w w w . r e u m a t o l o g i a . c o m . b r
REVISTA
BRASILEIRA
DE
REUMATOLOGIA
Original
article
Electrocardiographic
changes
in
dermatomyositis
and
polymyositis
Leticia
Miranda
Alle
Deveza,
Renata
Miossi,
Fernando
Henrique
Carlos
de
Souza,
Andrea
Yukie
Shimabuco,
Maria
Helena
Sampaio
Favarato,
José
Grindler,
Samuel
Katsuyuki
Shinjo
∗FacultyofMedicine,UniversidadedeSãoPaulo,SãoPaulo,SP,Brazil
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Articlehistory:
Received1May2014 Accepted17August2014
Availableonline27November2014
Keywords:
Heart
Dermatomyositis Electrocardiogram
Idiopathicinflammatorymyositis Polymyositis
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Introduction:Cardiacinvolvementisfrequentininflammatorymyopathies. Electrocardio-gram(ECG)mayshowevidenceofthisinvolvementanditschangesshouldbewell-known anddescribed.
Objectives: Duetothelackofstudiesintheliterature,weconductedananalysisoftheECG findings inpatientswithdermatomyositis(DM)andpolymyositis(PM),comparingthem withacontrolgroup.
Methods:Thiscross-sectionalstudycomparedtheECGof86individualswithnorheumatic disorders(controls)with112patients(78DMand34PM),during2010–2013.TheECGfindings betweenDMandPMwerealsocompared.
Results:Demographiccharacteristics, comorbiditiesandECGabnormalitiesweresimilar betweencontrolsandpatients(p>0.05),exceptforahigherfrequencyofleftventricular hypertrophy(LVH)inpatients(10.7%vs.1.2%,p=0.008).Demographiccharacteristics, comor-bidities,clinicalandlaboratorymanifestations,werealsosimilarbetweenthegroupsPMand DM,exceptforthepresenceofcutaneouslesionsonlyinDM.One-thirdofthepatientshad ECGabnormalities,whichweremoreprevalentinPMthanDM(50%vs.24.4%,p=0.008). LVH,leftatrialenlargement,rhythmandconductionabnormalitiesweremorefrequentin PMthanDM(p<0.05forall),especiallytheleftanteriorfascicularblock.
Conclusions: WeshoweddistinctECGchangesbetweenDMandPMandahigherfrequency ofLVHinpatientscomparedtocontrols.Investigationofcardiacinvolvementshouldbe consideredeveninasymptomaticpatients,especiallyPM.Furtherstudiesarenecessaryin ordertodeterminethecorrelationofECGfindingswithothercomplementarytests,clinical manifestations,diseaseactivityandprogressiontoothercardiacdiseases.
©2014ElsevierEditoraLtda.Allrightsreserved.
∗ Correspondingauthor.
E-mail:[email protected](S.K.Shinjo). http://dx.doi.org/10.1016/j.rbre.2014.08.012
Alterac¸ões
eletrocardiográficas
em
dermatomiosite
e
polimiosite
Palavras-chave:
Corac¸ão Dermatomiosite Eletrocardiograma Miopatiasinflamatórias idiopáticas
Polimiosite
r
e
s
u
m
o
Introduc¸ão: Acometimentocardíaconasmiopatiasinflamatóriaséfrequente. Eletrocardio-grama(ECG)podemostrarindíciosdesseacometimentoesuasalterac¸õesdevemserbem conhecidasedescritas.
Objetivos: Devidoàescassezdetrabalhosnaliteratura,analisamosasalterac¸õesdeECGem pacientescomdermatomiosite(DM)epolimiosite(PM)eascomparamoscomumgrupo controle.
Métodos:EsteestudotransversalcomparouECGsde86indivíduossemdoenc¸as reumatológ-icas(controles)com112pacientes(78DMe34PM),de2010a2013.Tambémcomparamos osECGsentreDMePM.
Resultados: Característicasdemográficas,comorbidadesealterac¸õesdeECGforam semel-hantesentrecontrolesepacientes(p>0,05),excetopelamaiorfrequênciadesobrecargade ventrículoesquerdo(SVE)nospacientes(10,7%vs.1,2%;p=0,008).Características demográ-ficas, comorbidades,manifestac¸õesclínicase laboratoriaistambémforam semelhantes entreosgruposPMeDM,excetoporlesõescutâneasapenasempacientescomDM.Um terc¸odospacientesapresentoualterac¸õesdeECG,queforammaisprevalentesemPMdo queemDM(50%vs.24,4%,p=0,008).Sobrecargadecâmarasesquerdas(SCE),distúrbiosdo ritmoedaconduc¸ãoforammaisencontradosemPMdoqueemDM(p<0,05paratodos), sobretudoobloqueiodivisionaldoramoanterossuperior.
Conclusões:Encontramosalterac¸õesdistintasdeECGentrePMeDMefrequênciaaumentada deSVEempacientesquandocomparadoscomcontroles.Investigac¸ãodoacometimento cardíaconessasdoenc¸asdeveserconsideradamesmoempacientesassintomáticos, espe-cialmenteemsetratandodePM.Maisestudossãonecessáriosparacorrelacionarosachados deECGcomoutrosexamescomplementares,manifestac¸õesclínicas,atividadedas miopa-tiaseevoluc¸ãoparaoutrasdoenc¸ascardíacas.
©2014ElsevierEditoraLtda.Todososdireitosreservados.
Introduction
Dermatomyositis (DM)and polymyositis(PM) are immune-mediated inflammatory disorders characterized by the presence of progressive proximal muscle weakness of limbs. In DM, typical skin changes, such as heliotrope and/orGottronsign,stilloccur.Furthermore,extramuscular manifestationssuchasjoint,cardiopulmonaryand gastroin-testinal tract involvement can also be present in these diseases.1,2
Inparticular,cardiacinvolvementinidiopathic inflamma-torymyopathiescanoccurin9–72%ofcases,dependingon thediagnosticmethodusedandselectionofpatients.3–6
Among patients with cardiac involvement, the clinical manifestationsareinfrequentand,whenpresent,aremainly related to congestive heart failure.6,7 In contrast, the vast
majority of patients are asymptomatic and characterized especiallybychangesintheconductionsystem,hypertrophy anddilatationoftheheartchambersanddiastolicleft ventri-culardysfunction.4,5
To date, there are few studies in the literature specif-ically assessing changes in the electrocardiogram (ECG) in idiopathicinflammatorymyopathies.3–5,7–13Whentheyexist,
are essentially limited to vague and brief descriptions of electrocardiographicfindingsinthispopulation.7,9,10,12,13
Fur-thermore,noneofthesestudies7,9,10,12,13presentedacontrol
group,norexaminedsystematicallyandcomparatively possi-bleelectrocardiographicabnormalitiesfoundinpatientswith DMandPM,whichmotivatedustoconductthisstudy.
Materials
and
methods
Thisisacross-sectional,single-centerstudy.Onehundredand twelveconsecutivepatientswithDMandPMwereevaluated accordingtothecriteriaofBohanandPeter14,15intheperiod
between 2010 and 2013, and followed at our department. NopatientswithamyopathicDM,inflammatorymyopathies associatedwithothercollagendiseases,malignanciesorother causesofmyopathywereincluded.Asacontrolgroup,86 out-patient subjectswithnohistoryofrheumaticdisease were selected.Allparticipantsofthisstudywereaged≥18years,did notuseantiarrhythmogenicdrugsnorhadahistoryofangina, palpitations,myocardialinfarction,heartfailure,valvular dis-easeand/orhyperthyroidism.
ThestudywasapprovedbythelocalEthicsCommittee[HC 0039/10].
manifestations (constitutional symptoms, dysphagia, joint involvement[arthritisand/orarthralgia]);pulmonary involve-ment (confirmed by computed tomography: presence of interstitial lung disease, “ground-glass” or honeycombing lesions); skin changes (heliotrope, Gottron papules, ulcers, calcinosis, cutaneous vasculitis, V-sign, shawl sign); labo-ratory abnormalities (serum creatine phosphokinase [CPK], with areference value of 24–173U/L obtained byan auto-matedkineticmethod;antinuclearfactorassessedbyindirect immunofluorescenceusingHep-2cellsassubstrate,anti-Jo-1 antibodybyimmunoblottingmethod).
Demographicdataand comorbidities(hypertension, dia-betesmellitusandhypothyroidism)ofallparticipantswere alsoobtained.
A resting 12-Lead ECG was ordered to all patients for thisstudy;thesetestswere performedandanalyzedatthe
Service ofElectrocardiologyinthe sameInstitute.The con-trol group consistedof sequentialindividuals who had an ECGperformedinthesame service,mostlyinthe preoper-ativeworkupofnon-vascular/cardiacsurgery.Thefollowing electrocardiographic changes were analyzed in the study: rhythmdisturbances(ventricularextrasystole,sinus arrhyth-mia,atrialfibrillation,atrialectopicrhythm, supraventricu-lar tachycardia, atrioventricular block and supraventricular extrasystoles),chamberhypertrophy(leftandrightventricles, left andrightatria),conductionabnormalities(rightbundle branchblock[RBBB],leftbundlebranchblock[LBBB], anterosu-periordivisionalbranchblock[ASDBB])anddiffuseventricular repolarizationchanges(DVRC).Thechangesfoundwere com-paredamongcontrol,PMandDMgroups.
Resultswereexpressedasmean±standarddeviation(SD), median[25–75%interquatiles]orpercentage(%).Forstatistical
Table1–Demographicandclinicaldataandcomorbidities(dermatomyositisandpolymyositis)ofpatientsandcontrol group.
Control(n=86) DM/PM(n=112) *p
Meanage(years) 49.0± 15.3 48.9± 15.4 0.944
Femalegender 70(69.0) 80(71.4) 0.923
Diseaseduration(years) – 5[2–12] –
Comorbidities
Hypothyroidism 10(11.9) 15(13.4) 0.877
Hypertension 31(36.9) 51(45.5) 0.231
Diabetesmellitus 16(19.0) 17(15.2) 0.654
Electrocardiographicchanges 30(35.8) 36(32.1) 0.761
1item(A,B,CorD) 26(31.0) 29(25.9) 0.525
2items(A,B,CorD) 4(4.8) 5(4.5) 1.000
3items(A,B,CorD) 0 2(1.8) –
4items(A,B,CandD) 0 0 1.000
(A)Rhythmchanges 7(8.1) 12(28.6) 0.631
Ventricularextrasystole 2(2.4) 3(2.7) 1.000
Sinusarrhythmia 0 3(2.7) –
Atrialfibrillation 1(1.2) 1(0.9) 1.000
Atrialectopicrhythm 0 2(1.8) –
First-degreeA-Vblock 2(2.4) 3(2.7) 1.000
Supraventriculartachycardia 0 1(0.9) –
Supraventricularextrasystole 2(2.4) 1(0.9) 1.000
Changeof1sub-itemofA 7(8.3) 10(8.9) 1.000
Changeof2sub-itemsofA 0 2(1.8) –
Changeof3ormoresub-itemsofA 0 0 1.000
(B)Chamberhypertrophy 3(3.6) 13(11.6) 0.063
Leftventricular 1(1.2) 12(10.7) 0.008
Rightventricular 0 0 1.000
Leftatrial 2(2.4) 5(4.5) 0.607
Rightatrial 0 0 1.000
Changeof1sub-itemofB 3(3.6) 9(8.0) 0.237
Changeof2sub-itemsofB 0 4(3.6) –
Changeof3ormoresub-itemsofB 0 0 1.000
(C)Conductiondisturbance 6(7.1) 6(16.0) 0.766
ASDBB 4(4.8) 5(4.5) 1.000
RBBB 1(1.2) 1(0.9) 1.000
LBBB 1(1.2) 1(0.9) 1.000
Changeof1sub-itemofC 6(7.1) 5(4.5) 0.766
Changeof2sub-itemsofC 0 1(0.9) –
Changeof3ormoresub-itemsofC 0 0 1.000
(D)DVRC 18(21.4) 14(12.5) 0.161
Table2–Demographicandclinicaldataandcomorbiditiesofpatientswithdermatomyositisandpolymyositis.
Total(n=112) DM(n=78) PM(n=34) ap
Meanage(years) 48.9±15.4 49.1±15.9 48.3±14.3 0.789
Femalegender 80(71.4) 58(74.4) 22(64.7) 0.298
Diseaseduration(years) 5[2–12] 6[3–15] 4[1–10] 0.121
Clinicalmanifestations
Constitutionalsymptoms 27(24.1) 19(24.4) 8(23.5) 0.565
Dysphagia 27(24.1) 20(25.6) 7(20.6) 0.329
Jointinvolvement 46(41.1) 31(39.7) 15(44.1) 0.661
Pulmonaryinvolvement 37(33.0) 28(35.9) 9(26.5) 0.329
Skinlesions
Heliotrope 65(58.0) 65(83.3) 0 –
Gottron’spapules 73(65.2) 73(93.6) 0 –
Ulcers 32(28.6) 8(10.3) 0 –
Calcinosis 10(8.9) 10(12.8) 0 –
Cutaneousvasculitis 11(9.8) 11(14.1) 0 –
Vsign 9(8.0) 9(11.5) 0 –
Shawlsign 3(2.7) 3(3.8) 0 –
Laboratoryabnormalities
InitialCPK(U/L) 723[244–4012] 533[170–3748] 2076[728–4868] 0.221
Antinuclearfactor 49(43.8) 36(46.2) 13(38.2) 0.437
Anti-Jo-1antibody 8(7.1) 4(5.1) 4(11.8) 0.261
Comorbidities
Hypothyroidism 15(13.4) 10(12.8) 5(14.7) 0.770
Hypertension 51(45.5) 37(47.4) 14(41.2) 0.541
Diabetesmellitus 17(15.2) 10(12.8) 7(20.6) 0.390
CPK,creatinephosphokinase;DM,dermatomyositis;PM,polymyositis.
a Comparisonbetweenpatientswithpolymyositisanddermatomyositis.
analysis,Student’standMann–Whitneytestsforcontinuous variablesandchi-squaredorFisher’sexacttestforcategorical datawereused.Thesecalculationswereperformedwiththe computerprogramSTATAversion7.0(STATA,CollegeStation, TX,USA).p-Values<0.050wereconsideredstatistically signif-icant.
Results
Thegeneralcharacteristicsofthe112patients(DMandPM) and of the control group (n=86) are presented inTable 1. Demographic data and comorbidities were similar in both groups(p>0.05).TheECGfindingswere alsosimilar,except forahigherfrequencyofleftventricularhypertrophy(LVH)in patientswhencomparedtothecontrolgroup(10.7%vs.1.2%,
p=0.008).Changesofrhythmweremorefrequentinpatients
versuscontrols,butwithoutstatisticalsignificance(28.6%vs.
8.1%,p=0.631).
An additional analysis comparing 78 DM and 34 PM patientswillbepresentedinTable2.PatientswithDMandPM werecomparableregardingdemographic,clinicaland labora-torydataandalsoastocomorbidities,exceptforthepresence ofskinlesionsonlyinpatientswithDM.
One-thirdofthepatientshadelectrocardiographic abnor-malities (Table 3), which included rhythm disturbances (ventricularextrasystole,sinusarrhythmia,atrialfibrillation, ectopic atrial rhythm, first-degree AV block, supraventric-ular tachycardia, supraventricular extrasystoles), chamber hypertrophy (atrium and left ventricles) and conduction disturbances(ASDBB,RBBBandLBBB)andDVRC.Thepresence
ofchangeswasfoundmorefrequentlyinpatientswithPM whencomparedtopatientswithDM(50%vs.24.4%,p=0.008). MorethanachangeofECGperpatientwasobservedonlyin PMgroup,andnocaseshavebeenreportedinpatientswith DM. None ofthe study participants showedright-chamber changes.
Thefrequencyofrhythmdisturbancesandchamber over-load were higher in patients with PM compared to those withDM(p=0.007andp=0.003,respectively),andbothLVH and left atrium hypertrophy were predominant inpatients withPM(p=0.007andp=0.029,respectively)(Table3).Atrial fibrillation, supraventricular extrasystoles and first-degree atrioventricular block were observed onlyin patients with PM, and nocase have been reported inpatients with DM. Supraventriculartachycardiawasobservedonlyinonecase intheentirestudy,inapatientwithDM.
Conductiondisordersalsoweremorefrequentinpatients withPM(p=0.010),withemphasisforASDBB(p=0.029).No patienthadRBBBorLBBBintheDMgroupcomparedtoacase ofeachinPMgroup.
Discussion
Table3–Demographic,clinicalandlaboratorydataandcomorbiditiesofpatientswithdermatomyositisand polymyositis.
Total(n=112) DM(n=78) PM(n=34) p
Electrocardiographicchanges 36(32.1) 19(24.4) 17(50.0) 0.008
1item(A,B,CorD) 29(25.9) 19(24.4) 10(29.4) 0.367
2items(A,B,CorD) 5(4.5) 0 5(14.7) –
3items(A,B,CorD) 2(1.8) 0 2(5.9) –
4items(A,B,CandD) 0 0 0 1.000
(A)Rhythmchanges 12(28.6) 4(5.1) 8(23.5) 0.007
Ventricularextrasystole 3(2.7) 1(1.3) 2(5.9) 0.166
Sinusarrhythmia 3(2.7) 2(2.6) 1(2.9) 1.000
Atrialfibrillation 1(0.9) 0 1(2.9) 0.304
Atrialectopicrhythm 2(1.8) 1(1.3) 1(2.9) 0.517
First-degreeA-Vblock 3(2.7) 0 3(8.8) –
Supraventriculartachycardia 1(0.9) 1(1.3) 0 –
Supraventricularextrasystole 1(0.9) 0 1(2.9) –
Changeof1sub-itemofA 10(8.9) 3(3.8) 7(20.6) 0.008
Changeof2sub-itemsofA 2(1.8) 1(1.3) 1(2.9) 0.517
Changeof3ormoresub-itemsofA 0 0 0 1.000
(B)Chamberhypertrophy 13(11.6) 4(5.1) 9(26.5) 0.003
Leftventricular 12(10.7) 4(5.1) 8(23.5) 0.007
Rightventricular 0 0 0 1.000
Leftatrial 5(4.5) 1(1.3) 4(11.8) 0.029
Rightatrial 0 0 0 1.000
Changeof1sub-itemofB 9(8.0) 3(3.8) 6(17.6) 0.022
Changeof2sub-itemsofB 4(3.6) 1(1.3) 3(8.8) 0.083
Changeof3ormoresub-itemsofB 0 0 0 1.000
(C)Conductiondisturbance 6(16.0) 1(1.3) 5(14.7) 0.010
ASDBB 5(4.5) 1(1.3) 4(11.8) 0.029
RBBB 1(0.9) 0 1(2.9) –
LBBB 1(0.9) 0 1(2.9) –
Changeof1sub-itemofC 5(4.5) 1(1.3) 4(11.8) 0.029
Changeof2sub-itemsofC 1(0.9) 0 1(2.9) 1.000
Changeof3ormoresub-itemsofC 0 0 0 1.000
(D)DVRC 14(12.5) 10(12.8) 4(11.8) 1.000
DVRC,diffuseventricularrepolarizationchanges;A-V,atrioventricular;ASDBB,anterosuperiordivisionalbranchblock;RBBB,rightbundle branchblock;LBBB,leftbundlebranchblock;DM,dermatomyositis;PM,polymyositis.
inECGwerefoundinmostpatientswithPM,whencompared
topatientswithDM,especiallyforthehigherfrequencyofleft chamberhypertrophy,changesofrhythmandthepresenceof ASDBBinthefirstgroup.
So far, there are few studies evaluating
electrocardio-graphic changes in patients with DM and PM; and, when
present,thesearelimitedtoasmallnumberofcasesand/or in the absence of a control group,7,9,10,12,13 differently of
thepresent study.ECGchangesinidiopathic inflammatory myopathiesoccurin33–72%.3–5,7–13
Our data show that LVH was significantly present in patientswithDMandPMcomparedtothecontrolgroup. Fur-thermore,anadditionalanalysisshowedthe persistenceof left-chamberhypertrophy in patientswith PM, when com-paredtopatientswithDM. Thisfinding couldbeexplained bythehighprevalenceofsystemichypertension,commonly foundinpatientswithDM andPM,16,17 althoughthis
find-ingwassimilartothe prevalenceinthecontrolgroup.Itis necessary,however, toevaluatetheduration and degreeof hypertensionintheseindividuals,aswellastheirblood pres-surecontrol.AndthepathogenesisofDMandPMitselfalso mayhavecontributedtoleftchamberhypertrophy.Thereare indicationsofpresenceofinfiltrationofmononuclear inflam-matory cellsin the endomysiumand inperivascular areas
of infarction, as well as degeneration of cardiac myocytes andareasofmyocardialfibrosis.12,13,18,19 Thisinflammatory
process in the myocardium can lead to remodeling, with anatomicalandfunctionalchangesofthemyocardiumand, ultimately,possibleleftventriculardysfunctionandrestrictive cardiomyopathyandheartfailure.Thus,ECGchangesmaybe anearlyfindingofthesecomplications.
Corroboratingourdata,Sharrattetal.10showedthat5of
13patientswithPMhadcardiacabnormalitiesseenby clini-calexamination,ECGand/orchestradiography.Ofthesefive, four patientshadabnormalities ofleft ventricular dysfunc-tion,diagnosedbyECG.Ontheother hand,Gonzalez-Lopez etal.4observedinacaseseriesof32patientsthepresenceof
diastolicdysfunctionofleftventriclein42%ofcases.Schwartz etal.11compared59patientswithjuvenileDMwith59healthy
subjectsandobservedthatonlytheirpatientshad subclini-caldiastolic(leftventricular)dysfunction,showingthatsuch changeisinherenttothedisease(juvenileDM).
withECGandHoltermonitoringinpatientswithDMandPM showedseveralabnormalities inthispopulation: atrialand ventricularextrasystoles,atrialtachycardia,ventricular tachy-cardia, atrial fibrillation, atrioventricular conduction block, bundlebranchblocks,abnormalQwavesandnonspecificST-T segmentchanges.3,4,6,7
Instudieswithautopsy,histologicalchangeswerefoundin theconductionsystem,withthepresenceoflymphocyte infil-trationandsinoatrialnodefibrosis,18,19whichmayexplainthe
presenceofconductionfindings inpatients withidiopathic inflammatorymyopathies.
Ourstudy haslimitations,bynotpresentingastructural cardiacevaluationnoranassessmentformyocardium inflam-mation.However,asthe cardiac involvementisafactor of worseprognosisforpatientswithmyopathies,20theECGmay
serveasanoninvasive,cheapand practicaltoolforan ini-tialassessmentandintrackingthisproblem.Anotherlimiting factoristhefailuretoestablishacorrelationofECGfindings, particularlywithdisease activity(DM/PM).Furthermore,we mustconsiderthatthelackofelectrocardiographicdifferences betweenpatientsand controlsmay alsobeduetoa selec-tionbias,sincethecontrolgroupcamefromthepreoperative (andclinical)sector,withafrequencyofcomorbiditiessimilar tothatofourpatientsandperhapswithahigherfrequency ofelectrocardiographicchangesversushealthysubjects. How-ever,webelievethatthisbiasisnotsignificant,duetothefact thatmostECGsincontrolgroupwereperformedonpatients involvedinpreoperativenon-cardiac/vascularsurgery.
Insummary,we observeda higherfrequency ofLVH in patientswithinflammatorymyopathies.Furthermore,there wasadistinctionoftheECGfindingsbetweenpatientswith DMandPM,withhigherprevalenceofleft-chamber hypertro-phyandrhythmandconductiondisturbancesinpatientswith PMcomparedtoDM.
Morestudies areneededtodeterminethecorrelationof ECGfindingswithothercomplementarytests,clinical man-ifestations,myopathyactivityandevolutiontoothercardiac diseases.
Conflicts
of
interest
Theauthorsdeclarenoconflictsofinterest.
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