Rev. Bras. Reumatol. vol.57 número6

r e v b r a s r e u m a t o l . 2017;57(6):620–622

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

Lyme

disease

and

juvenile

idiopathic

arthritis

–

A

pediatric

case

report

夽

Doenc¸a

de

Lyme

e

artrite

idiopática

juvenil

–

Relato

de

caso

clínico

pediátrico

Mário

Correia

de

Sá

,

Catarina

Moreira

,

Cláudia

Melo

,

Álvaro

Sousa

,

Sónia

Carvalho

a_{CentroHospitalardeVilaNovadeGaia/Espinho,Servic¸odePediatria,VilaNovadeGaia,Portugal}

b_{UnidadedeSaúdeFamiliarRibeirão,VilaNovadeFamalicão,Portugal}

c_{CentroHospitalardoMédioAve,Servic¸odePediatria,VilaNovadeFamalicão,Portugal}

a

r

t

i

c

l

e

i

n

f

o

Articlehistory: Received2March2015 Accepted14August2015

Availableonline11December2015

Introduction

Lymedisease (LD)isaninfectiousdisease causedby spiro-chetesofthegenusBorreliaandtransmittedbytickbite.

TheclinicalpresentationofLDisdividedintothree sep-arated phases: an early localized disease,characterized by erythemamigrans(EM),anearlydisseminateddiseasewith potential involvement of the central nervous system and heart, and a late stage of disease with monoarticular or oligoarticulararthritisoflargejoints.Duringtheearlyphase, oral antibiotics and, in the case of disseminated disease withneurologicalorcardiacdamage,intravenousantibiotics, areindicated.ThearthritisofLD(latestage)shouldbe ini-tiallytreatedwithoralantibioticsforamonth;intravenous

夽

StudyconductedatServic¸odePediatria,CentroHospitalardoMédioAve,VilaNovadeFamalicão,Portugal. ∗ _{Correspondingauthor.}

E-mail:mario.s.sa@gmail.com(M.C.Sá).

treatmentshouldbelimitedtopatientswithsevereor persis-tentdisease.

Several studies have suggested a possible influence of severalinfectiousagents,includingBorrelia,inthe etiopatho-genesisofJuvenileIdiopathicArthritis(JIA).1

Case

report

A 6-year-old female patient with no relevantpast medical historywasreferredtoPediatricRheumatologyconsultation, withcomplaintsofpainandswellingoftheproximal inter-phalangealjoints(PIP)ofhandsandwristandtibiotarsaljoints bilaterallywithseveralmonthsofdevelopmentand progres-siveworsening.Therewasnofeverorhistoryoftrauma.She

http://dx.doi.org/10.1016/j.rbre.2015.09.006

2255-5021/©2015ElsevierEditoraLtda.ThisisanopenaccessarticleundertheCCBY-NC-NDlicense(http://creativecommons.org/

rev bras reumatol.2017;57(6):620–622

621

Fig.1–Proximalinterphalangealjointswithinflammatory arthritisobservedinaPediatricRheumatologyconsultation.

residedinanurbanenvironment innorthernPortugal,but withregularvisitstorelativeslivinginaruralarea,whereshe hadcontactwithdogs.Thefamilyhistorywasirrelevant.

Thegirl showedmultiplecircinateerythematouslesions of2–5cm diameter, with a 5-month progression, and was refractorytooralandtopicalantifungaltreatment.At phys-icalexamination,thepatienthadsignsofinflammationand limitationonactiveandpassivemobilizationofallPIPjointsof herhands(Fig.1)andoftheknee,tibiotarsaljointsandwrists bilaterally;andalsomultiplecircinateerythematouslesions withirregularshapes,scatteredonthetrunk,upperandlower limbsandneck(Fig.2).

Given the clinical picture suggestive of LD, antibiotic therapy was instituted with oral amoxicillin 1.5g/day and ibuprofen30mg/kg/dayfor21days.

The laboratory investigation showed normal complete bloodcount(CBC)andgeneralbiochemistry(renalfunction,

Fig.2–Multiplecircinateerythematouslesionswith irregularshapes,scatteredonthetrunkandupperlimbs.

ALT,AST,alkalinephosphatase,thyroidfunction,ionogram); erythrocyte sedimentation rate 24mm/1st hour, C-reactive protein1mg/dL,immunologicalstudieswithANA,ANCAand rheumatoid factor negative, C3 and C4 slightly increased (184mg/dL and 46mg/dL, respectively), and serology (HIV, CMV,EBV,toxoplasma,VDRL,Weil-FelixreactionandWright reaction)negative.SerologicalstudiesforBorreliaburgdorferi werepositive(indirectimmunofluorescence,IgG53.30AU/mL [positive,>10AU/mL];IgM:1.37[positive,>1.09]).Intheface ofsuchresults,the diagnosisofEMand arthritisina con-textofLymediseasehasbeenconfirmed.Thechildshowed nochangesinherheartandeyeexamination.

Inspiteofthetreatment,thechildrelatedthesame com-plaintsofarthralgia,withtheappearanceofnewlesionsof EM.Acycleof28daysofintravenousceftriaxone2g/daywas thenintroduced,withcompletedisappearanceofskinlesions. The resolution of joint symptoms was only temporary; about two months later, re-aggravation of arthralgia com-plaintsandmobilitylimitationofwristandPIPjointsoccurred. Therewasnorecurrenceofothersignsofarthritisandeven ofEM.

Taking into account the persistence of the signs and symptomsofchronicarthritis,anti-inflammatorytreatment withoraldeflazacort(7.5mg/day)andnaproxen(500mg/day) and also immunosuppression with oral methotrexate (14.5mg/m2_{/week)were initiated.Thenthechildpresented} a progressive improvement of her pain complaints, but with persistence of a slight limitation to wrist extension. Analytically, there were no new changes. Currently, the patientremainsinremission,dependingonthistherapy;her behaviorandresponsetotherapyareabsolutelyidenticalto what happens withJIA. Worsening ofcomplaintsoccurred whenareductionofmedicationwasattempted.

Discussion

LDispredominantlycausedbyBorreliaburgdorferiand, espe-cially inEurope, byBorrelia afzelii and Borrelia garinii. Being consideredasazoonosis,LDistransmittedbytickbite, com-monlyofIxodesricinus.

With significant variations among different geographic areas,theincidenceofLDhasincreasedinasustained man-nerinthelastyears.2_{InPortugal,theincidenceis0.3casesper} 100,000inhabitants,closetowhathappensinotherEuropean countries.3

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obviousthantheassociatedpain.Often,thearthritisis migra-tory,andiftheantibiotictherapyisnotstarted,thecomplaints willpersistoverseveralweeks,followedbyspontaneous res-olution.However,theproblemmayrecurinanotherjoint.6

Unusual developments have been described by several investigators, notablythe development of joint complaints toward chronicity in an important proportion of pediatric patients,7_{andthepresenceoferosivearthropathyinchildren} unresponsivetoantibiotictreatment.8

ThepresenceofEMinanindividualwhoresidesorrecently traveledtoanendemicareaissufficientforthediagnosisofLD. Ontheotherhand,inthecaseofsymptomscompatiblewith disseminatedorlatedisease,serologicalconfirmationshould beperformedpriortotheinstitutionofantibiotictherapy.4

The antibiotic of choice in cases of localized disease is doxycycline,amoxicillin or cefuroxime-axetil orally in a courseof14–21days.Intravenousantibiotictherapyis indi-catedincasesofcardiacorneurologicalmanifestation,except forthoseisolatedcasesoffacialparalysis.Lymearthritiscan betreatedsuccessfullywithoraldoxycyclineoramoxicillinfor onemonth;however,insomecasesanintravenoustreatment mayberequired.4

TheassociationbetweenJIAandenvironmentalandother factorssuchasinfection,breastfeeding,immunization, etc. wasalreadymentioned.9 _{Thus,itisbelieved thatthe} pres-enceofoneormoreriskfactors,suchasaBorreliainfection ina genetically susceptibleindividual, may triggera clini-cal picture ofJIA.1 _{However, morestudies are stillneeded} toestablisharelationshipbetweenthisenvironmentalfactor andJIA,andalsotodeterminewhatistheactualpathogenic roleofdifferentenvironmentalriskfactorsintriggeringthis disease.

Inthis case,the childwasfoundwith skinlesions sug-gestive ofdisseminated disease; however, the evolution to polyarticular arthritis is not characteristic of Lyme arthri-tis (which typically is of monoarticular or oligoarticular type),but herclinical picture isstronglysuggestive of pol-yarticular JIA. The cutaneous manifestations disappeared after treatment, with persistence only ofchronic arthritis, which was controlled with anti-inflammatory agents and methotrexate.

Thepresentedevolutionsuggestsastronglikelihoodthat theinfectionbyBorreliawasthetriggeringeventofJIAinthis patient.

Conflicts

of

interest

Theauthorsdeclarenoconflictsofinterest.

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