Posner schlossmann syndrome

A presentation on complete clinical profile of Posner schlossmann syndrome.

1. POSNER -SCHLOSSMAN
SYNDROME
Dr Saurabh Kushwaha
Resident Ophthalmology

2. SCOPE
Introduction
Epidemiology
Pathogenesis
Etiology
Associations
Symptoms
Signs
Ancillary testing
Differential Diagnosis
Medical therapy
Surgical therapy
Prognosis

3. POSNER-SCHLOSSMAN SYNDROME
Posner&Schlossman1
st
reportedacaseseries
andcoinedthetermGlaucomatocycliticcrisisin1948
Thesepatientssufferedfromrecurrentunilateral
attacksofocularhypertensionandsharedthefollowing:
Unilateral
Recurrent
Milddiscomfortorblurringofvision
IncreasedIOPwithopenangles
MildanteriorchamberreactionorfinewhiteKPs
Criseslastingfromseveralhourstoweeks
NormalIOP&nosignsofuveitisbetweenattacks
Normalvisualfieldsandopticdiscs

4. EPIDEMIOLOGY
Typically affects between the age of 20-50 yrs
Males and females are equally affected
Generally, only one eye is affected at one time
Unilateral >> bilateral

5. PATHOGENESIS
Theexactpathogenesisisnotknown
Onelectronmicroscopy,mononuclearcellswere
seenintercalatedinthetrabecularmeshworkwithlong
pseudopods,possiblyimpedingtheoutflowofaqueous.
Theoriginofthesemononuclearcellsisstillunknown
Themechanismisspeculatedtobeacute
trabeculitisandthereisevidencethatinfection,
possiblyCMVorH.pylori,mayplayarole
Prostaglandinsarethoughttoplayaroleand
particularlyPGE2,wheretheaqueouslevelspositively
correlatewithIOP

6. PATHOGENESIS
(a)Electron microscopy of trabecularmeshwork in a PSS patient
(b)Higher magnification view

7. ETIOLOGY
Autonomicdysregulation
Posner&Schlossman1
st
postulateditasaresultofautonomic
dysregulation
Allergy
Theterm“glaucomaallergicum”wasfirstcoinedbyKraupain
1935whenhedescribed4casesofrecurrentunilateral
inflammationandglaucoma.
Variationofdevelopmentalglaucoma
Observationofbilateralangleabnormalitiesongonioscopy
duringattackssuchasabnormalirisprocesses,anteriorized
Schwalbe’sline,anda“greymembrane”coveringthetrabecular
meshwork
Vascularendothelialdysfunction
Ciliaryvascularabnormalities,suchasfocalirisischemiaand
leakagefromiris/ciliarybloodvesselsonangiogramduringattacks.
Thisvascularendothelialabnormalitymayexplainthe
propensityforglaucomatousopticnervedamageinthelongrun.

8. ETIOLOGY
Autoimmune/HLA-Bw54
Elevatedprostaglandinlevels,particularlyPGE2,havebeen
foundintheaqueousduringattacksandlevelspositivelycorrelate
withIOP.
PresenceofHLA-Bw54haplotypeimplicatedinsomecases.
Infectious
H.pylori
•AssociationbetweenH.pyloriantibodiesandanterioruveitis
•Associationbetweenanti-H.pyloriserumIgGandPSS.
HSV/VZV
•HSVPCRpositivityinaqueoussamplesinacuteattacks
•Furthermore,Acyclovirisfoundtobeineffectiveintreatment
CMV
•ActiveCMV-antibodyproductionoccursinasignificant
numberofPSSpatientsandtherehavebeensomereportsof
responsetoprophylacticbenefitofanti-viraltherapyspecificto
CMVsuchascidofovir,foscarnetandvalganciclovir.

9. ASSOCIATIONS
POAG:Uptoa45%concomitancebetweenPSS
andPOAGhasbeenreported
Non-arteriticischemicopticneuropathy(NAION):
presumedmechanismisdecreasedopticdisc
perfusionduetoanacuteriseinIOP

10. SYMPTOMS
Unilateralblurringofvisionwithmildeyediscomfort
orpain,haloesandsometimespain
Historyofpriorepisodesofblurredvisionandeye
discomfortthatmaybesuggestiveofpriorattacks;
theseepisodesmaybemonthstoyearsapartandlast
fromseveralhourstoweeks
Accountsfor0.4%ofalluveitis
Thecourseisvariable:somepatientsmayonly
experience1or2episodesintheirlifetime,whereas
somehavemanyrecurrences
Frequencyofattacksdecreaseswithincreasing
age

11. SIGNS
Vision:mayvaryfrom20/20tohandmotionorPL
Conjunctiva:Theeyeisquietwithnoorminimal
ciliaryflush
Cornea:IftheIOPisabove40mmHg,thecornea
canbecomeedematous
FineKP’scanappearafter2-3daysofinflammation
andresolverapidly
AC:deepwithamildiritiswithoutsignificantcellor
flare
Openangleongonioscopyisanimportantcriteria
fordiagnosis
Iris:Segmentalischemiamaybepresent

12. SIGNS
Posteriorsynechiaemaybepresent
Pupil:oftenslightlydilatedorsluggish
Opticnervecandemonstrateglaucomatous
cuppingduringanacuteattack.Reversiblecuppinghas
beendescribed
IOP:Itisgenerallyelevatedandintherangeof40-
60mmHg.Itisrelatedtothenumberofdaysofuveitis
andnottotheseverityofuveitis.Itisimportanttonote
thattheIOPiselevatedoutofproportiontotheamount
ofanteriorchamberinflammation

13. Small, white, discrete keratic precipitates

14. ANCILLARY TESTING
Irisangiogramsperformedduringacuteattacks
demonstratesegmentalirisischemia,vascular
congestionandvesselleakage
Opticnervetopographyandflowmetry
demonstratetransientdifferencesinmorphologyand
bloodflowduringattackscomparedtobefore/after
attacks
CupvolumeandareameasuredbyHeidelberg
RetinalTomographyincreaseduringanattack,but
pre-andpost-attackmeasurementsarecomparable

15. ANCILLARY TESTING
Flowmetrymayalsodemonstratedecreasedoptic
nerveperfusionduringanattack,particularlyatthe
peripapillarytemporalandnasalsectors,aswellasat
theleveloftheneuroretinalrim
Visualfieldsperformedduringanattackmay
demonstratenon-specificchanges,butingeneral,they
remainnormalfollowinganattack
LaboratorytestingforHSV,VZVorCMVtitresmay
beorderedincaseofstrongsuspicion
ViralPCRstudiesforthesamevirusesmayalsobe
performedonanaqueoustapsample

16. Iris angiogram demonstrating focal iris ischemia during
an attack

17. (a)Initial visual field change;
(b)Visual field progression 2 years later

18. DIFFERENTIAL DIAGNOSIS

19. MEDICAL THERAPY
Self-limitedocularhypertensionthatresolves
spontaneouslyregardlessoftreatment.
Initialtreatment-controllingIOPanddecreasing
inflammation.
Typicalfirst-linetherapeuticsincludetopicalbeta-
blockerssuchastimolol,alpha-agonistssuchas
brimonidine,andcarbonicanhydraseinhibitorssuchas
dorzolamide.
ProstaglandinanalogsmayalsobeusedforIOP
controlalthoughthereisatheoreticalriskof
exacerbatinginflammation.Oralcarbonicanhydrase
inhibitorsaresometimesusedacutelytolowertheIOP
quickly.
Topicalsteroidstocontrolactiveinflammation.

20. MEDICAL THERAPY
TopicalNSAIDsmayalsobeused.
OralNSAIDssuchasindomethacinmayalsobe
usedtoavoidapossiblesteroid-inducedglaucomaand
fortheiranti-prostaglandinproperties,aselevated
prostaglandinlevelsintheaqueoushavebeen
associatedwithattacks.
Benefitofantiviraltreatmentisunclear.
Mydriaticandcycloplegicagentsarenotcommonly
neededasciliarymusclespasmisuncommonand
synechiaerarelyform
Mioticsandmydriaticagentsarerarelyused.
Inparticular,pilocarpineshouldbeavoidedasthisis
thoughttoexacerbateapossibletrabeculitis.

21. SURGICAL THERAPY
WheretheIOPcannotbecontrolledusingmaximal
medicaltherapy,wheresignsofglaucomatousoptic
nervedamageorvisualfieldchangesappear.
Trabeculectomywithmitomycin-C
Filteringsurgeryimprovesaqueousoutflowand
alsoincreasestheegressofinflammatorycells.

22. PROGNOSIS
“Benign”disease
Mostlyrecoverwithoutlong-termsequelae
Afewmayshowlongtermglaucomatouschangesin
theopticnerveandonvisualfieldtestingessentially
becomingasecondaryglaucoma.
ItisthetotaldurationofelevatedIOP,notthe
frequencyofattacks,thatcontributestothedamage-
candidatesforsurgicaltherapy

23. THANK YOU