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Ey (9

Posterior Scleritis: a Clinical and Histologica Survey

C. M. CALHORPE, P. G WAON, A. C. E. McCARNEYLondon

SummaryThe clinical course of 4 patients with posterior scleritis is reviewed. Thogh clincal presentation varied widel, 3% of the patients presente with a visual acuity of 6/8 or less. Becausethe osteio scleritis was not always associated with pain or with anterior scletis, the diagnosis was often not consiered when the patt as st see. e most common nings inthe fundus were disc sweUng, retinal detachment, and macular oedema and the most useful in

vestiation was B scan ultrasound. No comm aetioy as fund, althouh 60% had a sysemic disorder which was accompanied by a vasculitis. Those who were diagnosed and treatedwith the minimum delay had te most satisaty iua utcom. Howeer, tere aears tobe a group of patients with no underlying systemic disease who fail to respond to intensivetherapy, and lose vision. A new sub-group of West Inians with the disease is descrie. Theisopathoogy of cases conmed the presence of cleral vasclitis of the vessels in andaround the sclera in al the secimens. Other signcat ndgs iue iammaty sigand focal loss of pigment epithelium togehe with choroidal vascular closur. This could accont fo the oescein angiogaphic ndings.

Poterior ceritis i an infammator diorderinvoving te scera and epicera of te pot-erior egment of te ee. It ma beassociated wit anterior ceritis, and mapread to invove uce and oter orbitatiue, or to invove coroid and retinaJ.e cnica presentation i ver variabe a itdepend on te exact ite of te inama-tion. i make te diagnoi difficut.Later in te coure of te diseae, compica-tion ma furter obscure te primar condi-

tion. Ear diagnois and initiation ofappropriate terap are eentia becauedea in treatment ineviab ead to omeo of vision. e preentation and cinicacoure of a serie of 47 patient aredecribed

Maeras an Meho

A retropective anai a been made of 64ees of 47 patient wit posterior sceritis.Fort one of tee were exained in te

ceriti Cinic at Moorfied Ee Hospita

and ix patient came to our notice trougte Patoog Department were teirenuceation pecimen were examined.

Relts

Sex, Race AgeOf te 47 patient tudied, 28 were femaeand 19 were mae. Fort two were caucaianand te remaining 5 were of Wet Indian ori-gin eir ages at preentation ranged from12 to 87 ear. e mean age of a patient

wa 49 ear. e mean age of te caucaianwa 57 ear tat of te West Indian wa 19ears. e mean age of te femae, 54 ears,wa iger tan tat of te maes, 42 ear.However, te average age of te mae wasweigted b 4 oung Wet Indian patients.

Symptomse mptoms on preentation are given inabe I Fort tree patients 91 %) compained of pain of some degree. Of tee,

on 5 decribed evere, intoerabe pain,

Corrpod o o, Moord Ey Hop, Cy od, Lodo EC

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which preented sleep. Three patients notedan ncrease in thei pai on ocular move-en. Six escribe radiation of pain to su-rounding structures, uh a the brow an theJaw.

T I Sos on psnion

So o % painpans

ain 43 91.48oor Vison 38 80.85e Eyes 36 76.59i welling 9 19.1hotophobia 4 851

Diopia 4 8.51oint in 3 6.38in Ulcers 2 4.25

T II Inial si o scls

o pains (%) Inial si o sclitis

239

1

(48.94)(19.15)(31.91)

Anterior + osteriorosteriorAnterior

III isal aci a sntaion o posiosclis

isal Aci

NPL-CF.6/60-6/366/24-/186/6/9

> 6/9

o s

221113

4

b x nsais

o ofs

njuctivl 33 eois

swei 2 18.5paire oclar 12 18.5

ettoi 9 14.0G eess 6 9.37

Thirty six ptets (76%), ote re eeintially. Tw ther paient develoed edeye laer i te corse of the diseas Othese, 3 d anteror scleritis and 19 aaterior ueitis. Eiheen patent had botanteror uveitis an anterior leritis. Opatient ha nether

Signsite of he iamaon. Fe o te pre-sentig ith oseor disease alone id ntdeelo atero sease durig te olloweriod (Table I). O thse preseti whaterior disease alone 1/15 develpe pst-erior isese at a ean itervl o 1.75 ears.Range imeiael to 7 ear). he ielin the reainn patient was unertai.Twelve ptients esente wth biatealscleritis, 0 wth blaeral osteror scleitis.At the en of te olowu erd, atiens d deeled bilateral scleritis: 1of tese d bilatera osteror sceris.

Visual acy A rou lo vsacuity (6/3 o less wa evidet i 5 ees. (ble II.

Se exteral manifestatins are lise iTable IV. he aximu degree of proptosisnoted s 5m. n 3 ees the degree roptosi as nt easred. ree atietshad impaired ocular moveent witot associated proptosis. No ocular nere lwas ound.

Uvesf 6 eyes, 5 (70% had uvetis. nterioruveit was esent in 35 ee, osteiuveitis n 33 eyes, an both anterio nd oerio ueitis n 23 ees.

haown h aro hbShallowing was ote n 9 ees, tho ts not atl eured. ee rnchrodal detahent as noed in fr.

GlucmEv n pesn. Oe had osue n cl e anterio uvei s, e scle ng ls. Tw btteio srtis i e s ndterior s.

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POSTERIOR SLERITIS A LINIAL AND HISTOLOGIAL SURVEY

Tabe V Fundal signs

Sign

Optic nerve swelling

Serus retinal detachmentMacular oedemaSubretinal massRing chrial

detachmentIntra-retinal depsitsChrial flsPigment epithelial detachmentSubretinal discluratin

Fundalsigns (Table V).h os coon sns w oc nv

swlln, (F. 1), sous dachn o hna (F. 2), and acua oda. Choo-da ods w nod a h oso o n 7ys. n addon o hos wh acuaoda, a uh 5 ys w nod o havaas o ha na oa, nado cncay ovous suna asss R-na vascus was no nod cncay n anyo hs ans. Howv, was d

osa on oscn anoahy non an. Pn ha vaonsw sad a h oso o. na-na ca o wh sons sn hadxudas w scd n 8 ys. hy wno od n aon o suna asss,o na oa. hy occ oh a hoso o and n he ha na.Fo ans ha sca, a, -ha, oan, yow o a on,a h v o h, n-

ad o ovous sua asss. cnoahy was a wc was co-on o h ou o youn, al, s n-an as no o conaon ocncal sns coud assocad wh anyaca oun o ans, as o a,sx o assocad dsas. Eys coonyha o han on na ansaon. h 17 wh acua oda, 11 had ocnoay, a a sna ass naddon. 29 ys wh oc

noahy, svn aso a na ach-n. Exc o on an, a ho wh cooa acn aso a sos -na dachn.

No. ofeyes eyes

2 4531

25 3061 2656 118 1406

8 1250 1035 814 625

Ocular investigatinFluoscn anoahy was cad ou on17 ans. Alhouh uvs and vousha on ndd h vw ndsnc, hnvsaon was ound o usul n dsnshn cnal sous noahy and s-ous na dachn assocad wh os-o scs (F 3)8scan uasonoay was cad ou on20 ans. hs s has co usu n -cn yas whn ovd chus hav

aowd h a donsaon o hck-nd oso ocua coas. h chaacs-c sns a usad n Fu 4. hs n-cud hcknn o h sca and chood,and an cho on wn h os-o od o h scla and non's caps, ca oa oa.

sd axa ooahy was ca-d o on 4 ans. Howv, was h-u n an a anoss n ony on.

Assciated Ssteic Diseasexn ans (34%) w on o havan assocad sysc condon (a V).h os coon was hao ahs,whch was sn n 2 ans. Evn a-ns had o ha on assocad cond-on. a o hos sd, 2 ans hadyo anos, an a h 3 ha as-c aa cl anos. on o hs a-ns had ov cnc sas acnhs oans. n on an who was ss-

c cncay o havn nsGanuoaoss sval oss o snus,n, ad skn ssu va a onsccas. h ans dd dun h ol

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0 C M. CALTHORPE ET AL

low-up eriod: one of myocara infarctonassocate th oarets, the secon orenal failure, also associaed wih polyareriis, and he hird of a perforaed pepic

ulcer secondary o seroid herapy. They alldied wiin 4 years o e onse o eirscleral disease.

Tale Associated systemic diseaseDisease Number of

patients

Rheumatoid Arthritis 1Multiple Systemic Auto-immne 5

Disease

Systemic Arteritis Be"hets Disease 1Myasteia Gravis 1Gout 1Hasimotos Thyroiditis 1Idiopathic hrombocytopenic 1

PurpuraWegeers Graulomatosis

Tale Systemic Investigation

Haeatoogica screeImmue screeSerum uric acidV.DRL., .PHA.Chest X Ray

F Swll ee i et who e-s wh eo ses, eengvisin. The diagnsis was cnirmed ultrasun

Ssmc nsnA aens nerent hose inves tgatonsisted in abe VII Frher ess were carriedou if indicaed on clinica assessmen

Laboraory esing, however, did no revealany sysemic disease unsuspeced aer isory aking and clinical examinaion

oow-upTe mean ollow-up perio was yearsTe range was mons o years A ollowup o years or more was obained in% o paiens Poserior scleriis is oen arecurren or cronic condiion (Tabe VIII).Tweny paiens suered recurren episodesO ese, remained quiescen beweenepisodes only on mainenance erapy

Tale Activity of te disease

Number f patients Number of episodes

18963119

1378Never became quiescet

F 2. Ss ehe o h t he s-e le The oss was conme byltsn CT san Tee was n nteor .

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POSTERIOR SLERITIS A LINIAL AND HISTOLOGIAL SURVEY 271

eatmentn h cs Cnc a Moods Ey Hospial all pains iniiay civ a nonsoal aniinaaoy (N.S.A.. agnxcp hos wih anio ncosingsciis hos quiing syseic soid oh oh y a pvious pisod o aad sysic condiion. soid isqud Pdnsoon 800 g s pscid day o 3 days wih a apdy ducing dos ov h olowing wks. Thospans no sponding o hs gi cvan navnous puls o wn 500 g and ga o hypdnisoon. Cyclophosphad 500 g s gvn navnousy inaddon h pan s aady akng sysic soid o h a hgh vls oiun copxs. Howv no apains n hs ss w ad accodng

Tab IX Mode/draton of treatment

% Paiensean duaeamen(n

N.S.A.!.

149010

o hs poocol Twny nin pans wanagd nay o vayng pods andsix anagd whoy a oh cns. Thod o an o h ni ss opans ad s duaon a shown n TaX.

O h six pains who spondd oN.S.A.. agns non had an assocad sysc dsas and h scs povdwihn wks o sang an. Asvnh pan was ony gvn N..A..agns. H nv sd was no gvn anyoh an an h y was nucado a dagnosis was ad. hs painhad haod ahs and cos.

O hos ad wh sysic soid wiho wihou ..A.. agns (9 pains 5ad o s. h disas and qusan n on pan on annanc hapy

Sytemc Steod+/-N.S..'

61703

Sytemc Sterod+othermmno

ppeve aent

234024

Fig. a,b. oreen anora of an area of lert wh overln retnal detahment, howhorodal houoreee (a), and h uoreee a laer frme (b) due o leke f uresento the bnerorenal ae.

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272 C. M. CALTHORPE ET AL.

alon. O s oup 36 ad an assocadsysc dsa. Elvn pans qud cyclopospad o azaopn naddon o sysc sod In 7 o 11

pns s was ans y plsapy. In sp o s al o slyou p cn o s pans a anassocad sysc dsas On panwos nal acn al o sla ons o sysc sod and cyclopospa apy, unwn sucalxploaon. A ossly cnd scla wason, conscn a vox vn. A wnowo scla was ov, lvn conscon. na aly aac pos

opavly and as and so o olowp o o 1 ons.Foy pans w ad on sysc

sod o a an pod o 17 ons andlvn qud azaopn o cyclopospad n addon T dus usdaddd consdably o obdy. Sxnpans (34%) dvlopd on o o sysc coplcaons o an. Ts alsd n Tal X. On pan dd o a poad ppc ulc nducd by ssc

sod, wc was qud o o uaod as an sclal sas

isual utcmePans a dvdd no 4 oups accodno vsual ouco (Tal XI) Ov50% o ys ad a vsual acuy o 6/9 o a nd o ollowup pod. an nval o ons o sypos oakn danoss s so n os wa vsual ouco. lay n nsu

Table X Systemic compications o reatment

Compication

Hig bl sugrentl cngesPeptic ulcertinGstritisAneiypty

HypertensinObesitySteri cne

Nmer opatients

5

111

n adqua an acd vsualoco T os coon caus bn c o polond nlaaon and nv lay daon assocad w opc

nuopay. Foun ys ad a vsual

F B-scan trason in eyes wih pserior

sceriis Seros eevation o the retina, thickeneposterir ocar coas, an an echo-ree region etween the posterior orer o the scera a Te'scapse (arrows)

Table XI Visa acity reate to mean interva toianosis

Nmer o eyes isa Otcome Mean interva(% to iagnosis

5156)

8 150)9 106)

1 188)

6/96/4

6/24-6/12CF-6/36

NPH

6

65800

108

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POSTERIOR SCeRTIS, CINIC ND HISTOOGIC SURVEY 273

Tabl XII iua acuity bfor ad aftr tratmt

iua Acuity Before ratmtNumbr of y (%

Aftr ratmtNumbr of y (%

N.P.L.-M.F - 6/366/24 - 6/126/9 - 6/4

7 0.94)28 43757 26562 875

421889 14068 25

335156

acuity ranging from NPL to at eend of te followup period (7 eyes were intis state wen first seen) (Tabe XII) Teean interval from onset of symptoms to teestablisment of te diagnosis and institution

of treatment was longer in tis group (TableXII)

West nian PatientsSince tis group of patents was considerablyyounger teir features will be consideredseparately in addition to being incuded inte main group Seven eyes of patients pre-sented wit posterior scleritis Four of te patients were male Teir mean age was 19years Te mean interval to diagnosis was13 monts Te visual acuity before andafter treatment is sown in Table XIII Allad anterior scleritis and all ad evidence ofoptic nuropaty None ad an associatedsystemic disease Tree patients sufferedrecurrent episodes of scleritis Four patientswere treated wit systemic steroid and onewit a NSAI agent alone Toug temean interval to treament was long tisgroup responded rapidly to treatment Telongest period of treatment required was 4onts Five of 7 eyes ad a visual acuity of6/12 or better at te end of te followupperiod Permanent loss of vision was due tooptic atropy following prolonged opticnerve swelling

ncleatinEigt eyes were enucleated or variousreasons (Table XIV) Of tese te diagnosiswas made only on istoogical examination insix Six of tese patients were female Four

ad multiple systemic autoimmune diseaseTe remaining paients were wel Te meaninterval from onset of te condition to teestablisment of te diagnosis was 3

Tabl XIII iua acuity of Wt Indian patientsEy

1234567

iua Acuity

for tratmt

CFNPLCF6/606/606/186/8

Aftr tratmt

6/2NPL6/66/606/66/66/9

Tabl XIV

Numbr of y Rao for nucatio

Painful Blin Eye2 Suspecte Malignancy

Phthisis Bulbi

monts Seven patients were treated witsystemic steroid owever 6 of tese werenot treated according to te protocol in use atpresent None responded to treatment andtey suffered prolonged periods of inlama-

tion (mean 42 monts) prior to teir enucleation

Patlgaterial was availabl for examination inseven cases (Table XV) Two of tese (cases1 and 3) were scleral biopsies Tese eyeswere examined in celloidin tus immunois-tocemistry ws not possible Te dominantcell types found were cronic inlamatorycells Tese were lympocytes and plasma

cells (7 cases) macropages (4 cases) andgiant cells (2 cases) Acute inlammatorycells were seen in 3 cases ut were few innumber Active scleral vasculitis was noted in

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274 C M. CALTHPE ET AL

Table XV

ase

1

234567

Systemic Disease

Rh.A. ,ClitisM-A-ID*M-.A-I.D.

Scea vascuitis

+

++++++

* = Multi system atimmune disease

6 cae Previou vacuii diagnoed byonion kin ickening' wa preen inanoer (cae 1) Smudging and fragmenation of cera coagen wih o of poariaion wa een in 3 cae Ony 1 (cae 2) hadnecroiing cerii

Te choroid wa invoved in a enuceaedeye and wa ickened wi infammaoryinfae (Fig a) Choda vac wanoted in 4 cae and onion kin tickeningw occuion of vee in one of ee (Figa) evidence of choda infacn afound

In 2 cae e reina pigmen epiheium(RPE) wa aben focay wih infammaionn he ondn Fig 5b) heR\en wee n cnny w ndeying chooid and c Rena vacacuffng wa noed n 4 cae F 6 Howev na vac a n fnd Sneuorena exudae wa fond n 2 caeand wa rad aa of cea vac

DiscussinPoerior cerii a many feaure wichare ared wi other deae Tu i habeen confu d wih ange coure gaucomaopic neurii cenra erou retina deachen pigmen epihia deachmen rhegmaogenou reina deachmen primaryor econdary chooida umour orbainfammaory diae or ma he uvea effuion yndrome pacoid pigmenepiheiopathy acceaed hypernionand Harada yndrome Ahough heepaien pren eary becaue heirymptom ar dficu o ignore he dagnoi i ofen deayd Thi i borne ou by

i tudy in wich the mean inerva frompeentaton o dagnoi wa over 7 monhThe diagnoi of poerior cerii i aided bya hig eve of upicion and confirmed byuraonography Thu poerior cerii iamo cerainy a more common condiionan a been previouy upoed

Thi erie of 47 paien i imiar in ageand ex diribuion o hoe previouydecribed excep for ha of Singh et eigh of woe 9 cae were mae

The mot common preening ympom ofi erie were pain and o of viion Toewih poerior dieae aone ad eiter nopain or pain of a mid degree The pain mayamo cerainy be acribed o aneriorcerii wic wa preen in 81% ofpaien Oher poibe caue of painincude Tenonii reching of nerve paing rough Tenon' capue and cera; andopic neurii aociaed wih infammaion ofe nerve hea

A poor correced viua acuiy of /18 ore wa noed in 73% of eye a peenaion Ti conrated wi te near normaviua acuiy of Sing' paien

Anerior cerii wa preen or had occurred in 81% of our paien Evidence of poerior deae houd erefore be oughhroughou he coure of any epiode ofanerior ceriti bu equay e abence ofanerir infammaion oud no precudehe poibiiy of poeior cerii Uveiiwa preen in 69% of eye Thi i a conideraby iger figure han ha een in aneriorcrii aone he preence of uveii wihanerior ceri herefore i a uefu indicaor of poerior dieae

Ony one paien deveoped gaucoma a adrec reu of poerior egmn dieaeand preened wih ange coure gaucomaTi mode of preenaion ha beendocumened in e pat The remaining 11paen wih gaucoma ad uveii or ceriinvoving e drainage area or bo Thiagree wih he obervaion of Wihemu e ha damage o e rabecuar meworby uveii and overying corneocera infammain i he m common caue of raiedinra ocuar preure in cerii

A wde variey of funda gn een inpoerior c rii4.5,1516 ee incude opi

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POSTERIOR SCLERIS, A CLNICAL AND HSTOLOGICAL SURVEY 275

Fig.5a,b a icened iammed cooid, i obieaion o a ma ee o 180 H+Eeoidinb cie cea and cooida inammaion ao o bu io o RPE ao iue e o diuion o x 80 +E eoidin ciecue and iniaion b inammaoyce

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276 M ATRE ET A.

Fig. Mared cuing a retina vesse (arrw) inactive chrnic inammatin invving the retinate thicened chrida vesse wa (arrw) and

ca disturbance the RP with incntinence igment (arrw) x 180 H+ eidin

disc swelling, retinal detachment, macularoedema, subretinal mass, choroidal detachent, choroidal olds and pigment epithelialdetachment This presentation should not beoverlooked in children These patients maybe pain ree and without evidence o anteriorscleritis or uveitis The diagnosis o posteriorscleritis should be consideed, thereore, inany atient ith a undal lesion, in whom airm diagnosis has not been made partrom th association o optic disc swellingith the group o young West Indians noother maniestation could be associated withan particular subgroup o this series basedon age, sex, or associated systemic disease

Ultrasonography is he s use invigation since it provides diagnosic sign operir sceriis and see Fig. noru

nately the angiographic eatures whist help re nt bste ptnmic posterior scleritis For exampe, the darkchoroid, shwn in Fig 3 is seen in other conditions and in posterior scleritis, may be dueto coroida vscuar occusion, andor msking o choridal uorescence by thickened,inamed RPE, since both are seen on histological examination

The natural history o posterior scleritis isnot benign Six o the 8 enucleated specimenswere undiagnosed cases o posterior scleritisFurthermore, those cases whose diagnosisand treatment were delayed longest had avery poor visual outcome

When the interval rom onset o posteriorscleritis to initiation o treatment was short,the visual outcome was most avourableEven so 3 patients who were in excellenthealth and were treated energetically at presentation lost vision permanently partrom their poor therapeutic response, initially they were indistinguishable roothers

Though the patients in this series were nottreated according to one protocol, at leas85% o patients were treated on longtersystemic steroid or a mean period o 21months The associated morbidity was considerable Pulsed intravenous steroid another immunosuppressive therapy wereintroduced to minimise the side eects osteroids

Sixteen patients (34%) had an associatesystemic disease This is less than thatreported with anterior scleritis It was thesepatients who more oen required systemisteroid with additional immunosuppressivtherapy and needed longer periods o treatment

The seven cases that were examined histologically illustrate the wide range o eatures that are seen in posterior scleritis oo RPE, which has not been noted beore sseen in 2 cases This retrospective exaition conirms the previous observation thachronic inlammatory cells are prevalnt athe presence o ast cells is a proinent fea-ture Increased numbers o mast cells erseen in 3 cases These cels are thought tinitiate the cascade o T cellmdidelayed hypersensitivity, and inluence B l

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POSTERO SCLERITIS: CLNCL ND HISTOLOGICL SUVEY

y nd h con o vocv c Ony on o h 7 ca w o h ncon vy, ooy n hnc how h yc dd nd

nd con wh wddoo chn Evdnc o cc w nod n 7 c connh vc n ndyn a o

We thank Professor A . Bird for advice on the in-terpretation of te fluorescein angiography, Miss M.Restri for the ultrasond studies ad MorfeldsEye Hopital Audivisual Department.

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