Lovsnal of Elinical Pevsotcience 17 (200:) 12731279

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Lovsnal of Elinical Pevsotcience

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Tegiex

Iianu cell asuesiuit

U.U.N. Ehex, P.O. Mess, J.X. Faneth-Oezes *
Department of Ophthalmology, Faculty of Medical and Health Sciences, The University of Auckland, Private Bag 92019, Auckland, New Zealand

a r t i c l e

i n f o

Article history:
Teceiged 6 Cpsil 200:
Cccepued 7 Oaz 200:

Keywords:
Csuesiuit
Iianu cell
Vemposal

a b s t r a c t
Iianu cell asuesiuit (IEC) it an immvne-mediaued gatcvliuit affecuing indigidvalt oges 60 zeast of age. Ku it
chasacuesited bz gsanvlomauovt inflammauion uhau affecut medivm-tized and lasge asuesiet. Vhe xide
tpecusvm of clinical manifetuauiont can be digided inuo uhote selaued uo uittve itchemia fsom gatcvlas
letiont and uhote selaued uo a tztuemic inflammauosz setponte. Vhe pauhogenetit of uhete gsovpt alto
appeast dituincu, xiuh gatcvlas letion fosmauion uhovghu uo be an adapuige immvne setponte, and uhe tztuemic inflammauosz seacuion an innaue immvne setponte. Elinical tvtpicion of IEC mvtu semain etpeciallz high in uhote xiuh nevsological os gitval tzmpuomt and if xassanued, psompu useaumenu xiuh
high-dote cosuicotuesoidt it ingalvable in haluing diteate psogsettion.
200: Gltegies Nud. Cll sighut setesged.

1. Introduction
Iianu cell asuesiuit (IEC), alto knoxn at uemposal asuesiuit os
gsanvlomauovt asuesiuit, it a tztuemic inflammauosz gatcvliuit uhau
affecut medivm-tized uo lasge asuesiet. Csuesial xall inflammauion
leadt uo lvminal occlvtion and uittve itchemia, xhich cavte uhe
clinical manifetuauiont of uhit gatcvliuit. Uvtcepuible tiuet inclvde;
uhe gatcvlas bed of uhe csanial nesget, opuic nesge, matteues mvtclet, and uhe potuesios ciscvlauion of uhe cenusal nesgovt tztuem.
Kngolgemenu can eyuend uo uhe aosua and iut psimasz and tecondasz
bsanchet, inclvding uhe tvbclagian and ayillasz asuesiet, xhich
leadt uo vppes-eyusemiuz itchemia.
C clotelz selaued enuiuz, polzmzalgia shevmauica, it a tztuemic
inflammauosz diteate uhau manifetut at mzalgiat of uhe neck,
thovldes, and pelgic gisdle. Iianu-cell asuesiuit and polzmzalgia
shevmauica fservenulz occvs uogeuhes in uhe tame pauienu. Yheseat
uhe diagnotit of gianu-cell asuesiuit can be psogen bz uemposal asuesz bioptz, no pauhognomonic uetu it agailable fos polzmzalgia
shevmauica.
Xition lott it a fservenu complicauion of IEC, and once iu occvst
iu uendt uo be bouh pesmanenu and psofovnd. Vhit it uzpicallz uhe
setvlu of asuesiuic anuesios itchemic opuic nevsopauhz (CCKQP), an
infascuion of uhe opuic nesge head tecondasz uo inflammauion of
uhe xall of uhe potuesios ciliasz asuesiet.
2. Epidemiology
Iianu cell asuesiuit it uhe motu fservenu psimasz gatcvliuit,1 psedominanulz affecuing Eavcatian people2 oges 60 zeast of age, xiuh
* Eossetponding avuhos. Vel.; +75 : 383 86::y97265.
E-mail address: h.danethmezesBavckland.ac.nz (J.X. Faneth-Oezes).
0:78-6979/$ - tee fsonu mauues 200: Gltegies Nud. Cll sighut setesged.
doi;10.1017/j.jocn.200:.06.002

xomen affecued au leatu uxice at ofuen at men.3 Kut ogesall incidence it 16 uo 26 pes 100 000/zeas in indigidvalt oges uhe age of
60 zeast;5 hoxeges, incidence incseatet xiuh age; fsom 2.3 pes
100 000/zeas among pauienut in uheis tiyuh decade of life, uo 55.8
pes 100 000/zeas among pauienut in uheis ninuh decade and oldes.6,7
IEC it mose fservenu among people of Ucandinagian and Posuhesn
Gvsopean detcenu, issetpecuige of uheis place of setidence.79 Vhit,
along xiuh seposut of concosdance xiuh monozzgouic uxint: and
familial clvtuesing,10 tvggetut an inhesiued componenu uo uhe
diteate.
Uome tuvdiet hage fovnd an attociauion xiuh JNC-FT5 haplouzpe and 70% of pauienut eypsett uhe D1*0501 os D1*0505/9 gasianu
of uhe JNC-FT5 haplouzpe.11 Vhete allelet appeas uo be a tusong
sitk facuos fos diteate and a maskes fos tuesoid setituance in tome
popvlauiont.12 Vhe inuescellvlas adhetion molecvle 1 (KECO-1)
genet maz alto pseditpote uo IEC.13 KECO-1 it tusonglz eypsetted
in uhe inflammauosz infilusaue of uhe uemposal asuesz in IEC and I/
T 251 polzmosphitmt in uhe KECO-1 gene ase attociaued xiuh IEC
tvtcepuibiliuz.13
3. Clinical features
IEC hat a xide tpecusvm of clinical manifetuauiont selaued uo
eiuhes uittve itchemia fsom gatcvlas letiont, os tztuemic inflammauion. Fve uo uhe xide tpecusvm of tzmpuomt, pauienut maz nou
tponuaneovtlz offes uhe necettasz infosmauion, making disecu and
tpecific rvetuioning impesauige xhen uhese it tvtpicion of IEC.
3.1. Medium-vessel symptoms
Csuesiuit ofuen occvst in uhe casouid asuesiet and uheis bsanchet,
tvch at uhe tvpesficial uemposal, occipiual, ophuhalmic, potuesios

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S.S.L. Chew et al. / Journal of Clinical Neuroscience 16 (2009) 12631268

ciliasz and gesuebsal asuesiet. Jeadachet uhau ase nex and tegese
ase uhvt common in uxo-uhisdt of pauienut. Vheis locauion it chasacuesituicallz uemposal, aluhovgh uhez maz be fsonual, pasieual, os
occipiual.15 Ucalp uendesnett, os disecu uemposal asuesz uendesnett,
thovld alto heighuen tvtpicion of IEC and pauienut ofuen seposu
pain attociaued xiuh bsvthing uheis hais. Lax clavdicauion, selaued
uo sedvced blood flox uo uhe matteues and uemposalit mvtclet, it
highlz tpecific fos IEC and it detcsibed at a csamping uhau incseatet xiuh conuinved matuicauion os tpeech uhau it selieged bz
setu of uhete mvtclet.16 Ease mvtu be uaken uo diffesenuiaue jax
clavdicauion fsom ouhes cavtet of uemposomandibvlas joinu ditcomfosu uhau ase nou itchemic in osigin. Nett fservenu ase tzmpuomt selaued uo itchemia of uhe uongve, face os neck.17

180 IEC pauienut, xiuh a bseakdoxn of gasiovt gitval tzmpuomt

and cavtet of gitval lott (Vable 1).30 Vhe ocvlas tignt and tzmpuomt of IEC ase csiuical uo secognize becavte, if lefu vnuseaued, uhez
can lead uo issegocable blindnett. Xitval tzmpuomt inclvde usantienu os pesmanenu gitval blvssing os lott in one os bouh ezet,
and diplopia. Quhes sases psetenuauiont inclvde gitval hallvcinauiont and phouoptia (vtvallz befose gitval lott), pvpillasz dituvsbancet, osbiual itchemia and ocvlas pain. Qcvlas ingolgemenu
xiuhovu uhe psetence of ouhes IEC tzmpuomt, os occvlu IEC, occvst in beuxeen 6% and 39% of pauienut and uhvt mvtu be inclvded
in uhe diffesenuial diagnotit of an eldeslz pauienu xiuh usantienu os
pesmanenu gitval lott os diplopia, egen xhen uhe pauienu hat no
ouhes uittve itchemia os tztuemic tzmpuomt.31

3.2. Large-vessel symptoms

3.6.1. Permanent visual loss

Resmanenu gitval lott, xhich it vtvallz tegese and can be bilauesal in vp uo one-uhisd of pauienut,32 it uzpicallz uhe setvlu of itchemic opuic nevsopauhz, seuinal os chosoidal itchemia, os saselz
occipiual infascuion.30
CCKQP it uhe motu common ocvlas manifetuauion in IEC,
setpontible fos 89% uo ::% of gitval lott.31,33 Rauienut uzpicallz
psetenu xiuh tvdden, painlett and psofovnd vnilauesal gitval lott;
hoxeges, bilauesal ingolgemenu maz occvs oges hovst uo dazt, vtvallz in uhe teuuing of delazed diagnotit.
Qn eyaminauion uhese it vtvallz psofovnd gitval lott. Kn 70% uo
90% of pauienut, gitval acviuz in uhe affecued eze it lett uhan 7/70
and 20% hage no lighu pescepuion.31,35,36 Vhe clattic finding on fvndotcopz it a pale chalkz xhiue txollen opuic nesge head. Pesge
fibse lazes hemosshaget ase nou vncommon. Vhese maz be couuon
xool tpout and an attociaued cilioseuinal asuesz occlvtion in vp uo
21% of pauienut.37
Kngolgemenu of uhe thosu potuesios ciliasz asuesiet setvlut in
infascuion of uhe laminas os seusolaminas posuion of uhe opuic nesge
head.38 Vhit it folloxed bz psogsettige lirvefacuion of uhe seusolaminas opuic nesge head oges 5 uo 9 xeekt, leading uo fibsotit bz
5 monuht.38 Vhete opuic nesge head changet ase attociaued xiuh
seusogsade degenesauion of seuinal ganglion cell bodiet in uhe seuina
xiuh psetesgauion of uhe ouhes seuinal lazest and anuesogsade
degenesauion in uhe opuic chiatm setvluing in ayonal lott and
demzelinauion.39 Uesial eyaminauion of uhe opuic nesge head segealt tignificanu eycagauion and enlasgemenu of uhe opuic cvp folloxing CCKQP tecondasz uo IEC.3: Vhe end-tuage opuic ditc
appeasance it a pale and cvpped opuic nesge head. CCKQP it au
uimet difficvlu uo dituingvith fsom non-asuesiuic anuesios itchemic
opuic nevsopauhz (PCKQP). CCKQP pauienut maz hage ouhes tztuemic feauvset of IEC, and ase vtvallz oldes xiuh mose tegese gitval lott. Yhile bilauesal timvluaneovt CCKQP it common, PCKQP
it genesallz vnilauesal os affecut ezet tervenuiallz. Qphuhalmotcopic findingt alto diffes, xiuh a hzpesemic opuic ditc txelling
in PCKQP pauienut sauhes uhan uhe pale txelling teen in CCKQP.
Yhile cvpping and pallos of uhe opuic nesge head occvst in motu

Vhe pauuesn of lasge-gettel asuesiuit, psedominanulz affecuing uhe

tvbclagian and ayillasz asuesiet, hat been incseatinglz secognited.18 Cttociaued tzmpuomt inclvde aosuic asch tzndsome xiuh
clavdicauion in uhe asmt, abtenu os atzmmeusical pvltet and blood
psettvse seadingt, pesiphesal pasetuhetiat, and occationallz uittve
gangsene.19 Vhese it ofuen a lack of medivm-gettel csanial tzmpuomt, xhich maz complicaue uhe diagnotit. Cosuic ingolgemenu it
ofuen clinicallz tilenu, bvu can lead uo asuesial dilauauion and anevsztm fosmauion.1:
3.3. Systemic symptoms
Vhe tztuemic inflammauosz componenu of IEC deligest tzmpuomt of joinu tuiffnett, feges of vnknoxn osigin, nighu txeaut, anoseyia and psogsettige xeighu lott.20
3.4. Polymyalgia rheumatica symptoms
Rolzmzalgia shevmauica (ROT) it chasacuesited uzpicallz bz
bilauesallz tegese aching pain and mosning tuiffnett in uhe neck,
thovldes and pelgic gisdlet. Vhe thovldest ase mose fservenulz ingolged uhan uhe neck and pelgit and mosning tuiffnett vtvallz latut
30 minvuet os longes. C tztuemic inflammauosz setponte inclvding
lox-gsade feges, xeighu lott, anoseyia and malaite can occvs in vp
uo 50% of pauienut, fas fexes uhan teen in IEC.21 ROT occvst in uhe
tame popvlauion at IEC, bvu it uxo uo uhsee uimet mose common.22
Vhez ase clotelz selaued condiuiont and abovu 50% of uhote xiuh
IEC hage concvssenu ROT, xheseat 10% of uhote iniuiallz diagnoted at ROT hage hituological egidence of IEC. Iigen uhau each
diteate affecut timilas popvlauiont, thoxt a timilas incseate in
acvue-phate seacuanut and hat a sapid setponte uo glvcocosuicoidt,
IEC and ROT mighu be diffesenu manifetuauiont of uhe tame vndeslzing diteate psocett.21,23
3.5. Neurological involvement
Pevsological manifetuauiont occvs in abovu 30% of pauienut, of
xhich 15% contitu of nevsopauhiet, inclvding mononevsopauhiet
and pesiphesal polznevsopauhiet of uhe vppes os loxes eyusemiuiet.25,26 Nett common (35%) ase usantienu itchemic auuackt, and
tusoket cavte bz tegese obtusvcuion os occlvtion of uhe inuesnal casouid os gesuebsal asuesiet.27 Knflammauion of inusacsanial os inusadvsal asuesiet it sase.28
3.6. Ocular involvement
Vhese it tignificanu ogeslap beuxeen nevsological manifetuauiont and ocvlas ingolgemenu. Vhe sange of ocvlas ingolgemenu it
xide and iut incidence it beuxeen 15% and 80%.29,2: Jazseh eu al.
seposued a 60% incidence of ocvlas ingolgemenu in a cohosu of

Table 1
Qcvlas tzmpuomt and cavtet of gitval lott in 96 pauienut xiuh gianu cell asuesiuit
(Cdapued fsom Jazseh eu al.30)
Uzmpuomt/cavtet
Symptoms
1. Cmavsotit fvgay
2. Xitval lott
3. Fiplopia
5. Gze pain
Causes
1. Cnuesios itchemic opuic nevsopauhz
2. Eenusal seuinal asuesz occlvtion
3. Eilioseuinal asuesz occlvtion
5. Rotuesios itchemic opuic nevsopauhz

Rsetence (%)
30.7
:7.:
6.:
9.2
91.1
15.1
21.9
8.1

S.S.L. Chew et al. / Journal of Clinical Neuroscience 16 (2009) 12631268

pauienut xiuh CCKQP folloxing setolvuion of uhe opuic ditc txelling,

onlz tegmenual pallos bvu no cvpping it egidenu in PCKQP.50
Teuinal asuesz occlvtion it a lett common cavte of gitval lott in
IEC, affecuing 10% uo 13% of uhote xiuh ocvlas ingolgemenu. Vhe
cenusal seuinal asuesz it motu commonlz affecued; hoxeges, bsanch
seuinal and cilioseuinal asuesz occlvtion hat been detcsibed.51 IEC
mvtu uhesefose be eyclvded in pauienut oges uhe age of 60 psetenuing xiuh seuinal asuesz occlvtion xiuhovu egidence of embolic
occlvtion on fvndotcopz.
Rotuesios itchemic opuic nevsopauhz it a lett common cavte of
gitval lott in IEC cavted bz infascuion of uhe seusobvlbas opuic
nesge. Vhit posuion of uhe opuic nesge it tvpplied inusaosbiuallz bz
uhe pial pleyvt, and inusacsaniallz bz bsanchet of uhe inuesnal casouid, anuesios cesebsal, and anuesios commvnicauing asuesiet. Elinical feauvset uhau tvggetu uhit ase psofovnd decseate in gitval
acviuz, a selauige affesenu papillasz defecu, and no egidence of opuic
nesge head txelling.
3.6.2. Transient visual loss
Vsantienu gitval lott hat been seposued in 2% uo 30% of pauienut
xiuh IEC.30 Wnilauesal usantienu gitval lott (amavsotit fvgay) dve
uo sedvced ocvlas blood flox it mvch mose common uhan uhe bilauesal usantienu gitval lott setvluing fsom potuesios ciscvlauion itchemia.52,53 Rauienut of uhe appsopsiaue age xho psetenu xiuh
amavsotit fvgay xiuhovu tignt of seuinal emboli xassanu sapid
eyclvtion of IEC at uhit can psecede pesmanenu gitval lott fsom
itchemic opuic nevsopauhz os cenusal asuesz occlvtion in abovu 8%
uo 19% of pauienut.55

1276

4.2. The innate immune response

Vhe acvue-phate setponte mediaued bz uhe innaue immvne tztuem it setpontible fos uhe tztuemic inflammauosz componenu of
IEC. Ccvue-phate setpontet ase tztuemic seacuiont uo tegese tusett
and injvsz and ase genesaued gia a catcade of tignalt, xiuh inueslevkin-7 being csiuical bz tuimvlauing uhe psodvcuion of acvue-phate
psoueint in uhe liges.70,71 Eiscvlauing monoczuet ase a majos tovsce
of inueslevkin-7; hoxeges, uhe mechanitm and tiue of monoczue
acuigauion in IEC ase vnknoxn.72
5. Histopathology
Jituologicallz IEC it chasacuesited bz gsanvlomauovt inflammauion affecuing all lazest of medivm-tized and lasge asuesiet uhau hage
an inuesnal elatuic lamina.73 Vhe inflammauosz infilusaue it compoted
of acuigaued EF5+ V cellt, macsophaget, and gianu cellt.75 Cluhovgh
nou necettasz fos a diagnotit of IEC, xhen psetenu, mvluinvcleaued
gianu cellt ase vtvallz clote uo a fsagmenued inuesnal elatuic lamina.
Nvminal tuenotit os occlvtion maz be gitvalited dve uo inuimal
hzpesplatia and tvbtervenu mvsal uhickening tecondasz uo mzofibsoblatu migsauion and psolifesauion.76 Vhe diteate hat a gasied uemposal and tpauial covste, uhvt tegmenut of vningolged segiont
adjacenu uo aseat of inflammauion, os tkip letiont, ase common.
6. Diagnosis

3.6.3. Diplopia
Ktchemia of eyusaocvlas mvtclet, ocvlas mouos nesget os uhe
bsaintuem maz be setpontible fos diplopia and ocvlas mouos imbalance, usantienu os contuanu, in 2% uo 16% of IEC pauienut. Vhe occvlomouos (KKK) nesge it uhovghu uo be motu commonlz ingolged, ofuen
tpasing uhe pvpil; hoxeges, ingolgemenu of uhe usochleas (KX) and
abdvcent (XK) nesget hat been seposued.30,56

Vhese it no tingle clinical tzmpuom, tign os labosauosz uetu tpecific fos IEC.16 Kn 1::0, uhe Cmesican Eollege of Thevmauologz
(CET) fosmvlaued clattificauion csiuesia uhau xese effecuige in dituingvithing IEC fsom ouhes fosmt of gatcvliuit (Vable 2). Kn a pauienu xiuh a diagnotit of tome fosm of gatcvliuit, uhe psetence of
uhsee of uhe fige csiuesia xat deemed uo be :5% tentiuige and :1%
tpecific fos uhe diagnotit of IEC.77 Vhe CET did nou hoxeges, uake
inuo accovnu ouhes imposuanu facuost tvch at gitval tzmpuomt, jax
clavdicauion, neck pain and saited E-seacuige psouein.

4. Etiology and Pathogenesis

6.1. Erythrocyte sedimentation rate

Yhile uhe eyacu euiologz of IEC it vnknoxn, a gasieuz of infecuiovt agenut hage been tvggetued at pouenuial immvne usiggest fos
uhe diteate, inclvding hespet gisvt, pasainflvenza gisvt, czuomegalogisvt, pasgogisvt D1:, chlamzdia and mzcoplatma.575: Vhese it
liuule dovbu uhau immvne-mediaued psocettet facuos in uhe pauhogenetit of IEC, and bouh uhe adapuige and innaue immvne tztuemt
hage been implicaued.60

Glegauion of uhe eszuhsoczue tedimenuauion saue (GUT) it a nontpecific indicauos of inflammauion and it incseated in malignancz,
infecuion, connecuige uittve ditosdest, usavma, anemia and hzpescholetuesolemia. Yhile a tignificanu elegauion of uhe GUT it ofuen
psetenu in IEC, iu maz be nosmal in vp uo 18% of diagnoted catet,
and uhvt cannou be vted uo eyclvde IEC.78 Vhe vppes limiu fos a
nosmal GUT it uhovghu uo incseate xiuh age and hat been defined
at uhe age digided bz uxo fos men, and uhe age plvt 10 digided
bz 2 fos xomen.79 Kn motu IEC pauienut xiuh GUT elegauion, iu
can be vted accvsauelz uo moniuos diteate acuigiuz.7:

4.1. The adaptive immune response

Knappsopsiaue acuigauion of uhe adapuige immvne tztuem bz
dendsiuic cellt it uhovghu uo cavte uhe gatcvlas letiont teen in
IEC.6062 Kmmauvse dendsiuic cellt nosmallz inhibiu V-cell acuigauion in uhe pesigatcvlas tpace, bvu vpon acuigauion uhez auusacu bouh
V-cellt and macsophaget uo uhe gettel xall gia uhe gata gatosvm. Kn
uhe adgenuiuial lazes, telecuige clonal eypantion of EF5+ V cellt
leadt uo uhe seleate of czuokinet tvch at inuesfeson gamma (KHPc), xhich secsviu macsophaget inuo uhe gettel xall uo fosm a psominenu gsanvlomauovt seacuion.6366 Vhe effecu of uhe macsophaget
dependt on uheis locauion xiuhin uhe asuesial xall67 and uhez maz
cavte a pso-inflammauosz effecu in uhe adgenuiuia, oyidauige
detusvcuion in uhe media,68 and hzpesplatia in uhe inuima.69 Vhe
covste of uhe inflammauion in uhe gettel xall dependt on uhe uzpe
of V-cell diffesenuiauion. Kf telecuion it uoxasd high KHP-c seleate,
masked inuimal hzpesplatia, uittve itchemia and lvminal occlvtion
it mose likelz.6: Kf uhese it biat uoxasd lox KHP- c and high inueslevkin-2 legelt, gatcvliuit xill egolge xiuhovu lvminal occlvtion.

6.2. C-reactive protein

E-seacuige psouein (ETR) it an acvue-phate psouein seleated bz
hepauoczuet in setponte uo inflammauion. Cn elegaued ETR hat a
Table 2
Vhe Cmesican Eollege of Thevmauologz 1::0 csiuesia fos uhe clattificauion of gianu cell
asuesiuit77
Vhe psetence of uhsee os mose of uhe folloxing csiuesia it servised fos uhe
diagnotit of gianu cell asuesiuit;
1. Cge of onteu gseaues uhan 60 zeast
2. Qnteu of nex headache
3. Vemposal asuesz abnosmaliuz (uendes os sedvced pvltauion)
5. Glegaued eszuhsoczue tedimenuauion saue, defined at 60 mm/h vting uhe
Yetuesgsen meuhod
6. Cbnosmal asuesial bioptz thoxing necsouizing gatcvliuit xiuh psedominanu
mononvcleas cell infilusauion os gsanvlomauovt inflammauion

tpecificiuz of vp uo :8.6% in pauienut xiuh IEC, and maz be elegaued in uhe psetence of a nosmal GUT.80 Yhile iu it mose tentiuige
uhan an elegaued GUT (8797%), xhen bouh uetut ase vted in conjvncuion, uhe tentiuigiuz it incseated uo oges ::%.80 Kn conusatu uo
GUT, ETR hat an accvsauelz defined nosmal sange (>6 mg/N) uhau
doet nou gasz xiuh age os tey, and iut vte in diteate moniuosing
it lett xell docvmenued.
6.3. Full blood count
Vhsomboczuotit hat been potiuigelz cosselaued xiuh bioptz-psogen IEC and semaint an imposuanu maskes fos IEC.81,82 Kn facu, an
elegaued plaueleu covnu (@500  103/N) maz be egen mose tpecific
uhan bouh GUT and ETR in uhe diagnotit of IEC.83 Cbovu 20% uo
60% of pauienut xiuh IEC xill alto hage a nosmoczuic nosmochsomic anaemia.85
6.4. Other laboratory tests
Glegaued platma gitcotiuz, fibsinogen, complemenu, inueslevkin7 (KN-7) and a2-and b-globvlint hage been fovnd in IEC.86,87 KN-7
maz be mose tentiuige uhan GUT in indicauing diteate acuigiuz.88
Cbnosmal liges fvncuion uetut, in pasuicvlas saited alkaline photphauate, maz occvs in vp uo one half of pauienut xiuh IEC.89
Cnui-casdiolipin anuibodiet ase ofuen saited in IEC and dvsing selaptet. Wnlike GUT and ETR, uhez do nou incseate in non-IEC-attociaued inflammauion.8:
6.5. Temporal artery biopsy
Vemposal asuesz bioptz (VCD) it uhe gold tuandasd fos uhe diagnotit of IEC and thovld be pesfosmed on all pauienut in xhom uhe
diagnotit of IEC it tvtpecued. Jituological confismauion it secommended tince long-uesm cosuicotuesoid useaumenu it nou xiuhovu
complicauiont.90 Joxeges, osganitauion of uhe VCD thovld neges
delaz uhe intuigauion of useaumenu. Qpuimallz, VCD thovld be pesfosmed xiuhin one xeek of iniuiauing tztuemic cosuicotuesoidt;
hoxeges, hituological egidence semaint vp uo tiy xeekt folloxing
useaumenu.91 Vo agoid a falte negauige setvlu dve uo tkip letiont, a
minimvm lenguh of 20 mm thovld be obuained. Ku thovld be
semembesed uhau uhe uemposal asuesz can conusacu tvbtuanuiallz
afues eycition and befose uittve fiyauion.92 Kn tuvdz of 38 negauige
and 8 potiuige bioptiet fos IEC uhe mean conusacuion xat 12%
fos potiuige tpeciment and 22% fos negauige tpeciment.92 Qgesall
uhe tentiuigiuz of VCD it 98%.93 Kf uhe VCD setvlu it negauige and
uhe tvtpicion of IEC it high, a conusalauesal VCD thovld be
pesfosmed.93
6.6. Other investigations
Hlvosetcein angiogsaphz it vtefvl in pauienut xiuh ocvlas IEC.
Rsolonged chosoidal and cenusal seuinal asuesial filling uimet, and
chosoidal non-pesfvtion os filling defecut ase tvggetuige of
CCKQP.95 Wlusatonogsaphz of affecued asuesiet it a vtefvl non-ingatige uechnirve uo deuecu changet in uhe asuesial xall attociaued
xiuh IEC, changet in osbiual blood flox, os uo usack uhe asuesial
covste psios uo bioptz.96
OTK and potiuson emittion uomogsaphz (RGV) tcanning hage
been vted tvccettfvllz uo diagnote lasge-gettel gatcvliuit and
aosuiuit.97,98

7. Treatment
Vhe goal of useaumenu it uo psegenu ongoing itchemic damage
uo ouhes osgant in uhe bodz. Kn pasuicvlas, if uhe gitval lott it

uhe majos tzmpuom, uhe aim it uo halu uhe psogsettion of gitval

lott in uhe affecued eze and psegenu ingolgemenu of uhe conusalauesal eze.
7.1. Systemic corticosteroids

Uztuemic cosuicotuesoidt ase uhe maintuaz of useaumenu. Jighdote cosuicotuesoid useaumenu psogidet anuioyidanu and gatodilauosz effecut on uop of uheis anui-inflammauosz effecut.99 Fetpiue uhit,
iu it xell secognited uhau psogsettige gitval lott maz occvs detpiue
uhe easlz intuiuvuion of high-dote cosuicotuesoidde8Vm
[(Kndhetti2(eff%:j
07.0):

S.S.L. Chew et al. / Journal of Clinical Neuroscience 16 (2009) 12631268

7.4. Aspirin
Vhsombotit tecondasz uo inflammauion, inuimal hzpesplatia and
tuenotit mighu be a majos conusibvuos uo itchemic complicauiont. C
seusotpecuige tuvdz of pauienut xiuh IEC fovnd uhau ogesall 11 of
79 pauienut (17.2%) had an itchemic egenu xhile seceiging anuiplaueleu os anuicoagvlanu uhesapz compased uo 37 of 86 pauienut
(59.0%) nou seceiging tvch uhesapz (p > 0.0006).:7 Po incseate in
hemosshagic complicauiont xat teen.:7 Vhe avuhost conclvded
uhau anuiplaueleu agenut maz sedvce uhe sitk of itchemic egenu in
IEC; hoxeges, fvsuhes tuvdiet ase needed.
7.5. Other treatments
Czauhiopsine, czclophotphamide, daptone and czclotposine
hage been tvggetued at pottible cosuicotuesoid tpasing agenut,
aluhovgh uhese ase no conclvtige daua au uhit uime.:8::
8. Prognosis
Eavtet of mosualiuz attociaued xiuh IEC inclvde casdiogatcvlas,
nevsological and gatusoinuetuinal egenut. Xatcvliuit of uhe cosonasz
asuesiet maz setvlu in mzocasdial infascuion os congetuige heasu
failvse.100 Cosuic anevsztm svpuvse os aosuic dittecuion it pottible.
Ktchemic bsain damage it uhe tecond motu common cavte of IECselaued deauh afues casdiac diteate.101 Vhit maz occvs bz eiuhes
inflammauosz obtusvcuion of uhe gesuebsobatilas os casouid asuesial
tztuemt, embolitm os psopagauion of uhsombvt fsom inflamed
asuesiet uo vninflamed asuesiet. Pecsouizing tegmenut of boxel
ase vncommon, bvu can alto be faual.
Vhe psognotit fos gitval secogesz it poos folloxing IEC.
Cluhovgh impsogemenu in gitval acviuz hat been seposued in a
tmall minosiuz of pauienut,33 uhit it likelz tecondasz uo eccenusic
fiyauion os leasning cvsge sauhes uhan usve impsogemenu in vtefvl
gition. Hvsuhesmose, gitval deuesiosauion occvst in abovu 28% of
ezet detpiue high-dote inusagenovt meuhzlpsednitolone.37 Vhe
gseauetu sitk of gitval deuesiosauion it in uhe fistu tiy dazt.37 Joxeges, useaumenu xiuh inusagenovt cosuicotuesoidt it effecuige in
sedvcing uhe likelihood of fellox eze ingolgemenu.31 Yiuhovu useaumenu uhe sitk of fellox eze ingolgemenu it 65% uo :6%.31
9. Conclusion
Iianu cell asuesiuit it a tztuemic gatcvliuit xiuh a xide clinical
tpecusvm, inclvding tignificanu nevsological and ocvlas manifetuauiont uhau hage degatuauing contervencet. Reshapt one of uhe motu
tignificanu it gition lott tecondasz uo CCKQP. Eliniciant mvtu uhesefose hage a high indey of tvtpicion fos IEC, etpeciallz in uhe teuuing of vneyplained gitval os nevsological tzmpuomt. Cluhovgh
uhese it no tingle labosauosz uetu uhau etuablithet uhe diagnotit,
uhe psetence of uittve itchemic and tztuemic tzmpuomt in attociauion xiuh elegaued inflammauosz maskest tvch at GUT and ETR ase
tignificanu csiuesia uhau thovld be vted fos easlz deuecuion and
psompu useaumenu xiuh high-dote cosuicotuesoidt. Uince uhit line
of useaumenu it nou xiuhovu tignificanu complicauiont, bioptz confismauion it ettenuial xhese pottible. Hvuvse seteasch xill no dovbu
be focvted au degeloping nex cosuicotuesoid-tpasing useaumenu
modaliuiet.
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