Rev. Bras. Reumatol. vol.57 número6

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r e v b r a s r e u m a t o l . 2017;57(6):626–629

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

REVISTA

BRASILEIRA

DE

REUMATOLOGIA

Case

report

Initial

isolated

Takayasu’s

arteritis

of

bilateral

pulmonary

artery

branches

Arterite

de

Takayasu

com

acometimento

inicial

isolado

de

ramos

bilaterais

da

artéria

pulmonar

Yu-Hui

Zhang

a

_,

_Wei-Min

_Song

a

_,

_Mei

_Wu

a

_,

_Jing

_Zhu

b,∗

a_People’s_Hospital_of_Bozhou,_Department_of_{Rheumatology,}_Bozhou,_Anhui,_China

b_Sichuan_Provincial_People’s_Hospital,_Department_of_{Rheumatology,}_Chengdu,_Sichuan,_China

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Articlehistory: Received3April2015 Accepted13October2015 Availableonline11March2016

Introduction

Takayasu’s arteritis chiefly affects the aorta and its major branches,includingthepulmonaryartery.Clinical presenta-tions ofTAare proteanand notspecific, the vastmajority ofpatientspresentedvascularinsufficiencysuchasstenosis, occlusion,oraneurysm.SuchfeaturesconfuseTAwiththose ofchronicthromboembolic diseases,fibrosingmediastinitis orneoplasia,resultinginanerroneousinitialdiagnosis.1_The mediandelayofthediagnosisofdiseasewas10monthsafter the onset of first symptoms.2 _Severe _{hypertension,} _severe functional disability, and evidence of cardiac involvement were good predictors for either death or major event on follow-up,whichhelptheprognosisassessmentandelective interventions.3

Case

report

An18-year-oldyellowAsianfemalepatienthadbeen suffer-ingfrom fatigueoneffort,cheststuffiness,chest pain,and

∗ _{Corresponding}_author.

E-mail:[email protected](J.Zhu).

dyspneafor8months.Twomonthsago,shewasadmittedto thedepartmentofcardiologyinourhospital.Bloodpressure was126/83mmHgwithoutdifferencebetweenbilateralarms. Theechocardiography(ECHO) showedexcessivestenosisof bothbranchesofpulmonaryartery,theinitialportionofthe rightbranchwithalumenof14mmandtheleft16mm,and asignificantlyhighpulmonaryarterypressureof118mmHg (Fig.1A–B).Dualsourcecomputedtomography(DSCT)verified aconsiderablepartialstenosisofbothbranchesofpulmonary arteryandnoevidenceofaortainvolvement(Fig.1C–D).All theclinicalmanifestationsofthispatientdemonstrated criti-calpulmonaryarteryhypertensionwithstenosisfollowedby left heart involvement.Therefore, she was diagnosedwith congenitalheartdiseasefromthesefindings.

Onemonthago,tricuspidvalveannuloplastyandbranch pulmonaryarteriesangioplastyweregiveninanotherhospital duetoexacerbatedsymptoms.Laboratorytestspostoperative pointedoutimmunologicalaberration.Erythrocyte sedimen-tationrateis89.0mm/h,andrheumatoidfactoris9.1IU/mL.

Subsequently the patient was admitted to the depart-mentofrheumatologyofourhospital.DiagnosisofTAwas

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Fig.1–(A–B)Echocardiography6monthsaftersymptomonset.Itshowedcolorfulbloodflowinleftbranchofpulmonary arteryduetothestenoticlesion(A)andremarkedtricuspidregurgitation(B).(C–D)Dualsourcecomputedtomographyof pulmonaryartery4monthsaftersymptomonset.Itrevealedtheconsiderablystenosisofbothbranchesofpulmonary artery(arrows)andnoevidenceofaortainvolvement.LV,leftventricle;LA,leftatrium;RV,rightventricle;RA,rightatrium; AO,aorta;PA,pulmonaryartery;LPA,leftbranchofpulmonaryartery;RPA,rightbranchofpulmonaryartery;RVOT,right ventricularoutflowtract.

suspected, and as such a complete aortogram was made butprovedtobenormal.RepeatedECHOshowedreduction of tricuspid regurgitation and pulmonary artery pressure, andanormalaorticarchanditsbranches(bilateralinternal andexternalcarotidartery,and bilateralsubclavianartery). Digital subtraction angiography (DSA) findings referred to no vascular lesions of the aorta and branches, presented as stenosis, narrowing, occlusion and irregularity of wall (Fig.2A).Anti-nuclearantibody(ANA)waspositiveinatiter of1:40,andperinuclearanti-neutrophilcytoplasmicantibody (p-ANCA)positivein1:10.Onadmission,shewasdiagnosedas TA.Prednisone30mg/dayandaspirin50mg/daywereadded. Six months later, the patient suffered from pulmonary tuberculosis. Treatment with isoniazid, rifampicin, etham-butol and pyrazinamidefor 3months wasineffective, and then turned to 4-aminosalicylic acid, protionamide, mox-ifloxacin and clarithromycin intermittent for 15 months, eventually achieving relief. Prednisone administration per-sistedthroughoutthe procedure.During thefollow-up, she developedgreatdepressionandwassuccessfullyrescuedafter taking20mgdigoxin onceherself. Forty-twomonths later, the patient returned again, beingattacked by cervicodynia anddizzinessfor4months.Computed tomography angiog-raphy (CTA) revealed a considerable stenosis of the right

brachiocephalictrunk,leftcommoncarotidarteryand subcla-vianartery(Fig.2B).Consultationfordepartmentof cardiovas-cularsurgerywasrequiredandangioplastywillbeundergone.

Discussion

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Fig.2–(A)Digitalsubtractionangiographyoftheaortaandbranchesaftertricuspidvalveannuloplasty.Imagesofthoracic aorta,abdominalaortaandiliacarterywereintegratedintoonefigure.Itreferredtonovascularlesionsthatinvolvedthe aortaandbranches.(B)Computedtomographyarteriogramsoftheaortaandbranches42monthsafterTAdiagnosis. Considerablyremarkablestenosisoftherightbrachiocephalictrunk(largearrow),leftcommoncarotidartery(smallarrow) andsubclavianarterywasnoted.

isolatedpresentation isseldomseen.Oneliteraturereview performedintheMedline/PubMeddatabasefrom1975to2009 eventuallyfound14patientswithisolatedpulmonary vasculi-tisand8patientswithpulmonaryinvolvementwastheinitial presentation.6 _This _patient _experienced_other _new _arteries involvementoftherightbrachiocephalictrunk,leftcommon carotidarteryandsubclavianartery,50monthsafterthefirst presentingmanifestations.

AtypicalpresentationsofTAwithpulmonarysymptoms contributetoanincorrectinitialdiagnosis.Clinical manifes-tationsofsystemicarteryinvolvementappearedafterseveral years,justasthiscasewereportthatvasculitis of brachio-cephalictrunk,commoncarotidarteryandsubclavianartery appeared3yearsafterthefirstpresentation,resultinginan erroneous initial diagnosis. Thepulmonary artery involve-ment confused TA with those of chronic thromboembolic diseasesandpulmonarydiseasesleadingtoamisdiagnosis.7 Diseasesofpulmonaryvasculitisareclassifiedintothree cat-egoriesaccordingtoimagingfindings,localizednodularand patchyopacities (angiitis granulomatosisgroup),diffuseair spaceconsolidation(diffuse pulmonaryhemorrhage dueto capillaritis),andaneurysmorstenosisofthelargepulmonary arteriessuchasTakayasu’sarteritis,Behcet’sdisease.8_Despite theinitialpresentationsofthispatientarenotspecific,ina youngfemalepresentingwithaclinicalpictureofacute pul-monaryembolismandanelevatederythrocytesedimentation rate(ESR)andCRP,withnoriskfactorsforthromboembolic

diseaseandnoevidenceofothersystemicvasculitidesoflarge vessels, TA must beconsidered in the differential diagno-sis.Theeffectivemethodtoanearlyandaccuratediagnosis should be consideringTA inthe differential diagnosis and activescreening.PatientsofTAwithoutspecificsymptomsof vasculitisshowedinflammatoryactivityinthevesselwallsof theaortaforPET-CTwith(18)F-FDG.9

Althoughclinicallysignificantpalliationusuallyoccurred after angioplasty or bypass of severely stenotic vessels, restenosiswascommon.2,10,11_Whereas,_treatment_of tricus-pidvalveannuloplastyandballoondilatationinthispatient pulseprolongedglucocorticoidadministrationhavepalliated both the pulmonary and cardiac conditions and stenotic bilateralpulmonaryarteryduring3-yearfollow-up,although stenosis of the right brachiocephalic trunk, left common carotidarteryandsubclavianarteryoccurred.Arteries recon-structedaftersurgicalbypassmayhavesuperiorpatencyto those reconstructed by endovascular treatment.12 _Biologic therapysuchasanti-TNFandanti-IL-6receptoragentsmay beausefuladjuncttosteroids.13–16

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superantigensandtheelevatedproinflammatorycytokines.19 FurtherstudyoftherelationshipbetweenTAandtuberculosis mayshowtheexactroleofM.tuberculosisinthepathogenesis ofTakayasu’sarteritis.

Conflicts

of

interest

Theauthorsdeclarenoconflictsofinterest.

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