Rev. Bras. Reumatol. vol.55 número4

r e v b r a s r e u m a t o l . 2015;55(4):384–386

w w w . r e u m a t o l o g i a . c o m . b r

REVISTA

BRASILEIRA

DE

REUMATOLOGIA

Case

report

Chorea:

A

rare

manifestation

of

Takayasu’s

arteritis

Anandreia

Simões

Lopes

_,

_Gleice

_Clemente

_,

_Claudio

_Arnaldo

_Len

_,

Marcelo

Rodrigues

Masruha

_,

_Maria

_Teresa

_Terreri

a_{DivisionofPediatricRheumatology,DepartmentofPediatrics,UniversidadeFederaldeSãoPaulo,SãoPaulo,SP,Brazil}

b_{DivisionofPediatricNeurology,DepartmentofNeurologyandNeurosurgery,UniversidadeFederaldeSãoPaulo,SãoPaulo,SP,Brazil}

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Articlehistory: Received4April2013 Accepted16September2013 Availableonline26November2014

Keywords: Vasculitis

Takayasu’sarteritis Chorea

Neurologicsymptoms Child

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ThecaseofagirlwithrecurringchoreaandaTakayasu’sarteritisdiagnosisisreported. Thisclinicalmanifestationhasbeenreportedinonlyonepatientwiththisvasculitisinthe pediatricgroup.

©2013ElsevierEditoraLtda.Allrightsreserved.

Coreia:

uma

manifestac¸ão

rara

da

arterite

de

Takayasu

Palavras-chave: Vasculite

ArteritedeTakayasu Coreia

Sintomasneurológicos Crianc¸a

r

e

s

u

m

o

Relatamosum casode umameninacomcoreiarecorrenteediagnóstico dearteritede Takayasu.Estamanifestac¸ãoclínicafoirelatadaemapenasumpacientecomtalvasculite nafaixaetáriapediátrica.

©2013ElsevierEditoraLtda.Todososdireitosreservados.

Introduction

Takayasu’s arteritis (TA) is a chronicinflammatory disease withanunknownetiology,affectingtheaortaanditsmajor

∗ _{Correspondingauthor.}

E-mail:teterreri@terra.com.br(M.T.Terreri).

branches,aswellasthepulmonaryartery.1_{Itisararedisease,}

especiallyinchildhood,withpediatricpatientscorresponding to20%ofcases.2 _{Inthepediatricpopulation,symptomsare}

insidiousandsystemicmanifestations,suchasfever,weight loss,andmusculoskeletalsymptomspredominateearlyinthe

http://dx.doi.org/10.1016/j.rbre.2013.09.003

rev bras reumatol.2015;55(4):384–386

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disease,whichcontributestoalongertimeelapsedbeforethe diagnosisismadeinyoungerpatients.3,4

Hypertensionisthemainclinicalfindingofthediseasein childhoodand other findings includebruits,diminishedor absentpulses, difference ofblood pressure between limbs, heart failure, and claudication.4,5 _{Central nervous system}

(CNS)manifestationsarecommoninthepediatricpopulation andmightincludeheadaches,dizziness,syncope,visualloss, seizures,andstroke.2,3,6

Many studies highlight an association of chorea with autoimmune rheumatic diseases, such as rheumatic fever, systemic lupus erythematosus, and antiphospho-lipid syndrome.7–10 _{Reports on the association of chorea}

withBehcet’sdiseaseandChurg-Strausssyndromehavealso beenfound.11,12 _{However,onlyonecaseofchoreahasbeen}

describedinachildwithTakayasu’sarteritis.13

Asthis isararedisease and onlyonecaseofchoreais reportedinpediatricpatientswiththisvasculitis,wedescribe thecaseofachildwithTAmanifestingchoreaintheonsetof thediseaseandoveritscourse.

Case

report

A10-year-oldgirlstartedpresentingwithadailypaininthe handsandlowerlimbssinceshewas4.Thepainwasmore oftenfelt late inthe afternoon, worse incoldclimate and whenexercisingand wasimprovedwithrest.Swellingand feverwerenotpresent.Twoyearsaftertheonsetofthe condi-tion,thepainfulsymptomsweremaintainedandthepatient startedpresentinginvoluntarymovementsontheleftsideof herbody,which was worsewith stressand improved dur-ingsleep.Choreawasdiagnosedanditscauseremained to bedeterminedbytheneurologist,who prescribed haloperi-dol(0.5mg/day),withimprovementoftheconditionafterthe medicationwasusedfor1week.Brainmagneticresonance imagingwasnormalandtheEEGshowedaposteriortemporal andrightoccipitalparoxysmalactivity.

Thepatientremainedasymptomaticfor2yearsandthena dailyheadache and hypertensionwere foundandthus she was referred to a cardiologist. A difference in blood pres-surebetweenthearmswaspresent,bloodpressurewasnot observableinbothlowerlimbs,inadditiontoabsentfemoral, popliteal,pedal,andposteriortibialpulses.Sheunderwent anangiographywhichshowedseverecoarctationofthe des-cendingaorta.Theechocardiographyshowedamildtricuspid regurgitation.Captoprilandpropranololwereprescribedand the patient was referred to the department of pediatric rheumatology with an initial diagnosis of rheumatic fever orTA.Atthefirstvisittothepediatricrheumatologyclinic, laboratoryanalysesshowedslightelevationininflammatory activitytests(erythrocytesedimentationrate,andC-reactive protein)andabsenceofantinuclearantibody,anti-DNA, anti-bodiesto extractablenuclear antigens, anticardiolipin,and antineutrophil cytoplasmic antibodies. TA was diagnosed andmethotrexate0.6mg/kg/week,folicacid,amlodipine,and aspirinatantiaggregantdosagewereintroduced.Shereceived 3 methylprednisolone IV pulses (30mg/kg) and captopril, propranolol,and haloperidol were maintained. One month later, chorea recurred and then a penicillin G benzathine

prophylaxis (1.2 milion units IMq3wk) was initiated. Six monthslater,anonsetofrightankle,knee,andwristarthritis, claudication,andheadachewasobserved.Atthattime,arenal Dopplerultrasoundshowed>50%stenosisintheabdominal aortaabovethekidneysanda<50%stenosisbelowtherenal arteriesemergence.Magneticresonanceangiogramshowed infrarenal aorta and common iliac artery narrowing. Due to the major vascular impairment,cyclophosphamide was addedintothepulsemethylprednisolonetherapy.Onemonth later,haloperidolwaswithdrawnbecauseoftheabsenceof neurologicalsymptoms(thetreatmenthadbeenusedfortwo andahalfyears).Atthefourthcycleofcyclophosphamide,3 monthsafterhaloperidolhadbeenwithdrawn,chorea reap-pearedforthethirdtimeandhaloperidolwasreintroduced (1mg/day).

Thepatient currentlymaintainsavisual blurring, ankle jointpain,long-distanceclaudication,anddyspneaon exer-tion.Bloodpressureiscontrolled(bythreeantihypertensive drugs)and the patienthad aslightimprovementin lower-limbpulse amplitude.Following an8-month treatmentfor chorea, the neurologist stopped the haloperidol and the patienthashadnochoreafor1yearsincethelastrecurrence. Inflammatory tests were normal. A new echocardiogram showed segmental coarctation ofthe low aorticarch with hemodynamicrepercussionandmildconcentricleft ventri-clehypertrophy.Followingsevencyclophosphamide(1g/m2₎

infusions,thepatientmaintainedthecomplaintsandabrain magneticresonance angiogram showedan internalcarotid artery stenosis. As a result,we decided tostart infliximab (5mg/kg).Asthechoreawassuggestedtobeassociatedwith Takayasu’s,wechosetostopthepenicillinGbenzathine pro-phylaxis.Thepatientiscurrentlyonmethotrexate,folicacid, amlodipine, captopril, propranolol, aspirin, ranitidine, and infliximab.Shehasbeenclinicallybetteroninfliximabfor8 months,stillpresentingdyspneaonexertion,butthe arthral-giaandthevisualblurringhaveimproved.

Discussion

Wehavereportedthecaseofa10-year-oldgirlwithrecurring episodesofchoreaassociatedwithhypertension,diminished peripheralpulses,andanangiogramconsistentwithTA.Other rheumaticandnonrheumaticcausesofchoreawereruledout. The patient met the validated classification criteria for childhoodTA,14 _{whichincludedangiographic abnormalities}

associatedwithhypertension,adifferenceinbloodpressure betweenthelimbs,diminishedperipheralpulses,and claudi-cation.ThepenicillinGbenzathineprophylaxiswaschosenat firstbecauserheumaticfeverisstillthemostcommoncause ofchoreainchildhood,15_{althoughthepatientdidnotmeetthe}

criteriaforthedisease.Theyoungesthadtwomoreepisodes ofchoreainevolution,andoneoftherecurrencescameoff whenthegirlwasinuseofhaloperidol,andtheotherwhenin secondaryprophylaxiswithpenicillinbenzathine,with ques-tioningofthediagnosisofrheumaticfever.

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abnormalfindings,whichruledotherneurologicalcausesout. Thyrotoxicosis,alsoacauseforchorea,wasruledout. Nega-tiveantinuclearantibodiesand antiphospholipidantibodies ruled out the diagnosis of systemic lupus erythematosus and the antiphospholipid syndrome as possible causes of chorea.Thepatientdidnothaveclinicalmanifestations con-sistent with other primary vasculitides, such as Behcet’s diseaseorpolyarteritisnodosa.Themostimportant differen-tialdiagnosiswasrheumaticfever,butthisassumptionwas unlikelybecauseoftheabsenceofothermajorcriteria,such ascarditisorarthritisearlyinthecourse,recurrencewhile on penicillin G benzathine prophylaxis and improvement from continuous immunosuppressive treatment. Constitu-tionalsymptomsandacutephasenonspecificsignsmaybe common to both rheumatic fever and Takayasu’s arteritis, howevermoreunique findings,suchasdiminished periph-eralpulses,hypertensionandadifferenceinBPbetweenthe arms,inadditiontoimagingshowinginvolvementoftheaorta anditsbranches,ledtothediagnosisofTakayasu’sarteritis. Inaddition,thepatienthasbeenoutofstreptococcal infec-tionprophylaxisfor18monthsandnootherchoreaepisodes occurred.

IntheBrazilianmulticenterstudyof71childrenand ado-lescents with Takayasu arteritis published this year, more than70%ofpatientshadneurologicalsymptomsearlyinthe disease.16 _{These symptoms wererepresented byheadache,}

convulsions,faintingandstroke.Therewasnodescriptionof chorea,exceptinthepatientreportedinthisarticle.

ThecaseofachildwithTApresentingasahemichoreahas beenrecentlypublishedinaSpanishreport.13_Yet,noother

reportswerepublished.

Antibodiestobasalganglia,inadditiontothepresenceof Tlymphocytes,leadingtoanalteredimmunecellresponse, wouldpossiblyexplaintheoccurrenceofthisrare manifes-tation.Furthermore,theinvolvementofintracranialvessels couldleadtohypoperfusionofbrainareas,especiallythebasal ganglia,andchoreaemergence.

Throughthiscase,wewanttodrawattentiontoarareTA manifestation.Thephysicianshouldbealerttoatypical man-ifestationsofthediseasesothatdiagnosisandtreatmentare promptlyaccomplished.

Conflicts

of

interest

Theauthorsstatenoconflictsofinterest.

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