Rev. Bras. Reumatol. vol.57 número2

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

Incidence

and

prevalence

of

systemic

sclerosis

in

Campo

Grande,

State

of

Mato

Grosso

do

Sul,

Brazil

Alex

Magno

Coelho

Horimoto

,

Erica

Naomi

Naka

Matos

,

Márcio

Reis

da

Costa

,

Fernanda

Takahashi

,

Marcelo

Cruz

Rezende

,

Letícia

Barrios

Kanomata

,

Elisangela

Possebon

Pradebon

Locatelli

_,

_Leandro

_Tavares

_Finotti

,

Flávia

Kamy

Maciel

Maegawa

_,

_Rosa

_Maria

_Ribeiro

_Rondon

_,

Natália

Pereira

Machado

,

Flávia

Midori

Arakaki

Ayres

Tavares

do

Couto

,

Túlia

Peixoto

Alves

de

Figueiredo

_,

_Raphael

_Antonio

_Ovidio

_,

_Izaias

_Pereira

_da

_Costa

a_{UniversidadeFederaldeMatoGrossodoSul(UFMS),CampoGrande,MS,Brazil}

b_{HospitalRegionaldeMatoGrossodoSul,Servic¸odeReumatologia,CampoGrande,MS,Brazil}

c_{UniversidadeFederaldeMatoGrossodoSul(UFMS),HospitalUniversitário,Servic¸odeReumatologia,CampoGrande,MS,Brazil} d_{UniversidadedeBrasília(UnB),Brasília,DF,Brazil}

e_{PrefeituraMunicipaldeCampoGrande,AmbulatóriodeEspecialidadesMédicas,CampoGrande,MS,Brazil}

f_{UniversidadeAnhanguera(Uniderp),FaculdadedeMedicina,AmbulatóriodeEspecialidadesMédicas,CampoGrande,MS,Brazil} g_{SantaCasadeCampoGrande,Servic¸odeReumatologia,CampoGrande,MS,Brazil}

h_{CaixadeAssistênciaaosServidoresdoMatoGrossodoSul(CASSEMS),AmbulatóriodeEspecialidadesMédicas,Coxim,MS,Brazil} i_{UniversidadedeSãoPaulo(USP),SãoPaulo,SP,Brazil}

j_{UniversidadeFederaldeSãoPaulo(UNIFESP),SãoPaulo,SP,Brazil}

k_{UniversidadeFederaldaGrandeDourados(UFGD),HospitalUniversitário,Servic¸odeReumatologia,Dourados,MS,Brazil}

a

r

t

i

c

l

e

i

n

f

o

Articlehistory:

Received12October2015 Accepted2May2016

Availableonline15October2016

Keywords: Systemicsclerosis Scleroderma Incidence Prevalence Brazil

a

b

s

t

r

a

c

t

Introduction:Systemicsclerosisisanautoimmunediseasewhichshowsextreme hetero-geneityinitsclinicalpresentationandthatfollowsavariableandunpredictablecourse. Althoughsomediscrepanciesintheincidenceandprevalenceratesbetweengeographical regionsmayreflectmethodologicaldifferencesinthedefinitionandverificationofcases, theymayalsoreflecttruelocaldifferences.

Objectives: Todeterminetheprevalenceandincidenceofsystemicsclerosisinthecityof CampoGrande,statecapitalofMatoGrossodoSul(MS),Brazil,duringtheperiodfrom JanuarytoDecember2014.

Methods:AllhealthcareservicesofthecityofCampoGrande–MSwithattendinginthe spe-cialtyofRheumatologywereinvitedtoparticipateinthestudythroughastandardizedform ofclinicalandsocio-demographicassessment.Physiciansofanyspecialtycouldreporta suspectedcaseofsystemicsclerosis,butnecessarilythedefinitivediagnosisshouldbe estab-lishedbyarheumatologist,inordertowarrantthestandardizationofdiagnosticcriteria andexclusionofotherdiseasesresemblingsystemicsclerosis.Attheendofthestudy,

∗ _{Correspondingauthor.}

E-mail:[email protected](A.M.Horimoto). http://dx.doi.org/10.1016/j.rbre.2016.09.005

15rheumatologistsreportedthattheyattendedpatientswithsystemicsclerosisandsent thecompletedformscontainingepidemiologicaldataofpatients.

Results:TheincidencerateofsystemicsclerosisinCampoGrandefortheyear2014was11.9 permillioninhabitantsandtheprevalenceratewas105.6permillioninhabitants.Systemic sclerosispatientsweremostlywomen,white,withameanageof50.58years,showingthe limitedformofthediseasewithameandurationofthediseaseof8.19years.Regarding laboratorytests,94.4%werepositiveforantinuclearantibody,41.6%foranti-centromere antibodyand19.1%foranti-Scl70;anti-RNAPolymeraseIIIwasperformedin37patients, with16.2%positive.

Conclusions: ThecityofCampoGrande,thestatecapitalofMS,presentedalower inci-dence/prevalenceofsystemicsclerosisincomparisonwiththosenumbersfoundinUS studiesandclosetoEuropeanstudies’data.

PublishedbyElsevierEditoraLtda.ThisisanopenaccessarticleundertheCCBY-NC-ND license(http://creativecommons.org/licenses/by-nc-nd/4.0/).

Incidência

e

prevalência

de

esclerose

sistêmica

em

Campo

Grande,

Estado

de

Mato

Grosso

do

Sul,

Brasil

Palavras-chave: Esclerosesistêmica Esclerodermia Incidência Prevalência Brasil

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e

s

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o

Introduc¸ão: Aesclerosesistêmica(ES)éumaenfermidadeautoimune,extremamente het-erogêneanasuaapresentac¸ãoclínicaesegueumcursovariáveleimprevisível.Embora algumasdiscrepânciasnastaxasdeincidênciaeprevalênciaentreregiõespossam refle-tirasdiferenc¸asmetodológicasnadefinic¸ãoeverificac¸ãodoscasos,elastambémpodem refletirasverdadeirasdiferenc¸aslocais.

Objetivos: ConheceraprevalênciaeincidênciadaESnacidadedeCampoGrande,capital doEstadodeMatoGrossodoSul(MS),Brasil,dejaneiroadezembrode2014.

Métodos: Todososservic¸osdesaúdedeCampoGrande(MS)quetinhamatendimentosna especialidadedereumatologiaforamconvidadosaparticipardoestudopormeiodeficha padronizadadeavaliac¸ãoclínicaesociodemográfica.Médicosdequalquerespecialidade poderiamreportarum casosuspeito deES,masobrigatoriamenteodiagnóstico defini-tivodeveriaserfeitoporumreumatologista,paragarantira padronizac¸ãodoscritérios diagnósticoseexcluiroutrasdoenc¸asqueseassemelhamàES.Nofimdoestudo15 reuma-tologistasrelataramteratendidopacientescomdiagnósticodeESeenviaramosformulários preenchidoscomosdadosepidemiológicosdospacientes.

Resultados: AtaxadeincidênciadeESemCampoGrandeem2014foide11,9por mil-hão/habitanteseadeprevalênciafoide105,6pormilhão/habitantes.Ospacientescom ESeramprincipalmentemulheres,dacorbranca,médiade50,58anos,formalimitadada doenc¸aetempodeevoluc¸ãomédiodadoenc¸ade8,19anos.Emrelac¸ãoaosexames lab-oratoriais,observou-seapositividadede94,4%paraoANA,41,6%paraACAe19,1%para anti-Scl70,oanticorpoanti-POL3foifeitoemapenas37 pacientes,compositividadede 16,2%.

Conclusões: AcapitaldoEstadodeMatoGrossodoSul,CampoGrande,apresentoudados de incidênciae prevalênciadeESinferioresaosencontradosem estudosamericanose próximosaosdadosobservadosemestudoseuropeus.

PublicadoporElsevierEditoraLtda.Este ´eumartigoOpenAccesssobumalicenc¸aCC BY-NC-ND(http://creativecommons.org/licenses/by-nc-nd/4.0/).

Introduction

Systemic sclerosis (SSc) is an autoimmune disease of the connectivetissue,extremelyheterogeneousinitsclinical pre-sentation, with the involvement of multiple systems and that follows a varied and unpredictable course.1 _Its etiol-ogyremainsunknown;amultifactorialcausewassuggested, possiblytriggeredbyenvironmentalfactorsinagenetically predisposedindividual.2

TheclassificationofSSc patientstakesinto accountthe extension of skin involvement and the presence of over-lapping with certain characteristics of other autoimmune rheumaticdiseases.3–5

casespermillioninhabitantsperyear.3_Astudyina south-ernstateinAustraliareported anannualincidenceof22.8 newcasesofSScpermillioninhabitantsandaprevalenceof 233casesper millioninhabitantsin1999,withvalues sim-ilar to those in American studies conducted in the same period.6

Morerecently,asystematicreviewstudyreportedsimilar prevalencesofSScobservedintheUKandJapan,with31and 38casespermillioninhabitants,respectively.7_{Itisnoteworthy} that,inadditiontoregionalgeneticvariations,environmental exposurescanalsohaveaneffectontheprevalenceand inci-dencerates.Forexample,silicaexposureappearstoincrease theriskofdevelopingSSc;however,thistriggeringactionis onlyimportanttoasmallproportionofmalepatients.7

Interestingly,therehasbeenanincreaseinSScincidence ratesindifferentgeographicalregions,6,8 _{possiblyduetoan} earlierdiagnosisandalsototheuseofnewclassification crite-ria.Forexample,intheUnitedStates,theratefornewcases increasedfrom 0.6 casesper million in 1947in Tennessee to19.0casespermillion in1991inthe Detroitarea.9 Like-wise,theprevalenceandincidenceofSScappeartobelarger in populations of European ancestry, and lower in groups ofAsiandescent.7 _{InTaiwan,theincidenceandprevalence} rateswere10.9and56.3casespermillioncases/inhabitants, respectively.10

In epidemiology studies of SSc, different results are observed in different regions of the world, and this also occursinonesamecountryorcity.6–8,10_{SScprevalencedata} inamulti-ethnicFrenchdistrictsuggestedthatthedisease appearstobemorefrequentandsevere inapopulationof non-Europeanorigin,whichspeaksinfavoroftheideathat theracecouldinfluencethesusceptibilitytothedevelopment ofSSc,andalsotheclinicalprofile.11_{Inthis sameline,the} EuropeangroupofSScresearchpointedoutthat geographi-calvariationsinpatientswithSScmayalsohaveaninfluence withregardtoantibodyassociationsandintherateof occur-renceamongwomenandmen,butnoassociationsbetween raceswerefound.12

Therearenopublisheddataontheprevalenceand inci-dence of SSc in the Brazilian population, since this is a rarecondition.Thus,duetothescarcityofnationalstudies andthehighdegreeofmiscegenationfoundintheBrazilian population13_{weaimedtostudytheprevalenceandincidence} ofsystemicsclerosisinthecityofCampoGrande,thestate capitalofMatoGrossodoSul,Brazil,duringtheperiodfrom JanuarytoDecember2014.

Objectives

Todeterminetheprevalenceandincidenceofsystemic scle-rosisinthecityofCampoGrande,MatoGrossodoSul,Brazil, duringtheperiodfromJanuarytoDecember2014.

Methods

AllhealthcareservicesinCampoGrande–MSwith Rheuma-tologyspecialtyparticipatedinthisprospectiveobservational study.

TheRheumatologyunitsinthecityaredistributedamong theMedicalSchoolTeachingHospitaloftheUniversidade Fed-eral de MatoGrosso doSul,the Regional Hospital ofMato GrossodoSul,SantaCasadeCampoGrande,theoutpatient clinicoftheMedicalSpecialtiesCenteroftheMunicipalityof CampoGrande,outpatientclinicsoftheMedicalSpecialties Center ofAnhanguera-UniderpMedicineSchool,outpatient clinics ofthe Caixade AssistênciadosServidoresde Mato GrossodoSul,andseveralprivateRheumatologyclinics.

Prior to starting this study, all rheumatologists were informed by e-mail and phone call about the procedures for data collection and objectives ofthis research. Period-ically, we asked (by e-mail or phone call) to all involved doctorstocompleteastandardizedformforcollecting demo-graphic and laboratory dataof all patients diagnosedwith systemic sclerosis and evaluated during the study period, regardlessofwhethertheywereneworoldcases.Any doc-torcouldreportasuspectedcaseofSSc(generalpractitioner, dermatologist, vascularsurgeon, gastroenterologist, pulmo-nologist,etc.),butthedefinitivediagnosisnecessarilyshould beestablishedbyarheumatologist,inordertoensurethe stan-dardizationofdiagnosticcriteriaandtoruleoutotherdiseases resemblingSSc,forexample,mixedconnectivetissuedisease (MCTD).

At the end of the study, 15 rheumatologists reported patients withSSc,andsent thecompletedformswith epi-demiologicaldataoftheirpatients;verbalorwrittenconsent from all patientswas requested.Thereasons forother MS rheumatologistsdidnotreportcaseswere:theydidnot exam-inepatientswithSScintheperiod,orthepatientsseendidnot liveinCampoGrande–MSorthepatientshadalreadybeen reportedbyanothercolleague(patient’s duplicity).Toavoid dataredundancyintheeventthatanindividualpatienthad beenassessedbymorethanarheumatologist,thesepatients wereidentifiedbytheinitialsoftheirnamesandtheirdateof birth.

PatientsdiagnosedwithSScandnon-residentsofCampo Grande–MSwerenotconsideredforincidenceandprevalence estimates.

Tobeselected,patientswithSScshouldmeetthefollowing criteria:

- Meetthe2013classificationcriteriaoftheACR/EULARfor SSc14_;

- Inthecaseofabsenceofskinthickening,patientsshould meetthe2001criteriaofLeRoyandMedsgerforearlySSc.15

Stateoforigin,provenance,age,genderandrace/colordata, andtimeelapsedfromfirstsymptomstodiagnosis,disease duration, and clinical formofSSc were collected,and lab-oratory tests suchasantinuclear antibody(ANA) anti-DNA topoisomerase I antibody(antiScl70), anticentromere anti-body(ACA)andanti-RNApolymeraseIII(anti-RNAPIII)also wereconducted.

Themethodsusedinautoantibodysurveywere, respec-tively:

a. Antinuclearantibodies(ANA)survey

Table1–Comparisonofincidenceandprevalenceratesofpatientswithsystemicsclerosis(SSc)inseveralgeographical regions.

Publication Geographicalregion Periodofstudy Prevalencepermillioninhabitants Incidencepermillioninhabitants

Horimotoetal. CampoGrande–Brazil 2014–2015 105.6 11.9

Rosaetal.26 _{BuenosAires–Argentina 1999–2004 296 21.2}

Mayesetal.28 _{Detroit–USA 1989–1991 242 19.3}

LoMonacoetal.37 _{Ferrara–Italy 1999–2007 254 32}

Alamanosetal.29 _{Greece 1981–2002 154 11}

Thompsonetal.30 _{Australia 1993–2002 232.2 20.4}

Tamakietal.35 _{Tokyo–Japan 1987–1988 38 7.2}

Kuoetal.10 _{Taiwan 2002–2007 56 10.9}

Theresultsareshowninnumberofcasespermillioninhabitantsperyear.

BrazilianConsensusonAntinuclearFactorinHep-2cells (2003)criteria16_{fortheinterpretationofresults.}

Serawereconsideredpositivewithatiter≥1/160,with dilu-tionanegativefluorescence.

b. Anticentromere survey – Indirect immunofluorescence techniquewithHEp2cellsasasubstrate,accordingtothe IIBrazilianConsensusonAntinuclearFactorinHep-2cells (2003)criteria16_{fortheinterpretationofresults.}

c. Anti-DNAtopoisomeraseI(antiScl70)survey– Immunoas-say technique17_{;thesamplewasconsiderednonreactive} withvalues<20units,weaklyreactivebetween20and39 units, moderatelyreactive between40and 80 unitsand stronglyreactive(highervalues)withvalues>80units. d. Anti-RNA polymerase III Antibody survey – ELISA

technique18_{; the sample was considered negative with} values<20units,weaklyreactivebetween20and39units, moderatelyreactivebetween40and80units,andstrongly reactive(highervalues)with>80units.

Statistical

analysis

IBGEdata19_{withestimatesoftheresidentpopulationinBrazil} andinUnitsoftheFederationandwithareferencedateofJuly 1,2014,wereconsideredforthecalculationoftheincidence andprevalenceofSSc.

Data is presented in absolute and relative frequencies, meansandstandarddeviations,andwithaconfidence inter-valof95%andstatisticallysignificantvaluesforp<0.05.

Results

During2014,atotalof166patientswithsclerodermaor sys-temicsclerosisweretreatedinvariousoutpatientclinicsand RheumatologyUnitsinthecityofCampoGrande–MS. Eighty-ninepatientswholivedinthecityhadadefinitivediagnosis ofsystemicsclerosisandwereclinicallyexaminedinthatcity duringthestudyperiod.

Ofthistotal,10werenewcasesofSScdiagnosedduring the year2014and 79 patientshad already been previously diagnosed.Therefore,theincidencerateofSScinthecityof CampoGrande–MSduringtheyear2014was11.9per mil-lioninhabitantsandtheprevalenceratewas105.6permillion

inhabitants. Thedataare presentedin Table1, whichalso listscomparisonswithincidenceandprevalenceratesinother countriesandregions.

Resultsobservedinsystemicsclerosis

Amongthe89patientswithSSc,86werewomen(96.6%)and 3 were men (3.4%) with a mean age of 50.58±13.85 years (mean±standarddeviation).

Thirty-onepatients(34.8%)withSScwereborninthecityof CampoGrande;31patients(34.8%)wereborninthe country-sideofMT,and27patients(30.4%)wereborninotherstates.

Ofthe89patientswithSSc,58patients(65.2%)reported beingwhite,20patients(22.4%)hadabrowncolor,8patients (9.0%)wereblackand3patientsyellow(3.4%).

Regarding clinical forms of SSc, 38 patients (42.7%) showed the limitedform, 24patients (27.0%)exhibited the diffuse form, 17 patients (19.1%) show overlapping with other collagen diseases, 6 patients (6.7%) exhibited the sine scleroderma form and 4 patients (4.5%) had the early form. Among the 17 patients with the overlapped form, 8 patients (47.1%) concomitantly had systemic lupus erythe-matosus,5patients(29.4%)hadrheumatoidarthritis,and4 patients(23.5%)hadtheirSScassociatedwithinflammatory myopathies.

PatientswithSScwerealreadypresentingsymptomsfor 4.74±5.01yearsbeforetheirdiagnosis,andthedisease dura-tion,ingeneral,wasabout8.19±7.40years.

ANAwaspositivein84patientswithSSc(94.4%),andthe main patterns found inthese patients were: fine speckled nuclearpattern(30patients–36.6%),centromericpattern(29 patients – 35.4%) and quasi-homogeneousnuclear pattern, withmetaphaseplatestainingfor5–10points(12patients– 14.6%). Amongall patients,37 (41.6%)hadpositiveACA,17 (19.1%)werepositiveforanti-Scl70and1patient(1.1%)was simultaneouslypositiveforbothautoantibodies.Ontheother hand,theanti-RNAPIII wasperformedinonly37 patients, beingpositivein6ofthese(16.22%).

Table2–Distributionofpatientsevaluatedinthisstudy andresultsofepidemiologicaldatainpatientswith systemicsclerosis(SSc).

Variable Results

Epidemiologicaldata

Age 50.58±13.85

Gender

Male 3(3.4)

Female 86(96.6)

Race

White 58(65.2)

Brown 20(22.4)

Black 8(9.0)

Yellow 3(3.4)

Diseaseduration

<5years 33(37.1)

Between5and10years 31(34.8)

>10years 25(28.1)

DurationofsymptomsbeforeDx 4.74±5.01

DurationofsymptomsafterDx 8.19±7.40

Clinicalform

Limited 38(42.7)

Diffuse 24(27.0)

Overlap 17(19.1)

Sinescleroderma 6(6.7)

Early 4(4.5)

Dx,diagnosis.

Theresultsarepresentedasmean±standarddeviationoras abso-lutefrequency(relativefrequency).

Table3–Resultsofautoantibodiesinpatientswith systemicsclerosis(SSc).

Variable Results

ANA

Positive 84(94.4)

Negative 5(5.6)

ANApattern(n=84)

Nuclearfinespeckled 30(35.7)

Centromeric 29(34.5)

Nuclearquasihomogeneous 12(14.3)

Others 13(15.5)

Anti-Scl70

Positive 17(19.1)

Negative 72(80.9)

ACA

Positive 37(41.6)

Negative 52(58.4)

anti-RNAPIII(n=37)

Positive 6(16.2)

Negative 31(83.8)

ANA,antinuclearantibody;Scl70,anti-DNAtopoisomeraseI anti-body; ACA, anti-centromere antibody; anti-RNAP III, anti-RNA polymeraseIIIantibody.

Theresultsarepresentedinabsolutefrequency(relativefrequency).

Discussion

Inthis study,anunprecedentedand representativesample

oftheMidwestofBrazilwasdefined,havingbeen

character-izedbyaheterogeneousgroupofpatientswithvariousspectra

ofdiseaseanddifferentstagesofclinicalmanifestationsand

activitiesofthedisease,butthatisverysimilartowhatoccurs

inotherpopulationsofpatientsinthiscountryandevenfrom

otherlocations.3,13,20–23

Asystematicreviewof32articlespublishedfrom1969to 2006indicatedthattheincidencerateforSScvariedfrom0.6 to122casespermillioninhabitants;ontheotherhand,the prevalencerate forthesame disease rangedfrom 7to489 casespermillioninhabitants,9_{whichisconsistentwiththe} ratesobservedinourstudy.Severalgeographicalvariations wereobserved,withahigherprevalenceofSScintheUnited States(276casespermillioninhabitants)andAustralia(233 casespermillioninhabitants)versusJapanandEurope,where onestillobservedanorth–southvariablegradient,withlower prevalenceratesinnorthernEuropeancountries.9,12

TheratesfoundforSScinourstudy(incidenceof11.9per millioninhabitantsandprevalenceof105.6permillion inha-bitants)aremoresimilartothoseforEuropeancountries.For example,theprevalenceofSScinaFrenchmulti-ethnic dis-trictwas158.3casespermillioninhabitantsin200111_;inthe northofEnglandaprevalenceof88.0casespermillion inha-bitantsin2000wasfound.24_{ApeculiarityofthecityofCampo} Grande–MSisthatitspopulationwasmainlycomposedof nationalimmigrantsandforeigners,whocamemainlyfrom thestatesofMinasGerais,RioGrandedoSul,Parana,andSao Paulo,and fromcountrieslikeGermany,Spain,Italy,Japan, Paraguay,Portugal,SyriaandLebanon.25

There are no Brazilian studies published on the inci-denceorprevalenceofSSc.InSouthAmerica,theincidence andprevalenceofSScobservedinBuenosAires–Argentina were 21.2 cases and 296 cases per million inhabitants, respectively.26 _{In the Caribbean, a lower incidence was} observed, with a total of 17 cases of SSc observed in the Afro-descendantpopulationofBarbadosduringan observa-tionperiodof10years(1996–2006).27_{InNorthAmerica,the} incidenceandprevalenceofSScobservedintheUnitedStates were higher, respectively 19.3 and 242.0 cases per million inhabitants.28

Althoughsomediscrepanciesintheincidenceand preva-lence of SSc between regions may reflect methodological differences inthe definition and verificationof cases,they canalsoreflecttruelocaldifferences.Theseregional differ-encescouldoccurduetothediversegeneticsusceptibilityto thedevelopmentofSSc,ortodifferentdegreesofexposureto environmentalfactorsincriminatedinthepathogenesis.6

(BarbadosandPuertoRico),theratesarerespectively26:127 and23:133_{;inNorthAmerica(USandCanada),theratesare} respectively6.1:18_and7.6:134_{;inAsia(TaiwanandJapan),the} ratesare respectively 3.5:110 _{and 14:1}35_{; intheMiddle East} (Iraq),therateis8.3:136_{;andinEurope(Italy,Germany,France} andEngland),theratesarerespectively9.7:1,37_5:1,21_11.5:1,11 and5.2:1.24

Regarding other demographic data found, the mean age of our patients with SSc (50.5 years) was almost consensus among the various studies in Brazil and overseas,6,11,13,20–24,28–31,33,34,36,37 _{with the diagnosis} estab-lishedbetweenthefourthandfifthdecadesoflife;onlythe African-Caribbeanpopulation27 _{hadayoungermeanageat} diagnosis (37.3years). Withregardto theraceinformed by the patient, there was a prevalence of white color in our patientswithSSc;however,onedonotrulesoutthe possi-bilityofabiasofracialclassification,duetothehighdegree ofmiscegenationfoundintheBrazilianpopulation.13_Inthe South20 _andSoutheast23 _{ofthiscountry,therewasahigher} prevalenceofwhitepeoplewithSSc.Ontheotherhand,inthe Northeast38_{region,ahighprevalenceofmulattoesandblacks} was observed, probably because the study was conducted inastatewithaknownpredominanceofAfro-descendants (Bahia).

In this same vein, the European researchgroup on SSc (EUSTAR)pointedoutthatgeographicalvariationsinpatients withSScmay alsohaveanimpactwithregardtoantibody associationsandintherateofoccurrencebetweenwomen andmen,12_{butthis,aswellasanotherstudy,foundno} asso-ciationbetweenraces.12,39 _{Inthisstudy,mostpatientswith} SScwereborninthesamestate(73.4%),andtheremaining patientswerefromdifferentlocations,butmainlyfromthe statesofSãoPaulo(13.44%)andParaná(5.04%).

In this study, the limited clinical presentation of SSc showedaslightpredominanceofthediffuseform,in accor-dance with other descriptions in the country13,20,23,37 _and inmostotherpopulations.11,21,22,24,28,33,36_{However,thereare} descriptionsinwhichthediffuseformismorecommon in populationsofBlacksvs.Caucasianpopulations,11,28,38 includ-ing,inthiscase,thoseofAfrican-Caribbeandescent,inwhich apredominanceofdiffuse(63%)overthelimited(37%)form wasobserved.27

Regardingthelaboratorytestsperformedinpatientswith SSc, antinuclear antibody (ANA) was present in 94.4% of patients–asimilar resulttomostnationalstudies13,20,23,37 and in other regions.11,21,28,36,40–42 _{The main patterns} observedwere:finespecklednuclear,centromeric,and quasi-homogeneous nuclear patterns. Bernstein et al. described ANA positivity in 97% of patients with SSc, mainly repre-sentedbyfinespeckledandcentromericpatterns,besidesthe observationofanassociationwiththenucleolar(speckledand homogenous)patternin33%ofpatients.42_{Hesselstrandetal.} reportedANApositivityin84%ofpatientswithSSc,andthe mostobservedpatterns were:finespeckled(41%), homoge-neous(25%),nucleolar(24%)andcentromeric(18%)patterns.40 RegardingspecificantibodiesforSScobservedinthisstudy, anti-centromere(ACA),anti-DNAtopoisomeraseI(antiScl70) and anti-RNA polymerase III (anti-RNAP III) positivity was observedin41.6%,19.1%and16.2%ofpatients,respectively, andthepercentageswerecomparabletothoseintwostudies

conductedinSouthernBrazil,13,20_{althoughMülleretal.had} foundsurprisinglyhighlevelsofanti-RNAPIII(41.18%).20_In thisstudy,60of89patients(67.4%)werepositiveforatleast oneofthosethreespecificautoantibodiestoSSc(anti-Scl70, ACA,oranti-RNAPIII).

Theliteraturereportsthattheprevalenceofhighlyspecific autoantibodiesassociatedwithSScorwithoverlapsyndromes withSSCfeaturesishighinpatientswithSSc,primarily repre-sentedbyACAandantiScl70,40–42_{hereincludingtheBrazilian} population with SSc.13,20 _{Although the studies report that} the coexistence of these specific autoantibodies is rare in patientswithSSc(1.6%),40–42 _{inthisstudy,weobservedtwo} patients(2.2%)thatwereconcomitantlypositiveforspecific autoantibodies:onepatientwiththediffuseformpresented concomitant positivityforantiScl70 andACA,and another patientalsowiththediffuseformhadconcomitantpositivity forantiScl70andanti-RNAPIII.

Our conclusion is that the city of Campo Grande, the state capital of MatoGrosso do Sul, presented lower inci-denceandprevalenceofSScversusthosefoundinAmerican studies and similar to those observed in European stud-ies. This incidence, however, may still be underestimated, especiallyinpatientswiththelimitedformofSSc,because in these individuals only the Raynaud’s phenomenon is apparent formany years,withlittle systemicinvolvement; thus,theymaynotseekmedicalattention.Wesuggestthat epidemiologicalsurveysinSScareconductedinother Brazil-ian cities, in order to reflect possible regional differences and environmental influencesin the development ofboth diseases.

Conflict

of

interest

Theauthorsdeclarenoconflictsofinterest.

Acknowledgements

We thankDr.Natalino Yoshinarifor hisencouragementto thisstudyandDr.AlbertSchiavetodeSouzaforthestatistical analysis.

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