningdose,inatapering
dosageschedule.
Oncethediagnosisofnoninfectiousuveitishasbeenconfirmedovertimeandinfectiouscauseshavebeen
completelyruledout,intravitrealinjectionsofsteroid,suchastriamcinoloneordexamethasone,arevery
usefuladjunctsincontrollingflareups.
Inpatientswithrepeatedrecurrences,intolerancetosystemicsteroidsorrecalcitranttotherapy,
immunosuppressivescanbeaddedwith/withoutsystemicsteroids.
Immunosuppressiveoptionsincludeantimetabolitessuchasazathioprine,methotrexate,and
mycophenolatemofetil,Tcellsuppressorssuchascyclosporineandtacrolimus,andcytotoxicagents
includingcyclophosphamideandchlorambucil.
Weusuallypreferazathioprine,[63,78]consideringthecostoftherapyandasithaslessersideeffects.Itis
giveninadoseof12mg/kg/dayinthreedivideddosesandtaperedmonthly.Athoroughknowledgeof
theusefulnessandthesideeffectsassociatedwiththevariousimmunosuppressivesisamustbefore
initiationofimmunosuppressives.
Thepatienthastobeexplainedtherisksandbenefitsassociatedwiththeuseofimmunosuppressivesand
theneedtomonitorthesideeffectswiththerespectivebloodtestsregularly.
Thedetailsregardingtheusageofimmunosuppressivesandotherdetailsaredescribedinotherappropriate
sections.Biologicalssuchasinfliximabforthetreatmentofrefractorynoninfectiveposterioruveitisand
severeSFU[69]anddaclizumabandtacrolimusforthetreatmentofBCR[7981]havebeenusedwith
favorableresponse.
Localdrugdeliverysuchasintravitrealtriamcinolone[82,83]forrefractoryCMEisalsoeffective.
InflammatoryCNVMhasbeensatisfactorilytreatedwithintravitrealtriamcinolone,bevacizumab[8486]
anecortaveacetateinserpiginouschoroiditisassociatedCNVM,[87]andsirolimusforMFCassociated
CNVM.[88]Fluocinoloneacetonide,alocaldrugdeliveryimplant,hasbeenfoundtobeusefulinalarge
multicentretrial.[8991]
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Roleofinterferonalpha[92]insevere,sightthreateningrefractoryuveitisisbeingconsidered,although
adverseeventslikeIFNalphaassociatedretinopathymaylimititsuse.
Masqueradesyndromesandotherdiseases[93,94]:Masqueradesyndromesarenonuveiticconditions
thatmimicandpresentlikeuveitis.Itisimportanttodifferentiateneoplasticdiseasesfromvariousother
posterioruveiticentities.
Tumorssuchaslymphomaandothersecondarymalignanciescanmimicviralretinitis.Lymphomahasto
beruledinnonresolvingoratypicalpresentationsofviralretinitisorintermediateuveitis.Theyare
especiallyseeninpatientswithHIVinfection.Inchildren,onehastobeawareofthepossibilityof
leukemiasandretinoblastoma,whileinadultsmalignantmelanomaandmetastasesareimportant
differentialdiagnoses.Knowledgeofnonuveiticentities,whichcanmimicposterioruveitis,isessential
beforeinitiatingtherapy.
Achoroidalmelanomaormetastasescanbemistakenforachoroidalabscess.USGBscanisvery
valuabletodifferentiatetheseentities.Choroidalmelanomashowsahightomoderatesurfacereflectivity
andvariablelowtomoderateinternalreflectivityonultrasound.Othertypicalfeaturesincludeacoustic
hollowingandchoroidalexcavation.Itisgenerallyassociatedwithashallowretinaldetachment,although
thismayalsobeseenoverlyingasubretinalabscess.
Choroidalmetastasesappearasmasswithhighsurfacereflectivityanduniformhightomoderateinternal
reflectivity.Acoustichallowingandchoroidalexcavationarenotpresent.Theyareusuallyassociatedwith
alargeretinaldetachment.
Vitreousandretinalbiopsyisveryusefulindiagnosticdilemma.
AIDSrelatedposterioruveitis[45]:Infectionssuchastoxoplasmosis,syphilis,andtuberculosiscanhave
atypicalocularmanifestationsinAIDS.Therearealsosomeuniqueentitiesthatgiveacluetothe
underlyingsystemicdisease.HIVretinopathy,raretumorssuchaslymphomas,andinfectionssuchas
cryptococcalandpneumocystischoroiditisareafewofthem.
Conclusions
Posterioruveiticentitieshaveverycharacteristicclinicalfeaturesanddiagnosisismainlyclinical.Itis
essentialtodifferentiateinfectiveandnoninfectiveconditionsastheirmanagementisdiametrically
opposite.Infectiveposterioruveiticneedstobemanagedwithspecificantiinfectivetherapyandsteroids.
Empiricaluseofsystemicsteroidsorimmunosuppressivesinallcasesofposterioruveitisshouldbe
absolutelyavoided.JudicialuseofancillarytestslikeFFA,ICG,USG,OCT,andelectrophysiologicaltests
ascomplimentarytoclinicalexaminationhelpsestablishtherightdiagnosis.Intraocularfluidtestingfor
PCRandantibodiesininfectiveconditionsisusefulindiagnosticdilemma.[9597]Itisessentialto
followupallpatientswithposterioruveitisevenaftertheresolutionoflesionsforcomplicationsrelatedto
thedisease,suchasCNVM,hemorrhage,orbreaks.Macularoropticdiscinvolvementcancause
irreversiblevisualimpairmentandhenceearlydiagnosisandappropriatemanagementisimportanttosave
vision.
Footnotes
SourceofSupport:Nil
ConflictofInterest:Nonedeclared.
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FiguresandTables
Table1
SUNworkinggroupclassificationofuveitisandtheprimarysiteofinflammation
Type
Primarysiteofinflammation
Includes
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5/25/2016
Anterior
Anteriorchamber
Iritis,iridocyclitis,anteriorcyclitis
Vitreous
Parsplanitis,posteriorcyclitis,hyalitis
Retina/choroid
Focal,multifocal,diffusechoroiditis,chorioretinitis,
uveitis
Intermediate
uveitis
Posterior
uveitis
Panuveitis
retinochoroiditis,retinitis,neuroretinitis
Anteriorchamber,vitreousand
retinaorchoroid
Table2
Basicmanagementapproachguidelines
1.Identifythecharacteristic/typicalfunduspicture
2.Confirmwithspecificinvestigations
3.Treattheprimarycausewithappropriateantiinfectiveagent
4.Usesystemicsteroidsasadditionalantiinflammatorytherapy
5.Avoidperiocular/intravitrealsteroids
6.Indiagnosticdilemmas,intraocularfluidevaluationforantibodiesorPCRforidentificationofgenome
Figure1
Funduspictureshowingatypicalpunchedoutmacularscarofahealedcongenitaltoxoplasmosis
Figure2
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Funduspictureshowingatypicalheadlightinthefogappearanceinapatientwithacquiredtoxoplasmosis
Table3
OtherantiToxoplasmicdrugs
st
nd
rd
1.Pyrimethamine(100mg1 day,75mg2 day,50mg3 day,followedby25mgoncedaily)+Sulfadiazine(4g
th
dailydivided6 hourly)for46weeks
2.Clindamycin[14](300mg6thhourlymaximumdose:1.8gm/day)for6weeks
3.Trimethoprim+sulphamethoxazolDStab160mg/800mgonetabtwicedailyfor6weeks
4.Spiramycin2g/dayintwodivideddoses
st
5.Azithromycinloadingdose1G1 day,followedby500mgoncedailyfor3weeks
6.Atovaquone[13]750mgevery6hfor46weeks
Table4
AntiToxoplasmatherapyinspecialsituations
Pregnancy ItrimesterSpiramycin+Sulfadiazine
IItrimester(>14weeks)Spiramycin+sulfadiazine+pyrimethamine+folinicacid
IIItrimesterSpiramycin+pyrimethamine+folinicacid
Newborn
Pyrimethamine,sulfadiazine,andfolinicacid
Mother
Reducesthelikelihoodofcongenitaltransmission
Figure3
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Funduspictureshowingatoxocaragranuloma
Figure4
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Funduspictureshowingatuberculoussubretinalabscess
Figure5
Funduspictureshowingtheclassicalcrackedmudappearanceinprogressiveouterretinalnecrosis
Figure6
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Funduspictureshowingatypicalpizzapieappearanceinapatientwithcytomegalovirusretinitis
Figure7
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Funduspictureshowingdiscedemaandmacularexudatescharacteristicofaneuroretinitis
Table5
Basicmanagementapproachtoacaseofnoninfectiveposterioruveitis
Identifytheclinicalentitybasedoncharacteristicclinicalfeature
Ruleoutinfectivecauses
Systemicsteroidsarethemainstayoftherapy
Useintravenousmethylprednisolonetherapyinvisionthreateninglesions
Useimmunosuppressives,withcaution,inrecalcitrantcases
Monitorsideeffectsoftreatment
Table6
Comparativecharacteristicsofclinicalpresentationsofwhitedotsyndrome[52]
Age
Sex
Laterality
APMPPE
Birdshot
PIC
MEWDS
MFC
GHPC
POHS
Young(2040)
Middleaged
Middleaged
Young(20
Myopic(20
Variable(30
Middleaged
Rarelychildren
(4060)
(myopes)
40)myopes
60)
60)
M=F
F>M
F>M
F>M
F>M
M>F
M=F
Bilateral,
Bilateral
Bilateral
Unilateral
Bilateral
asymmetric
Viralillness
Onset
Duration
Bilateral
Bilateral
asymmetric
asymmetric
+/
+/
Abrupt
Insidious
Abrupt
Abrupt
Insidious
Variable
Abrupt
Weeksmonths
Chronic
Weeks
Weeks
Chronic
Chronic
Chronic
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5/25/2016
months
months
Recurrence
Rare
Recurrent
Recurrent
Rare
Vitritis
Mild
Moderatewith
Absent
Mild
ERG/EOG
AbnormalEOG
HLA
B7,DR2
Recurrent
Recurrent
Rare
Moderate
Mild
Absent/mild
Normal
Abnormal
B7
HLADR2
discedema,
andanterior
CME
uveitis
AbnormalERG
A29
Abnormal
Abnormal
Abnormal
ERG
ERG
HLAB7
Fundus
active
Multifocal,flat
Multiple
Multiple,
Multiple
Multiple
Macular,
Peripapillary
graywhiteplacoid
depigmented
discrete,flat,
small(100
yellowor
peripapillaryor
atrophy,
lesionsprimarily
yellowwhite
yellow,
200),
graylesions
ampigenous
atrophic
posteriorpoleat
patches
roundlesion
round,
atthelevel
irregular,gray
chorioretinal
thelevelofRPE
scattered
(50300
slightly
ofchoroid
whiteorcream
lesions,CNV,
andchorio
throughout
microns)at
indistinct,
andRPE.
yellow
punchedout
capillaries
fundusinthe
thelevelof
white/yellow
Mid
subretinal
yellowlesions
postequatorial
RPEand
whitespots
periphery
infiltratesatthe
Linearstreak
region.These
inner
distributed
(50100)
levelofthe
smidperiphery
lesionsradiate
choroid.
overposterior
choriocapillaries
fromoptic
Concentrated
fundus,
andRPEsnake
nerveand
atposterior
especiallyat
likepattern
followlarger
pole
perifoveal
choroidal
and
vessels
peripapillary
regionsatthe
levelofRPE
Fundus
RPEclumpingand
Lesionshavea
Healsrarely
Punchedout
Healsfrom
healed
hyperpigmentation hyperpigmented
byscarring
atrophic
centertowards
scarsthat
periphery
edgebutare
Pathogenesis
DTH
frequently
develop
hypopigmented
pigmentation
inthecenter
overtime
Autoimmune
?Hormonal
Scars
Idiopathic/?
infective
WksWeeks,DTHDelayedtypeofhypersensitivity
Table7
ComparativecharacteristicsofFFAandICGfeaturesofwhitedotsyndrome[52]
FFA
active
APMPPE
Birdshot
PIC
MEWDS
MFC
GHPC
Early
Mild
Early
Earlypatchy
Early
Early
punctate
hypofluorescence
hypofluorescence
andlate
andlate
hypofluorescence hyperfluorescence hypofluorescence

andlate
andstainingin
andlate
hyperfluorescence
hyperfluorescence
latephase
hyperfluorescence
withlatedeep
hyperfluorescence hyperfluorescence.
stainingofRPE
Choroidalvessels
andperipapillary
areeasilyseen
area.Leakage
fromopticdisk
andretinal
capillaries.Early
25/28
5/25/2016
fluorescence
wreathlike
pattern.Choroidal
background
fluorescence
betweenlesionsis
normal
FFA
Windowdefects
Windowdefects
Windowdefects
Windowdefects
inactive
Windowdefects.
Windowdefects
Early
hyperfluorescence
andlatestaining
ICG
Markedchoroidal
active
hyperfluorescence
CNVMreveals
Multiple
Hypofluorescent
hyperfluorescence
hypofluorescent
CNVMreveals
hyperfluorescence
inbothearlyand
spotsinthe
latephase.Large
posteriorpoleand
choroidalvessels
hyperfluorescence
seen
aroundoptic
nervehead,
especiallyin
patientswith
enlargedblind
spots
ICG
Choroidal
active
hypofluorescence
Hypofluorescent
Hypofluorescent.
spotspersistuntil
patientrecovers
ERG/VEP
unilateral
negativeERG
markedlyreduced
awaveandearly
receptorpotential
amplitudes
suggesting
primary
involvementof
RPE
Visual
Enlargementof
fields
blindspotNo
correlationof
fielddefectswith
lesions
Table8
Cluetodiagnosisofwhitedotsyndrome
Characteristicsoflesions Consider
Subtlelesions
MEWDS
Prominentlesions
MFC
Placoidlesions
APMPPEifdiscreteandiftheyarecoalesced,considerampigenousorserpiginouschoroiditis
Discretelesions
MEWDS,DUSN,MFC,Birdshotchorioretinopathy
Figure8
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Funduspictureshowingplacoidlesionsofacuteposteriormultifocalplacoidpigmentepitheliopathy
Figure9
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Funduspictureshowingactivegeographichelicoidperipapillarychoroidopathy
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Current approach in the diagnosis and management of posterior uveitis

Current approach in the diagnosis and management of posterior uveitis

Current approach in the diagnosis and management of posterior uveitis

Current approach in the diagnosis and management of posterior uveitis

Current approach in the diagnosis and management of posterior uveitis

Current approach in the diagnosis and management of posterior uveitis

Current approach in the diagnosis and management of posterior uveitis

Current approach in the diagnosis and management of posterior uveitis

Current approach in the diagnosis and management of posterior uveitis

Current approach in the diagnosis and management of posterior uveitis

Current approach in the diagnosis and management of posterior uveitis

Current approach in the diagnosis and management of posterior uveitis

Current approach in the diagnosis and management of posterior uveitis

Current approach in the diagnosis and management of posterior uveitis

Current approach in the diagnosis and management of posterior uveitis

Current approach in the diagnosis and management of posterior uveitis

Current approach in the diagnosis and management of posterior uveitis

Current approach in the diagnosis and management of posterior uveitis

Current approach in the diagnosis and management of posterior uveitis

Current approach in the diagnosis and management of posterior uveitis

Current approach in the diagnosis and management of posterior uveitis

Current approach in the diagnosis and management of posterior uveitis

Current approach in the diagnosis and management of posterior uveitis

Current approach in the diagnosis and management of posterior uveitis

Current approach in the diagnosis and management of posterior uveitis

hypofluorescence hyperfluorescence hypofluorescence

Current approach in the diagnosis and management of posterior uveitis

Current approach in the diagnosis and management of posterior uveitis

Current approach in the diagnosis and management of posterior uveitis

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