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WhatisVasculitis?
TheImmuneSystem
GiantCellArteritis/TemporalArteritis
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WhatisGiantCellArteritis/TemporalArteritis?
GCAisadiseasecharacterisedbyinflammationoflargeandmediumsizedbloodvessels.An
alternativenameforthisconditionis"TemporalArteritis"asthebloodvesselsinthetemple
areaofthehead(sidesoftheforehead)arecommonlyaffected.Thegiantcellsreferredtoare
specificcollectionsofimmunesystemcellsseenintheareasofinflammationifabiopsyis
performed.
Whoareaffected?
Thisconditionisoneofthecommonestformsofvasculitis,affectingoneortwopersonsper
10,000peopleintheUK.Itismorecommonlyseeninolderpatientsandisseldomdiagnosed
belowtheageof50.
Whatistheaetiology(cause)?
DiseasesAffectingSmall
BloodVessels
ThecauseofGiantCellArteritis/TemporalArteritisisnotyetknown.
GlossaryofDrugs
Whatarethesymptoms?
GlossaryofProcedures
Symptomsoffatigue,lossofappetite,weightlossandfeverareoftenfound.Headache,with
painandtendernessoverthetemples,isaprominentfeatureofthisdiseasedueto
inflammationofthetemporalarteries.GCAiscloselyassociatedwiththePolymyalgia
RheumaticaSyndromewhichcausesgeneralweakness,painandstiffnessinmusclesand
joints.Theweaknessinthemusclescanbequitedebilitatingandtypicallyaffectstheupper
armsandlegs.Simpletaskssuchasbrushingthehair,cooking,gardening,gettingupandout
ofchairsorbedsandwalkingmaybecomedifficult.
GlossaryofBloodTest
Monitoring
FrequentlyAsked
Questions
Asthisconditionaffectsthemainbloodvesselssupplyingpartsofthebody,areductioninthe
bloodandoxygensupply(ischaemia)todifferentorganscanresult.Thiscanleadtoavariety
ofsymptoms.Aclassicsymptomofthisconditionispaininthetongueorjawwheneating
(jawclaudication).Reductionofthebloodsupplytothebraincancauseastroke.Reductionin
thebloodsupplytotheeyescanleadtoblurredvision,orinsomepatients,blindness.
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Makingadiagnosis
Aswithmosttypesofvasculitisthereisnoonetestthatwillgivethediagnosis.Thediagnosis
isusuallymadebecausethedoctorrecognisesthetypicalcollectionofsymptomsandclinical
signs.Bloodtestsforinflammation(ESRerythrocytesedimentationrate;CRPCreactive
protein;plasmaviscosity)willusuallybehighindicatinginflammationbutthesearenot
specificandcanberaisedinanytypeofvasculitis,manyinfectionsandothertypesof
inflammatorydisease.Ifthetemporalarteriesareinvolved(locatedatthesidesofthe
foreheadoverthetemples)thenabiopsymaybeperformed.Ifthebiopsyshowsinflammation
thiswillhelptoconfirmthediagnosis.Unfortunatelyabiopsywithoutinflammationdoesnot
excludethediagnosis.Sometimesanultrasoundscanofthearteriesisusedinsteadofa
biopsy.
Treatment
Thetwoaimsoftreatmentaretorelievepatient'ssymptomsandpreventdamagetoorgans
(especiallytheeyes)duetoreducedbloodsupply.Highdoseoralsteroids(Prednisolone)are
usuallygivenandthedoseisreducedslowlyovermonths,usingtheESRmarkerasaguide
toresponse.Treatmentwithsteroidsisusuallygivenforatleastoneortwoyears.
Insomepatientswithresistantorproblematicdiseaseotherdrugsaresometimesusedsuch
asmethotrexate,Azathioprineorantibodytherapysuchasinfliximab.Whilesomepatients
mayrespondwelltothesedrugsthereisnocurrentevidencethattheyareeffectiveinall
patients.
DrugsandSideeffects
ForinformationonthemaindrugsprescribedforGiantCellArteritissee:
Azathioprine
Infliximab
Methotrexate
Steroids
ForinformationonotherdrugsusedinthetreatmentofvasculitisseeGlossaryofdrugsand
sideeffects.
Prognosis
Thereisarelapserateofabout50percent.Withdrawalofthesteroidscanleadtoarelapse
ofthesymptoms.Insuchcasesthesteroidisrestartedanditisoftensufficienttoinduce
remission.Thesteroiddosecanagainbereduced.Inmostrelapsedcasesthepatientwillbe
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maintainedonasmallmaintenancedoseofsteroidsindefinitely.Theprognosisisgoodwith
correctandearlydiagnosisandtreatment.
KeyPoints
GCAisoneofthecommonestformsofvasculitis
Symptomsincludeheadacheandjawclaudication(crampingpain)
Temporalarterybiopsyorultrasoundscanmayhelpconfirmthediagnosis
Damagetotheeyesisusuallythemostseriouscomplication
Treatmentisusuallywithsteroidsandmaycontinueformanyyears.
RelatedVasculitisArticles
FertilityandVasculitisDrDavidJayne
VasculitisandtheEyesDrCatherineGuly
Furtherreading
GiantCellArteritis:Suspectit,treatit,treatitproperlyAlexandraVillaForte
TheTreatmentofGiantCellArteritisJ.AlexanderFraser,CorneliaM.Weyand,etal
LargeVesselDiseasesAshimaGulati,ArvindBagga
ReportResearchintoGiantCellArteritisScienceDaily17Jan2014
Usefullinks
OurUsefulVasculitisLinkspagecontainscontactdetailsfororganisationsofferinghelpand
supportforpatientswithGiantCellArteritis,PolymyalgiaRheumaticaandothervasculitis
diseases.
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