Rev. Bras. Reumatol. vol.56 número2

r e v b r a s r e u m a t o l . 2016;56(2):178–180

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

Association

between

rheumatic

fever

and

Takayasu’s

arteritis

–

Case

report

Natali

W.S.

Gormezano,

Maria

Carolina

dos

Santos

_,

_Eunice

_Mitiko

_Okuda,

Liane

Hulle

Catani,

Silvana

B.

Sacchetti

RheumatologyandCardiologyServices,DepartmentofPediatrics,IrmandadedaSantaCasadeMisericórdiadeSãoPaulo, SãoPaulo,SP,Brazil

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Articlehistory:

Received11February2014 Accepted5September2014 Availableonline20February2015

Keywords: Rheumaticfever Takayasu’sarteritis Cardiacmanifestations

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s

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t

Takayasu’sarteritis(TA)andrheumaticfeverarediseasesthatcanstartwithcardiac fea-tures,makingthediagnosisdifficult.TherearereportsofassociationofRFwithTakayasu’s arteritisbeginningwithcardiacinvolvementinpediatricpatients.Theaimofthisstudyis toreportthepossibleassociationofRFandTAinpatientswithcardiacabnormalities.We describethecaseofanadolescentinitiallydiagnosedwithRFwhoprogressedwithchanges thatallowedmakingthediagnosisofTA.TAandRF aretwoimportantcausesofvalve involvementthatmayhavesystemicmanifestations.

©2014ElsevierEditoraLtda.Allrightsreserved.

Associac¸ão

entre

febre

reumática

e

arterite

de

Takayasu

–

Relato

de

caso

Palavras-chave: Febrereumática ArteritedeTakayasu Manifestac¸õescardíacas

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e

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u

m

o

AarteritedeTakayasu(AT)eafebrereumática(FR)sãodoenc¸asquepodemteriníciocom manifestac¸ãocardíaca,oquedificultaodiagnóstico.Hárelatosdeassociac¸ãodeFRcom ATqueseiniciacomcomprometimentocardíaconafaixaetáriapediátrica.Oobjetivodeste estudoérelatarapossibilidadedaassociac¸ãodeFReATempacientecomalterac¸ãocardíaca. DescrevemosocasodeumaadolescentediagnosticadainicialmentecomoFRque apresen-tounaevoluc¸ãoalterac¸õesquepermitiramodiagnósticodeAT.AATeaFRsãoduascausas importantesdeenvolvimentovalvularquepodemapresentarmanifestac¸õessistêmicas.

©2014ElsevierEditoraLtda.Todososdireitosreservados.

∗ _{Correspondingauthor.}

E-mail:carolls@uol.com.br(M.C.Santos).

http://dx.doi.org/10.1016/j.rbre.2014.09.003

rev bras reumatol.2016;56(2):178–180

179

Introduction

Takayasu’sarteritis (TA)isachronicgranulomatous inflam-matory disease that canaffect the aorta, its branches and pulmonaryartery.1,2

Inchildren,itaffectswomenataratioof2:1,similarlyto thatfoundinaBrazilianmulticentricstudyof71childrenand adolescents(2.6:1).3_{Meanageofonsetis11.4years,with20%}

before19yearsand2%before10yearsofage.4

The initial manifestations may be insidious and non-specific, such as asthenia, headache, fever, night sweats, arthralgia,musclepainandweightloss,startingatvariable timesbeforevasculardisease,ormaybeacutewithhighblood pressure,seizuresandcongestiveheartfailure.5

Cardiac impairment occurs inabout 50% of patients in thecourseofthedisease,andanycardiacstructurecanbe affected.Whenpresentedwithvalveregurgitation,thepatient mayinitiallybediagnosedwithrheumaticfever.

WereportedacaseofTAinanadolescentwithcardiacvalve impairmentwithearlydiagnosisofrheumaticfever.

Case

report

A15-year-oldadolescent,female,white,wasadmittedwith ahistoryofpalpitationsassociatedwithadynamiaand7-kg weight loss, 10 monthsago. On physicalexamination, the patientwaspale,tachycardic,andafebrilewithbloodpressure (BP)of130×70mmHg(90-percentile).Oncardiacauscultation,

loudP2andsystolicmurmurinmitralarearadiatingtothe backpresentsymmetricalpulses.

Laboratorytestsshowedanemia, withmildleukocytosis andthrombocytosis,elevatedinflammatoryactivityand titra-tionofanti-streptolysinOantibody(ASLO)(Table1).

Chestradiographyshowedcardiacareaattheupperlimit, theechocardiogramshowedmoderateleftventricular dilata-tionwiththickeningofthemitralvalveandmitral(mild)and aortic(mildtomoderate)regurgitation,withanejection frac-tionof51%,andinflammationonmyocardiumscintigraphy. With these data, shewas diagnosedwith rheumatic fever (RF).Treatmentwithprednisone,digoxin,and hydrochloroth-iazide was initiated, with prophylaxis with benzathine penicillin,withnormalizationofinflammatoryactivity.In out-patientfollow-up,corticosteroidsweregraduallyreducedand

Table1–Progressoflaboratoryalterations.

Hospital admission

12daysafterthe

useofPRED

Postsuspension

ofPRED

18daysafter

reintroductionof

PRED

3monthsafter

PRED

6monthsafter

PRED

Current

Hemoglobin 9.04 12.8 11.6 14.6 11.5 11.5

Leucocytes 13,000 24,000 12,600 5300 19,230

Platelets 635,000 523,000 630,000 543,000 530,000 444,000

ESR/CRP 120/7.8 5 37/19.5 20/<0.3 40/4.4 26 18/<0.4

Alpha-1-acidglycoprotein 195 188 246 – 129 125 –

ASLO 1379 1560 – – 129 125 –

PPD – – – – – Negative –

Creatinine/serumurea – – – – – 0.5/14 –

discontinuedafterthreemonthsofitsinitiation,maintaining adequatesecondaryprophylaxis.

Afterdiscontinuationofcorticosteroids,sheshowed exer-tionaldyspnea,tachycardia,lowerlimbedema,auscultation ofepigastricmurmursandincreasedbloodpressure.On labo-ratoryinvestigation,inflammatoryactivitytestswerehighand PPDwasnegative.Echocardiographyrevealednormalmitral valveandmildaorticregurgitationandanejectionfraction of63%.Chesttomographydetectedparietalthickeningofthe aortaand itsbranches,and presenceofdilation (2.6cm)in thethoracoabdominaltransitionthatissuggestiveof arteri-tis. The hypothesis of TA was confirmed by the changes evidenced inarteriography. (Fig.1)Methotrexate and pred-nisonewereassociatedwithclinicalandlaboratorycontrolof inflammation.

Currently,thepatientisbeingfollowedondiagnosisofTA and RF, withprednisone, methotrexate, folic acid, digoxin, anti-hypertensives and prophylaxis with benzathine peni-cillin.

Discussion

TA is uncommon, especially in the pediatric population, althoughitisamajorcauseofrenovascularhypertension.

Genetic factors and infectious and immunological pro-cessesaredescribedinetiopathogenesis.

Inthecasedescribed,asJonescriteriaweremet,itallowed thediagnosisofRF,butthenonspecificmanifestationsthat precededFR symptomssuggest thepresenceofasystemic inflammatoryvasculitisofinsidiousonset.6_{Inthecourseof}

thedisease,thepresenceofepigastricmurmur,hypertension andabnormalangiographyallowedthediagnosisofTAbased ontherevisedcriteriaEULAR/PReS.7_{TheassociationofRFand}

TAisuncommon.8_Tejadaetal.9_{reported2casesofrheumatic}

heartdiseaseinaseriesof125patientswithTA.Atourservice, within11 casesofTA, onlythispatient hadheart changes consistentwithRF.

Cardiacinvolvementoccursinupto50%ofpatientswith TA,andmayimpairanyheartstructure.10,11_Coronary

arteri-tis,heartvalvelesion,andventricularaneurysmarethemain manifestationsreportedinchildrenwithTA.

180

Philips Ex. 11080

RECON ANGIO ABDOMINAL

RECON ANGIO ABDOMINAL

PL

P R

F

10 cm 512 X 512

Se: 10 / 13 Im: 7 / 9 Ax: 10 mm

0 mm W: 256 L: 127

Fig.1–Arteriographicalterations.

tricuspidvalvesinapproximately 20%ofTApatients, often withregurgitation,withnoevidenceofvalvethickening.11_In

autopsystudies,therearereportsofaorticvalvethickening secondarytoarteritisoftheaortaextendingtovalveandmural endocardium.12

Thepresenceofmitralvalvethickening,associatedwith streptococcus,withresolutionaftertreatmentwith cortico-steroidsdoesnotallowrulingoutTA-associatedRF.According toValeetal.13_{,thecombinationofRFandTAraisesthe}

possi-bilityofacommonimmunologicbasisinthepathogenesisof bothdisorders.

TAandRFaretwomajorcausesofvalveinvolvement,the presenceofsystemicmanifestations,echocardiographicand arteriographicfindingsarehelpfulinthediagnosisofcardiac involvement.

Conflicts

of

interest

Theauthorsdeclarenoconflictsofinterest.

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