r e v b r a s r e u m a t o l . 2016;56(1):90–92
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
Difficulties
in
the
differential
diagnosis
between
Takayasu
arteritis
and
rheumatic
fever:
case
report
夽
Taciana
Fernandes
Araújo
Ferreira
a,∗,
Marlene
Freire
b,
Reginaldo
Botelho
Teodoro
baUniversidadeFederaldoTriânguloMineiro(UFTM),Uberaba,MG,Brazil
bDisciplineofRheumatology,UniversidadeFederaldoTriânguloMineiro(UFTM),Uberaba,MG,Brazil
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o
Articlehistory:
Received8August2014 Accepted1December2014 Availableonline20November2015
Keywords: Takayasuarteritis Rheumaticfever Valvedisease Heartfailure
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Inthisarticle,wepresentthecaseofapatientwithheartfailurewithbiologicalaorticvalve prosthesisandmultiplevascularchangesconsistentwithTakayasuarteritis(TA)whowas seeninourdepartmentreceivingcorticosteroidsandsecondarypreventionofrheumatic fever(RF);itwasnotpossibletoexcludetheassociationbetweenbothdiseases.
©2015ElsevierEditoraLtda.Allrightsreserved.
Dificuldades
no
diagnóstico
diferencial
entre
arterite
de
Takayasu
e
febre
reumática:
relato
de
caso
Palavras-chave: ArteritedeTakayasu Febrereumática Valvopatia
Insuficiênciacardíaca
r
e
s
u
m
o
Apresentamosocasodeumapacienteportadoradeinsuficiênciacardíacacomprótese valvaraórticabiológicaealterac¸õesvascularescompatíveiscomarteritedeTakayasu(AT) quechegouaoservic¸oemusodecorticoideseemprofilaxiaparafebrereumática(FR).Não foipossívelafastaraassociac¸ãoentreambasasenfermidades.
©2015ElsevierEditoraLtda.Todososdireitosreservados.
夽
StudyconductedatDepartmentofInternalMedicine,DisciplineofRheumatology,UniversidadeFederaldoTriânguloMineiro(UFTM), Uberaba,MG,Brazil.
∗ Correspondingauthor.
E-mail:[email protected](T.F.A.Ferreira). http://dx.doi.org/10.1016/j.rbre.2015.07.001
rev bras reumatol.2016;56(1):90–92
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Introduction
Takayasuarteritis(TA)isalarge-vesselvasculitisofunknown etiology,affectingtheaortaanditsmainbranches.This dis-easeprimarilyaffectsfemalesaroundthethirddecadeoflife, indifferentpartsoftheworld.1Thediagnosisassociates clin-icalfindings and inflammatorylaboratory parameters with imagingstudies,giventhat,todate,nospecificbiomarkers wereidentified.2 Acriticalissue concernsits pathophysiol-ogy; an association with tuberculosis has been suggested, sincebothdiseasesshowgranulomatouslesionsandpresenta similargeographicdistribution,beingmoreprevalentinAsia, AfricaandSouthAmerica.3
Rheumaticfever(RF)isaninflammatorysystemicdisease determined by animmune response toinfection bygroup A beta-hemolytic Streptococcus pyogenes in genetically pre-disposedindividuals afterinfection ofupperairways,4 and generallyaffectschildrenandyoungadultsineconomically disadvantagedpopulations.5TheJonescriteria6areusedfor diagnosis,whichmustbeestablishedearlytoensure imme-diatetreatmentinordertopreventcarditis,itsmostsevere form,whichmayfollowachroniccourseandcausesignificant morbidityandmortality.7
RF patients may suffer pancarditis, and endocardial involvementis the most severe consequence, because the valvelesionsmayprogresstopermanentdamage, determin-ingtheclinicalpictureandtheprognosisofthedisease.The mitralandaorticvalvesaremostoftenaffected,with regur-gitationintheacutephase,andstenosiswithprogressionof thedisease.7
In this paper, we present the case of a female patient admittedtotheHospitaldeClinicas,UniversidadeFederaldo TrianguloMineiro(HC-UFTM)withheartfailure(HF) decom-pensation,referringaprevious diagnosisofTAandRF and withanunsystematictreatmentforbothdiseases.
Case
report
DPS,female,37yearsold,Caucasian,wasadmittedtothe HC-UFTMinJanuary2013withadecompensatedHF.Shereported thatattheageof15startedprogressivelywithexertional dysp-nea,asthenia,generalizedarthralgia,dailyhighfever,swelling inthelowerlimbsandlargejoints,andadifficult-to-control hypertension, having been diagnosed with RF and started secondarypreventionwithbenzathinepenicillininaddition toprednisone(intravenouslyandthenmaintenancedosesof 80mg/day on average) and captopril, digoxin, furosemide, spironolactone,nifedipineandamiodarone.Attheageof21 shepresentedanewdecompensation,beingdiagnosedwith TA withaortic, renal,carotid and subclavian involvement; indicatedanon-specified,non-performedsurgicalprocedure, optingto keepthe current clinical treatment atthat time. At the age of 28, the patient was submitted to an aortic valvereplacementprocedurewithbiologicalprosthesis.She refersthat, throughoutthis period,sheremained on pred-nisone(20mg/dayonaverage),benzathinepenicillinandthe other drugs mentioned above. At her last admission, she started presenting with chest pain, palpitation, exertional
dyspnea,asthenia,fainting,lowerlimbedemaand hypoten-sion. Physical examination revealed large discrepancies of bloodpressureandpulses,asfollows:rightupperlimb:60/40; pulsespresent–leftupperlimb:bloodpressureand uniden-tifiedpulses–rightlowerlimb:180/70;pulsespresent–left lower limb:120/70, thinpulses. Shealsopresentedan aor-ticandmitralsystolicmurmur,aorticdiastolicmurmur,and bilateralcarotidandrenalbruits,especiallyontheleftside. The patient was admitted to the ICU, when clinical treat-mentwasintroducedforHF,withimprovementofherclinical picture.
The patientbrought with herseveral old imaging stud-ies; oneofthesetests wasa coronaryangiography carried out in 2003, showing carotid (both), subclavian and aorta involvement– findingssuggestive ofTAand ofsevere aor-tic regurgitation. Anechocardiogram (ECHO) carried out in 2011foundmoderatemitralregurgitationanddoublelesionin heraorticvalveprosthesis.Thepatientalsobroughtabiopsy report of her native aortic valve, to which we had access only tothis report (and notto the slide), showing“strong fibro-hyalinosis, chronic inflammation and edema focuses compatiblewithvalvedegeneration.”Ofthetestsperformed duringhospitalization,bloodcount,biochemistryandprotein electrophoresiswerenormal,andinflammatorymarkerswere alwaysnegative;ECHOconfirmedthepreviousfindingsanda progressionofHFwithanejectionfraction(EF)droppingfrom 79%to35%;thecoronaryangiographyrevealed,inaddition topreexistinglesions,anostial occlusionofright coronary artery;andthearteriographydisplayedanaortic bioprosthe-siswithdiscretefailureandanintra-prosthesissaccularimage suggestiveofpseudo-aneurysmordissection,aswellassevere bilateralinvolvementofsubclavianandcarotidvessels,with vicariousvertebralarteries.
Although the patient had an indication for surgical approach,theprocedurewascontraindicatedduetothehigh cardiovascularrisk.ShewasassessedbytheRheumatology team,whichconfirmedthediagnosisofTAassociatedornot withRF.Anti-DNaseBandinflammatorytestswereordered, allofthemwithnegativeresults.Secondaryprophylaxiswith benzathine penicillin and oral treatment with prednisone weremaintained,besidesacarefulfollow-up.
Discussion
InbothTAand RF,cardiacinvolvementisthemajor deter-minant of morbidity and mortality. The early clinical and epidemiological picture ofthis patient is common to both diseases,1 andwhileTAoccursrarely, theprevalenceofRF inourpopulationmakesitimperativethatonealwayskeeps this diagnosis in mind. In a recent study among Pediatric Rheumatologists inSão Paulo, it wasfound that RF, albeit with a progressive reduction in incidence, was diagnosed moreofteninprivatehealthcenters;ontheotherhand,TA andother vasculitideswerediagnosedmoreofteninpublic system.8
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rev bras reumatol.2016;56(1):90–92Atthe time, our patient had no evidence of inflamma-toryactivity.Theseparameterscanhelpinthediagnosisand monitoringofTA,butdonotruleoutdiseaseactivity;inthis scenario,imagingstudiesarethegoldstandard,wherebyfrank diseaseprogressionwasobserved.
Thegreatestdifficultywasinrelationtothereferred diag-nosisofRF,sincetherewasnowaytoconfirmorrefutethis hypothesisatatime ofsuchadvancedcardiac lesionsand theconcomitant pathology determinantofstructuralheart damage.Althoughunlikely,wecouldnotruleout the asso-ciationofbothdiseases.InastudybyDoietal.,apatientwith coarctationofthe aortasecondarytoTAand mitral steno-sisduetoRFwasdescribed.9 Castlemainetal.described a femalepatientwithaorticregurgitationsecondarytodilation oftheaorticarch;theseauthorsproposedthehypothesisofRF, andsubsequentlyfoundthattheirswasacaseofTA.1 Gan-gahanumaiah et al.described the caseofa femalepatient aged29withHF,historysuggestiveofRFandwithanECHO showingseveremitralregurgitation,mildaorticregurgitation, pulmonaryhypertension,withleftventricularfunction main-tained.Bothvalveswerereplaced;andsignssuggestiveofTA wereobservedinpathologicalstudies.10Ravellietal.reported thecaseofateenagerwithnonspecificsymptomsfor2years who developedaortic regurgitation; the primarydiagnostic hypothesisproposedforthisteenagerwasRF,andafterwards TAwasdiagnosedbyimagingstudies.11
ValvechangesarenotuncommoninpatientswithTA,and usuallytheseproblemsstemfromstructuralheartand vas-cularinjuries.12Insuchcases,aorticregurgitation,followed bymitralregurgitation,occursmorefrequently.13Abid-Allah etal.describedheartvalveinvolvementin4casesofTA,as follows:anisolatedmitralregurgitation; twoisolatedaortic regurgitations;twoassociationsofmitralandaortic regurgi-tation,withhypertensioninallcases.12Bradyetal.described thecaseofafemalepatientwithTAandaorticregurgitation andheartfailurewithanormalmitralvalve.14InaBrazilian seriesofpatientswithRF,carditiswasthesecondmost fre-quentmajorsign,predominantlyoccurringinfemales,and themostcommonvalvechangewasmitralregurgitation.15
Difficultiesinmeetingdiagnostic criteriainTA are con-stantlymentionedinthe literature,and this contributesto hinderthedifferentialdiagnosis.16Withregardtoourpatient, althoughweconsidermorelikelythatanychangesarereally duetoTA,inourclinicalandepidemiologicalcontext,wemust alwaysconsiderthehypothesisofRFinthedifferential diag-nosisforallpatientswithinflammatorysystemicsignsand heartmanifestations, especiallywhenthereis mitralvalve involvement.
Conflicts
of
interest
Theauthorsdeclarenoconflictsofinterest.
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