Systemic Juvenile Idiopathic Arthritis - Clinical Manifestations and Diagnosis

20/10/2015
Systemicjuvenileidiopathicarthritis:Clinicalmanifestationsanddiagnosis
OfficialreprintfromUpToDate
www.uptodate.com2015UpToDate
Author
YukikoKimura,MD
SectionEditor
MarisaKleinGitelman,MD,MPH
DeputyEditor
ElizabethTePas,MD,MS
Alltopicsareupdatedasnewevidencebecomesavailableandourpeerreviewprocessiscomplete.
Literaturereviewcurrentthrough:Sep2015.|Thistopiclastupdated:Sep09,2014.
INTRODUCTIONSystemicjuvenileidiopathicarthritis(sJIA,formerlycalledStill'sdiseaseorsystemic
juvenilerheumatoidarthritis)isofficiallyclassifiedasasubsetofjuvenileidiopathicarthritis(JIA)thatdescribes
patientswithintermittentfever,rash,andarthritis.Itistermed"adultonsetStill'sdisease"whenitoccursin
patientsovertheageof16.sJIAaccountsforabout10to20percentofallcasesofJIA.Ittypicallyaffectsboth
sexesequallyandmaypresentinchildrenasyoungasoneyearofageoryounger.(See"Juvenileidiopathic
arthritis:Epidemiologyandimmunopathogenesis".)
TheclinicalmanifestationsanddiagnosisofsJIAarereviewedhere(table1).Themanagement,complications,
andprognosisofsJIA,aswellasoligoarticular,polyarticular,enthesitisrelated,andpsoriaticJIAandgeneral
topicsonJIAarediscussedseparately.(See"Systemicjuvenileidiopathicarthritis:Treatment"and"Systemic
juvenileidiopathicarthritis:Course,prognosis,andcomplications"and"Oligoarticularjuvenileidiopathicarthritis"
and"Polyarticularjuvenileidiopathicarthritis:Clinicalmanifestationsanddiagnosis"and"Spondyloarthropathyin
children"and"Psoriaticjuvenileidiopathicarthritis:Pathogenesis,clinicalmanifestations,anddiagnosis"and
"Psoriaticjuvenileidiopathicarthritis:Managementandprognosis"and"Classificationofjuvenilearthritis"and
"Juvenileidiopathicarthritis:Epidemiologyandimmunopathogenesis".)
OVERVIEWPatientswithsystemicjuvenileidiopathicarthritis(sJIA)fallintothecategoryofsystemicarthritis
inthe2004proposedclassificationofthechildhoodarthritides[1].Childrenwiththisillnesscomprisebetween10
and20percentofallcasesofjuvenileidiopathicarthritis(JIA).However,datasuggestthatthisillnessisaunique
conditionclosertotheautoinflammatoryfamilyofdiseases,withdistinctmanifestationsandtreatmentresponses
thatdistinguishitfromtheotherdiseasescategorizedasJIA[2,3].(See"Classificationofjuvenilearthritis"and
"Periodicfeversyndromesandotherautoinflammatorydiseases:Anoverview".)
ThisformofJIAmaybethemostdifficulttodiagnoseandtreatforthefollowingreasons:
Arthritis,althoughnecessarytoestablishadefinitivediagnosis,maynotbeevidentearlyinthecourseofthe
disease[1,4].
Thesystemicdiseasefeaturesmaynotbepresentintheirtypicalformsinitially,eventhoughthesetypical
featuresarealsonecessarytoestablishadefinitivediagnosis[1,4].
Therearenodiagnostictestsforthisdisorder.
Affectedchildrenoftenappearquiteillwithhighspikingfevers,rashes,markedlyelevatedwhitebloodcell
(WBC)counts,andanemia.Mostsuchchildrenareinitiallythoughttohaveaninfectionormalignancy,and
thecorrectdiagnosisisonlysuspectedaftertherehasbeennoresponsetoantibiotictherapyand
malignancyhasbeenexcluded.
Somechildrenwhopresentwithfever,rash,anddiffusejointpaindoinfacthaveinfections,leukemia,orother
seriousconditionsthatarenotsJIA.Thus,itisessentialthatcliniciansthoroughlyexcludeotherconditionsbefore
makingthisdiagnosis.
CLINICALMANIFESTATIONSChildrenwithsystemicjuvenileidiopathicarthritis(sJIA)maypresentwitha
varietyofarticularandextraarticularsignsandsymptoms.Inonelargecaseseriesfromatertiarycenter,136
patientswerediagnosedwithsJIAfrom1990to2005[4].Thefollowingmanifestationsatdiseaseonsetand
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relativefrequencywerenoted:
Fever98percent
Arthritis88percent(8percentwithmonoarthritis,45percentwitholigoarthritis,and47percentwith
polyarthritis)
Rash81percent
Lymphadenopathy31percent
ArticularmanifestationsArthralgiasarecommonearlyinthecourseofsJIA,butarthritisisnotalways
prominent.Anynumberofjointsmaybeinvolvedwhenarthritisbecomesapparent.Diseaseinthewrists,knees,
andanklesismosttypical,butthehands,hips,cervicalspine,andtemporomandibularjointsarealsosometimes
affected.Unliketheoligoarticularandpolyarticularsubtypesofjuvenileidiopathicarthritis(JIA),thearthritisof
sJIAmaybegininthehipsandmayprogressveryrapidly,causingseveredamageanddysfunctionandneedfor
earlyjointreplacementsurgeryaswellaslossofgrowthpotentialinyoungerpatients.(See"Systemicjuvenile
idiopathicarthritis:Course,prognosis,andcomplications",sectionon'Growthretardation'.)
MicrognathiaandcervicalspinefusionarecommonlypresentinchildrenwithlongstandingsJIA,butdonotappear
untilafterthediseasehasbeenpresentformonthstoyears(image1)[5].Inthepast,theseabnormalitiesaffected
asmanyas50percentofchildrenwithdiseaseonsetinthefirstdecadeoflife.Earlierandmoretargeted
treatmenthasledtoadecreaseinthesecomplications.(See"Systemicjuvenileidiopathicarthritis:Treatment".)
ExtraarticularmanifestationsThemostcommonextraarticularmanifestationsarequotidianfeveranda
macular,salmonpinkrash.
FeverChildrenwithsJIAusuallycometomedicalattentionurgentlybecausetheytypicallypresentwith
highfevers.Thekeyfindingthatsuggeststhediagnosisisthedaily,butintermittent,natureofthefever.The
diagnosisisstronglyindoubtifthepatient'stemperaturedoesnotspontaneouslyreturntonormalonadailybasis.
Thispatternofonehighspikeoffeveradaywithnormalorsubnormaltemperaturesduringtherestofthe24hour
periodiscalledquotidianfever.Somechildrenhavemorethanonespikeoffeveraday,buttheirtemperature
returnstonormalinbetweenthefeverspikes.Affectedchildrenarecharacteristicallyillappearingwhenfebrile,but
seemtoimprovedramaticallywhentheirtemperatureisnormal.Ifrashispresent(see'Rash'below),italsotends
toappearorworsenwhenthefeverispresent.Thesefeatures,inadditiontothelackofresponsetoantibiotics,
helptodistinguishpatientswithsJIAfromthosewithaninfection.(See'Differentialdiagnosis'below.)
Somechildrendonothavethistypicalfeverpatterninitiallyandhavecontinuousfevers,feverthatdoesnotoccur
daily,ornofeveratall.Ifuntreated,however,mostpatientswilleventuallydevelopthishighlyspecificfever
pattern.
RashAmacular,salmonpinkrashisfrequentlypresent(picture1).Itusuallyconsistsofmultipleroundto
ovalmaculesthatareslightlyraisedandareofdifferingsizes.Thesmalleroneshaveaslightpallorsurrounding
themandthelargeronescommonlyexhibitcentralpallor.Therashisoftendifficulttodetectormaynotbenoticed
atallindarkskinnedindividuals.Therashisbroughtoutbyheatandtouch,andisoftenfoundintheaxillaeand
aroundthewaist,butmaybepresentanywhereonthebody.Itismostprominentwhenthechildisfebrile.Unlike
viralexanthems,whichpersist,therashoftenfadesasthetemperaturereturnstonormal,onlytoreappearwith
thenextfeverspike.Therashalsomayappearfollowingstrokingoftheskinorotherminortrauma(Koebner
phenomenon)(picture2).Therashcanbeintenselypruriticinsomepatients.Itcanbecomesomewhatconfluent
andbecomeurticarialinappearance,especiallyinthosepatientswhoscratchthemselvesbecauseofthepruritus
[6].
OtherclinicalfindingsInaddition,thefollowingclinicalfeaturescanbeobserved:
Hepatomegaly,splenomegaly,andlymphadenopathyarecommonlyfound.Thiscombinationfrequently
raisessuspicionofamalignancy,butlymphnodebiopsyshowsbenignreactivehyperplasia.(See
'Malignancy'below.)
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PericarditisandotherformsofserositisareafeatureofsJIA.Asmallpericardialeffusionthatisnotusually
clinicallysignificantisoftennotedonechocardiogram.Largepericardialeffusionsarerare.Pericarditismay
resultintamponade,insomecaseswithafataloutcome[7].
Pulmonarymanifestationsincludepleuraleffusion,whichmostoftenpresentduringtheacutephaseofthe
illnessinpatientswithserositis.Interstitiallungdisease,alveolarproteinosis,andpulmonaryhypertension
arerare,butarebeingreportedwithincreasedfrequency[8].
SomechildrenmayinitiallypresentwithfeaturessuggestiveofKawasakidisease(KD)oratypicalKD,
includingcoronaryarterydilationonechocardiograminsomecases[911].Thesepatientsareonlydiagnosed
withsJIAwhentheyfailtorespondtotreatmentforKD.(See"Kawasakidisease:Clinicalfeaturesand
diagnosis"and"Incomplete(atypical)Kawasakidisease",sectionon'Clinicalpresentationoftypicalversus
incompleteKD'.)
LABORATORYFINDINGSCharacteristicpatternsoflaboratoryabnormalitiesfrequentlyarepresentand
suggestiveofthedisease,althoughtherearenolaboratoryfindingsthatarespecificforthediagnosis:
Whitebloodcell(WBC)countsinthe20,000to30,000/mm3rangearenotuncommon,andattimesthey
mayexceed60,000to80,000.Granulocytespredominate[4].
Thereisusuallyamarkedreactivethrombocytosisandtheplateletcountmayexceed1,000,000/mm3[4].
Thus,thrombocytopeniaorevenlownormalplateletcounts(eg,lessthan200,000/mm3)shouldprompt
carefulevaluationforanalternativediagnosis(eg,bonemarrowaspirationtoexcludeleukemia).Asudden,
rapiddropintheplateletcountmayheraldthedevelopmentofmacrophageactivationsyndrome(MAS),a
formofhemophagocyticlymphohistiocytosis.(See'Malignancy'belowand"Clinicalfeaturesanddiagnosis
ofhemophagocyticlymphohistiocytosis",sectionon'Rheumatologicdisorders/MAS'and"Systemicjuvenile
idiopathicarthritis:Course,prognosis,andcomplications",sectionon'Macrophageactivationsyndrome'.)
Anemiaiscommonlypresentandoftenprofound.Itisprimarilyduetodecreasedsynthesisandpoor
absorptionoforalironratherthanincreaseddestruction[12].Theanemiacanbeexacerbatedbychronic
gastrointestinalbloodlossinducedbynonsteroidalantiinflammatorydrugs(NSAIDs).Thus,childrenwith
systemicjuvenileidiopathicarthritis(sJIA)commonlybecomeirondeficientovertime,butoraliron
supplementationisoftenineffectiveuntiltheunderlyinginflammatoryprocesshasbeencorrected.
Intravenousorintramuscularironmayberequiredincasesofsevereirondeficiency[13].Oraliron
supplementationisoftennecessaryoncesystemicdiseasebeginstocomeundercontrol.(See"Iron
deficiencyininfantsandyoungchildren:Treatment".)
Theerythrocytesedimentationrate(ESR)istypicallyelevatedinchildrenwithsJIAandmayexceed100
mm/hour.AnormalESRisrarewhenthediseaseisactive.ThelevelofCreactiveprotein(CRP),another
acutephasereactant,isusuallyelevatedaswell.ThecombinationofadramaticfallintheESRanda
dramaticfallintheplateletcountmayheraldtheonsetofMASinanunwellchildratherthanclinical
improvement.Carefulevaluationofchildrenwiththiscombinationoffindingsisessential.Adramaticrisein
theserumferritinlevelorinfibrinsplitproducts(Ddimers)alsostronglysuggeststheonsetofMAS,anda
ferritin/ESRratioof80isaproposedbiomarkerofMAS[14].(See"Clinicalfeaturesanddiagnosisof
hemophagocyticlymphohistiocytosis",sectionon'Rheumatologicdisorders/MAS'and"Systemicjuvenile
idiopathicarthritis:Course,prognosis,andcomplications",sectionon'Macrophageactivationsyndrome'.)
Extremehyperferritinemia(oftengreaterthan1000oreven50,000ng/mL)isalsocommoninchildrenwith
activesJIA,evenifovertMASisnotpresent.ItcanbeusefulinraisingsuspicionforthediagnosisofsJIA,
sinceveryfewotherillnessesareassociatedwithsuchextremeelevationsinferritinlevels[3,15].(See
"Clinicalfeaturesanddiagnosisofhemophagocyticlymphohistiocytosis",sectionon'Rheumatologic
disorders/MAS'.)
Minorelevationsinaspartateaminotransferase(AST)andalanineaminotransferase(ALT),hypoalbuminemia,
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increasedglobulinlevels,andlowgradeDdimerpositivityareoftenpresent.However,markedlyelevated
hepaticenzymeabnormalities,prolongedclottingtimes,oranincreaseinDdimersshouldpromptimmediate
considerationofdiffuseintravascularcoagulation(DIC)orMAS.(See"Systemicjuvenileidiopathicarthritis:
Course,prognosis,andcomplications",sectionon'Macrophageactivationsyndrome'.)
TheurinalysisistypicallynormalinchildrenwithsJIA.Somechildrenmanifestlowgradeproteinuriawhen
febrile,butsignificantproteinuriaorhematuriaarealmostneverseeninpatientswithsJIA.Secondary
amyloidosisresultinginproteinuriathatcanreachthenephroticrangehasbeenreportedintheEuropean
literature,butisexceedinglyrare[16].Thus,thepresenceofhematuriaorsignificantproteinuriain
combinationwithfever,arthritis,andrashshouldpromptconsiderationofanotherdiagnosis,suchas
systemiclupuserythematosusorasystemicvasculitisratherthanjuvenileidiopathicarthritis(JIA).(See
"Systemiclupuserythematosus(SLE)inchildren:Clinicalmanifestationsanddiagnosis"and"Vasculitisin
children:Classificationandincidence"and"Vasculitisinchildren:Evaluation"and"Vasculitisinchildren:
Managementoverview".)
Antinuclearantibodies(ANA)andrheumatoidfactor(RF)rarelyareseeninsJIA.Theirpresenceshould
promptconsiderationofalternativediagnoses.ApositiveRF,forexample,maybeassociatedwithRF
positivepolyarticularJIA,Sjgren'ssyndrome,ortheearlyonsetofadulttyperheumatoidarthritis(RA)in
teenagers.BothRFandANAmaybepresentinchildrenwithmixedconnectivetissuedisease(MCTD).
However,theseconditionsrarelymimicsJIA.(See"Polyarticularjuvenileidiopathicarthritis:Clinical
manifestationsanddiagnosis"and"DiagnosisandclassificationofSjgren'ssyndrome"and"Clinical
manifestationsofrheumatoidarthritis".)
DIAGNOSISSystemicjuvenileidiopathicarthritis(sJIA)isadiagnosisofexclusion.ThehallmarkofsJIAis
thecombinationofintermittentbutdailyfeversgreaterthan38.5C(quotidianfeverpattern)andarthritis[1].The
fevermustbepresentforatleasttwoweeksandthearthritisforatleastsixweeksinordertomakeadefinitive
diagnosisaccordingtotheInternationalLeagueofAssociationsforRheumatology(ILAR)criteria,sincemanyviral
andotherpostinfectiousformsofarthritisareassociatedwithfever,asaremalignancies.However,these
diseasesoftenlackthecharacteristicintermittentspikingfeverpatternofsJIA.(See"Specificvirusesthatcause
arthritis".)
Theremaybeaprolongeddelaybetweentheonsetoffeverandthedevelopmentofarthritis,butadefinitive
diagnosisofsJIAcannotbemadeuntilarthritisispresentandisshowntobepersistent[4].Inthepresenceofa
characteristicfeverpattern,rash,andarthritis,atentativediagnosisofsJIAcanbemadeandappropriatetherapy
begunbeforetwoweekshaveelapsed[17].(See"Systemicjuvenileidiopathicarthritis:Treatment".)
Bydefinition,sJIAcannotbediagnosedifsymptomsoccuraftertheageof16years.Asimilarandprobably
identicalillnesshasbeendescribedinadultsandiscalledadultonsetStill'sdisease[18].Recurrentdiseaseafter
theageof16isnotadultonsetStill'sdisease,butsimplyacontinuationofsJIA.(See"Clinicalmanifestationsand
diagnosisofadultStill'sdisease".)
DIFFERENTIALDIAGNOSISSystemicjuvenileidiopathicarthritis(sJIA)canbeconfusedwithanumberof
otherdisorders,includingviralorpostinfectiousarthritis,otherrheumaticorautoinflammatorydiseases,
malignancy,ormalaria.
ViralandpostinfectiousarthritisPostinfectiousandviralarthritisoftenpresentwithfeverthatisnot
intermittent,rashthatdiffersinappearancefromthatofsJIA,andtransientarthritis.ParvovirusB19isoneofthe
mostcommonlyconfusedviralinfectionsthatcausearthritis[19].Themistakendiagnosisoftheseselflimited
conditionsassJIAisinlargepartresponsiblefortheincorrectnotionthatthelatterdisorderoftenresolves
completelywithoutaggressivetherapy.(See"Pathogenesisanddiagnosisofviralarthritis".)
Reactivearthritismayalsofollowavarietyofbacterialinfections.Thosetypicallyassociatedwithfever,rash,and
arthritisincludeneisserialandstreptococcalinfections.Subacutebacterialendocarditismaypresentwiththese
symptomsandmaybeassociatedwithapositivetestforrheumatoidfactor(RF).Theseentitiescanusuallybe
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differentiatedbytheirclinicalpicture,appropriatecultures,andtheresponsetoantibiotics.Reactivearthritis
followingentericinfectionmaybealsomistakenlydiagnosedassJIA.Thekeydifferentialfeaturespointingaway
fromadiagnosisofsJIAincludegastrointestinalcomplaints,ocularfindings,andlackoftheclassicsalmonpink
rash.(See"Clinicalmanifestationsanddiagnosisofacuterheumaticfever"and"Reactivearthritis"and"Clinical
manifestationsanddiagnosisofinfectiveendocarditis".)
OtherautoimmuneandautoinflammatorydiseasesArthritiswithfeverandrashmaybethepresenting
manifestationsinsystemiclupuserythematosus,polyarteritisnodosa,mixedconnectivetissuedisease(MCTD),
dermatomyositis,sarcoidosis,neonatalonsetmultisysteminflammatorydisease,andotherautoinflammatory
diseases.Theseillnessescanbeexcludedbytheclinicalpictureandappropriatelaboratoryevaluations.Asan
example,abdominalpainiscommoninpolyarteritisnodosaandshouldpromptconsiderationofthisillnessonce
othercauseshavebeenexcluded.ChronicabdominalpaininachildwithsuspectedsJIAshouldnotbeascribed
tobenignconditions,suchas"mesentericadenitis,"withoutthoroughinvestigationtoexcludeotherpossible
diagnoses,especiallyvasculitisandinflammatoryboweldisease.(See"Systemiclupuserythematosus(SLE)in
children:Clinicalmanifestationsanddiagnosis"and"Vasculitisinchildren:Classificationandincidence",section
on'Polyarteritisnodosa'and"Pathogenesisandclinicalmanifestationsofjuveniledermatomyositisand
polymyositis"and"Extrapulmonarymanifestationsofsarcoidosis",sectionon'Musculoskeletal'and"Cryopyrin
associatedperiodicsyndromesandrelateddisorders"and"Periodicfeversyndromesandotherautoinflammatory
diseases:Anoverview".)
MalignancyEverypediatricrheumatologycenterreportsthereferralofchildrenthoughttohavesJIAwhowere
foundtohaveleukemia,lymphoma,orothermalignancies.Clinicianawarenessisthekeytoproperdiagnosis.
sJIAisnotassociatedwiththrombocytopenia,lymphocytosis,neutropenia,orbonepain.ChildrenwithsJIAare
uncomfortableandmayhaveswollenjoints,buttheyrarelycryoutfrompainevenwhentheirjointsare
manipulated[20].Bycomparison,childrenwithleukemiaorothermalignanciesmaybeexquisitelyuncomfortable
andoftencomplainofpainwhentheshaftofalongboneiscompressedawayfromanyjoints(eg,midhumerusor
midfemur).Thepaininleukemiacanchangeandbefleeting,asopposedtothepainofchronicarthritis,which
mayinvolveseveraljoints,butusuallydoesnotmovefromplacetoplace.Refusaltoweightbearisanothersign
ofbonepainthatmaybeindicativeofamalignancy.(See"Evaluationofthechildwithjointpainorswelling"and
"Clinicalassessmentofthechildwithsuspectedcancer".)
MalariaMalariamayhaveadiurnalfeverpatternidenticaltothatofsJIAandisalsoassociatedwithchills,joint
pains,andevidenceofsystemicillness.Malariaisextremelyrareinnonendemiccountries,butappropriatethick
bloodsmearstoexcludethisdiagnosisshouldbeperformedifthepatientgivesahistoryofresidenceinortravel
tosuchareas.(See"Clinicalmanifestationsofmalaria".)
OtherMarrowinfiltrativediseases,suchasLangerhanscellhistiocytosis,andinheritedconditions,suchas
GaucherdiseaseandHunterandHurlersyndromes,alsomaypresentwithfever,jointpain,andrash.However,
noneoftheseillnessesmanifestthetypicallaboratoryfindings,thefeverpattern,orthesalmonpinkrashofsJIA.
(See"Clinicalmanifestations,pathologicfeatures,anddiagnosisofLangerhanscellhistiocytosis"and"Gaucher
disease:Pathogenesis,clinicalmanifestations,anddiagnosis"and"Mucopolysaccharidoses:Clinicalfeaturesand
diagnosis",sectionon'Hurlersyndrome'and"Mucopolysaccharidoses:Clinicalfeaturesanddiagnosis",section
on'MPStypeII(Huntersyndrome)'.)
SUMMARY
Systemicjuvenileidiopathicarthritis(sJIA,formerlycalledStill'sdiseaseorsystemicjuvenilerheumatoid
arthritis)isofficiallyasubsetofjuvenileidiopathicarthritis(JIA)thatdescribespatientswithintermittent
fever,rash,andarthritis.However,sJIAisprobablyanautoinflammatorydisorderratherthananautoimmune
disorder.(See'Introduction'aboveand'Overview'above.)
sJIAisthemostdifficultformofJIAtodiagnosisbecausetherearenospecificdiagnostictests,and
arthritis,whichisnecessaryfordefinitediagnosis,isoftennotevidentearlyinthecourseofthedisease.In
addition,infectionandmalignanciesmustbeconsideredpriortothediagnosisofsJIAinthesecases,
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becausepatientscaninitiallyappearquiteillwithhighspikingfever,rash,andoftenhave
hepatosplenomegalyorlymphadenopathy,andelevatedwhitebloodcell(WBC)countspriortothe
presentationofarthritis.(See'Overview'aboveand'Clinicalmanifestations'aboveand'Laboratoryfindings'
aboveand'Diagnosis'aboveand'Differentialdiagnosis'above.)
Thediagnosisismadeclinicallyandisbaseduponthepresenceofintermittentdailyhighspikingfevers
(quotidianfever)foratleasttwoweeksandarthritis.Therearenolaboratoryfindingsthatarespecificforthe
diagnosisofsJIA,butgranulocytepredominantleukocytosis,elevatedacutephasereactants,and
hyperferritinemiashouldraisesuspicionforthisdisorder.Asalmonpinkrashisalsosuggestiveofthe
diagnosis,particularlywhenotherclinicalfeaturesarepresent.(See'Diagnosis'aboveand'Laboratory
findings'aboveand'Clinicalmanifestations'above.)
Thedifferentialdiagnosisincludesarthritisassociatedwithinfections,otherautoimmuneand
autoinflammatorydisorders,malignancy,andmalaria.(See'Differentialdiagnosis'above.)
ACKNOWLEDGMENTTheeditorialstaffatUpToDatewouldliketoacknowledgeThomasJALehman,MD,
whocontributedtoanearlierversionofthistopicreview.
UseofUpToDateissubjecttotheSubscriptionandLicenseAgreement.
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4. BehrensEM,BeukelmanT,GalloL,etal.Evaluationofthepresentationofsystemiconsetjuvenile
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5. HensingerRN,DeVitoPD,RagsdaleCG.Changesinthecervicalspineinjuvenilerheumatoidarthritis.J
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inflammatorydiseaseinfiveadolescents:adultonsetstilldiseaseorsystemicjuvenileidiopathicarthritis
sinearthritis?PediatrDermatol200421:580.
7. ParvezN,CarpenterJL.CardiactamponadeinStilldisease:areviewoftheliterature.SouthMedJ2009
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idiopathicarthritisthediagnosticdilemma.IndianJPediatr201380:783.
10. KumarS,VaidyanathanB,GayathriS,RajamL.Systemiconsetjuvenileidiopathicarthritiswith
macrophageactivationsyndromemisdiagnosedasKawasakidisease:casereportandliteraturereview.
RheumatolInt201333:1065.
11. BinstadtBA,LevineJC,NigrovicPA,etal.Coronaryarterydilationamongpatientspresentingwith
systemiconsetjuvenileidiopathicarthritis.Pediatrics2005116:e89.
12. HarveyAR,PippardMJ,AnsellBM.Microcyticanaemiainjuvenilechronicarthritis.ScandJRheumatol
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13. MartiniA,RavelliA,DiFucciaG,etal.Intravenousirontherapyforsevereanaemiainsystemiconset
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14. GorelikM,FallN,AltayeM,etal.Follistatinlikeprotein1andtheferritin/erythrocytesedimentationrateratio
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juvenileidiopathicarthritis.JRheumatol201340:1191.
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Topic6413Version12.0
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GRAPHICS
Featuresofthreeofthemajorsubtypesofjuvenileidiopathic
arthritis(JIA)
SystemicJIA
Oligoarticular
JIA
Polyarticular
JIA
PercentofJIA
patients
10to15
50
30to40
Sex
F=M
F>M
F>M
Age
Any<17years
Peak2to3years,rare
Peaks2to5,10to14
>10
years
Joints
Anynumberandany
joint
Largejoints,butrarely
hips
Any,usually
symmetricalandrare
tostartinhips
Fever,rash,
Yes
No
No
Rare
20%,mostcommon
inpatientswhoare
Lessfrequentlyseen
thaninoligoarticular
ANApositive
JIA
lymphadenopathy,
hepatosplenomegaly
Uveitis
Laboratoryabnormalities
Leukocytosis
Marked
No
No
Anemia
Marked
No
Mild
ElevatedESR
Marked
Mild
Mild
ANA
Absent
Lowtitercommon
Lowtitercommonin
younger
Rheumatoidfactor
Rare
Absent
10to20%inthose>10
years
Elevatedferritin
Marked
No
Mild
Destructivearthritis
>50%
Rare
>50%
Diseasemodifying
Commonlyused
Rarelyused
Commonlyused
andbiologicdrugs
F:femaleM:maleANA:antinuclearantibodyESR:erythrocytesedimentationrate.
Graphic56224Version11.0
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Cervicalspinefusioninsystemicjuvenile
idiopathicarthritis
Fusionofthecervicalspinemayaffectupto50percentofchildren
withsystemicjuvenileidiopathicarthritisinthefirstdecadeoflife.
Apophysealjointspacenarrowingandbonyankylosisoccur,
especiallyatC2C3.Thesechangesarenotusuallypresentelsewhere
inthespine.
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Systemicjuvenileidiopathicarthritisrash
Asalmonpinkrashischaracteristicofthisjuvenileidiopathicarthritis
(JIA)subtype.Therashisbroughtoutbyheatandoftencanbefound
intheaxillaeandaroundthewaist,butmaybepresentanywhereon
thebody.
CourtesyofRobertSundel,MD.
http://www.uptodate.com/contents/systemicjuvenileidiopathicarthritisclinicalmanifestationsanddiagnosis?topicKey=ALLRG%2F6413&elapsedTimeMs
10/12
20/10/2015
SystemicjuvenileidiopathicarthritisrashwithKoebner
phenomenon
Systemicjuvenileidiopathicarthritisrashonapatient'sbackisshowninPanelA,witha
closeupviewinPanelB.Therashusuallyconsistsofmultipleroundtooval,salmon
pinkmaculesthatareslightlyraisedandareofdifferingsizes.Therashcanappear
followingstrokingoftheskinorotherminortrauma(Koebnerphenomenon).
CourtesyofYukikoKimura,MD.
11/12
20/10/2015
Disclosures
Disclosures:YukikoKimura,MDGrant/Research/ClinicalTrialSupport:Novartis[SystemicJIA(Canakinumab)].Consultant/Advisory
Boards:Novartis[SystemicJIA,polyarticularJIA(Canakinumab)]SOBI[SystemicJIA,polyarticularJIA(Anakinra)]Regeneron
[SystemicJIA,polyarticularJIA(Sarilimumab)].MarisaKleinGitelman,MD,MPHNothingtodisclose.ElizabethTePas,MD,MS
Nothingtodisclose.
Contributordisclosuresarereviewedforconflictsofinterestbytheeditorialgroup.Whenfound,theseareaddressedbyvettingthrougha
multilevelreviewprocess,andthroughrequirementsforreferencestobeprovidedtosupportthecontent.Appropriatelyreferenced
contentisrequiredofallauthorsandmustconformtoUpToDatestandardsofevidence.
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