Osteonecrosis of the distal tibia in systemic lupus erythematosus

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CASE REPORT – OPEN ACCESS

InternationalJournalofSurgeryCaseReports77(2020)126–128

ContentslistsavailableatScienceDirect

International Journal of Surgery Case Reports

jo u r n al hom e p a g e :w w w . c a s e r e p o r t s . c o m

Osteonecrosis of the distal tibia in systemic lupus erythematosus:

A rare case report

Ihsan Oesman, Danarto Hari Adhimukti

^∗

DepartmentofOrthopaedicsandTraumatology,FacultyofMedicine,UniversitasIndonesia,CiptoMangunkusumoHospital,Jakarta,Indonesia

a rt i c l e i nf o

Articlehistory:

Received29September2020 Accepted17October2020 Availableonline28October2020

Keywords:

osteonecrosis distaltibia

systemiclupuserythematosus

a b s t ra c t

INTRODUCTION:Osteonecrosis(ON)ischaracterizedbycellulardeathofbonecomponentsduetointer- ruptionofbloodsupplythatleadstoboneischemiaandpotentialjointdestruction.Therearemultiple riskfactorsandmedicalconditionassociatedwithON,includingsystemiclupuserythematosus(SLE).

ThemostcommonsitesofONarethefemoralhead,distalfemur,proximalhumerus,talusandlumbar spine.VeryfewcasesofnontraumaticONindistaltibiahavebeenreportedintheliterature.

CASEILLUSTRATION:Wepresentacaseof23-year-oldfemalediagnosedwithosteonecrosisofdistaltibia andhistoryofSLE.Thepatientalsohadhistoryofavascularnecrosisofrighthipandunderwentright totalhiparthroplasty.Wetreatedthepatientswithconservativetreatmentforintialmanagement.

DISCUSSION:TheriskofONinSLEpatientsislikelyduetotheresultsofboththeSLEitselfanduseofcor- ticosteroids.SystemicinﬂammationinSLEreducesthedevelopmentofosteoblasts,increasesosteoclast maturationandactivityandincreasesprotohromboticagentsthatcanleadtorapidboneloss.Corticos- teroidsarethemostconsistentriskfactorassociatedwiththedevelopmentofONinSLE.Conservative medicalmanagementiseffectiveintheearlystagesofthediseasebeforebonecollapse.

CONCLUSION:DespiteadvancesinthediagnosisandtreatmentofSLE,symptomaticONcontinuestobe asigniﬁcantcomorbidity.StrategiestodetectandmanageearlystageONisnecessarytopreventthe progressionofthisseriouscomplication.

1. Introduction

Osteonecrosis (ON),also knownasavascular necrosisof the bone,ischaracterizedbycellulardeathofbonecomponentsdueto interruptionofbloodsupplythatleadstoboneischemiaandpoten- tialjointdestruction.TheexactmechanismofONisstillunclear, somemechanismsinvolvedincludeischaemia,vascularocclusion, intraosseousmicrocirculationcoagulation,andmechanicalstress [1].Therearemultipleriskfactorsandmedicalconditionassociated withON,includingtrauma,alcoholabuse,smoking,vasculardis- ease,renaldisease,coagulationdisorders,andrheumaticdiseases, especiallyinsystemiclupuserythematosus(SLE)[1,2].

Systemiclupuserythematosus(SLE)isachronic,multisystem autoimmunediseasewithunknownaetiologythatpredominantly affectswomen.Severalfactorshavebeenassociatedwiththedevel- opmentofosteonecrosisinSLEbutthemostconsistentassociation iscorticosteroid(CS)therapy[3].Osteonecrosisoccursmainlyin SLEpatientswhoaretreatedwithcorticosteroidsandisextremely rareamongSLEpatientswhohadneverreceivedcorticosteroids

∗Correspondingauthor.

E-mailaddress:danardokter88@gmail.com(D.H.Adhimukti).

[4].ThereportedprevalenceofsymptomaticosteonecrosisinSLE is4%–15%andupto40%inasymptomaticpatients[5].Osteonecro- sismostoftenaffectsyoungadultsaged30–50years.MRIisthe goldstandarddiagnosticmethodtodetectbothsymptomaticand asymptomaticON[6].

The mostcommon sites of ON are the femoral head, distal femur,proximalhumerus,talusandlumbarspine[6].X-rays,MRIs, andscintigraphyarehelpfulmethodsfordetectinglesionsfrom osteonecrosis.ONinmultiplesitesisrare,beingreportedin3.3%

ofpatientswithON.MultipleosteonecrosisinpatientswithSLEis alsounusual.Thetermmultifocalosteonecrosisisusedtodescribe thepresenceofosteonecroticlesionsinthreeormoreanatomical sites[1].FewcasereportshavedescribedONaffectingtheankle, andthetalusisthemorecommonsiteofinjurythanthedistaltibia [2].VeryfewcasesofnontraumaticONindistaltibiahavebeen reportedintheliterature.Inthisstudy,wepresentacasereportof patientwithosteonecrosisofdistaltibiaduetohistoryofSLE.This paperhasbeenwrittenaccordingtotheSCARE2018statement[7].

2. Casereport

A23-year-old femalepresented withworsening painin the leftankleinthelast4weeksbeforeadmission.Patientpreviously

https://doi.org/10.1016/j.ijscr.2020.10.069

org/licenses/by/4.0/).

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CASE REPORT – OPEN ACCESS

I.Oesman,D.H.Adhimukti InternationalJournalofSurgeryCaseReports77(2020)126–128

treatedbyinternistwithcomplaintsofpainandswellingintheleft ankleinthelast2months.Paindidnotdecreasewithpainkillers and wereaccompaniedbyrednessoftheankles,warmnessand difﬁcultiestomove.Therewasnohistoryoftraumaandfever.The patientwasgivenantibioticsandthepainwasreducedbutstillfelt intermittently.

ThepatientwasdiagnosedwithSLEwithkidneyinvolvement 3yearsago.Akidneybiopsywasperformedwithresultoflupus nephritis.Patientconsumedmethylprednisolone2×4mgevery2 days.Twoyearsago,patientcomplainedofrightpelvicpainwith difﬁcultyinwalkinganddiagnosedwithavascularnecrosisdueto SLE.RighttotalhiparthroplastywasdoneatCiptoMangunkusumo Hospital.

Fromthephysicalexamination,theleftankleis swollenand hyperemic.Thereisnoopenwoundordischarge.Plantarﬂexion- dorsoﬂexionislimitedduetopain(5–20degree).Distalsensory andmotoricarewithinnormallimit.

Thelaboratoryresultshowedanincreasedleukocyte,C-Reactive Protein, and D-dimer. Others components were within normal limit. From radiological examination, there was multiple lytic lesionsinthedistal epi-meta-diaphysisofthelefttibia,accom- paniedbyasolidtypeperiostealreactiononthepostero-medial sideofthemetaphysisonthesideoftheleftdistaltibia.MRIofleft ankleshowedmultifocalappearanceofosteonecrosisinthedis- talepimethaphysisofthetibia,talus,calcaneusandleftﬁrstshaft metatarsal.

3. Discussion

Osteonecrosis can be classiﬁed into post-traumatic and nontraumatic. Post-traumatic osteonecrosis usually caused by traumaticdisplacementofbonefragments,whichleadstoimpaired bloodsupplyandischemiatotheaffectedbone.Ontheotherhand, nontraumatic osteonecrosis associated withvariety ofsystemic diseasesandclinicalconditions[8].Inthisstudy,wepresentacase ofa patientwithosteonecrosisofdistaltibiawithouthistory of trauma.Patienthasbeensufferedfromsystemiclupuserythemato- sussince3yearsagowithnootherdisease.Amongthesystemic diseases,osteonecrosisisstronglyassociatedwithSLE.Osteonecro- sisisoneoftheseriouscomplicationsforSLE[9].

ThepathophysiologyofONisnotcompletelyunderstood.Itis believedtoberesultofthecombinedeffectsofmetabolicfactors, geneticpredisposition,andseveralfactorsaffectingbloodsupply [10].MechanismsthathavebeenproposedfornontraumaticON includeincreasedintraosseouspressure,arterialemboliorthrom- bosis,venousocclusion,andcoagulationdisorder[11].Theriskof ONinSLEpatientsislikelytheresultofboththeSLEdiseasestate itselfand theuseofcorticosteroidsasmedication[8].Incidence ofONinSLEpatientswasaffectedbydiseaseactivity,thehigher diseaseactivityscoreissigniﬁcantlyassociatedwithaccelerated incidence ofON,while lowerdiseaseindicatescomparable safe statusforON[9].Theoveralleffectofglucocorticoidsonboneis multifactorial.

There are some proposed potential mechanisms. Systemic inﬂammation in SLE reduces the development of osteoblasts (byproducingoxidizedLDL),causesapoptosisofosteoblastand increases osteoclast maturationand activity(byincreasingTNF levels). Increased osteoclast maturationand reduced osteoblast maturation/activitymakerapidbonelosscanoccur[6].Systemic inﬂammation also increases protohrombotic agents (homocys- teine),whichmightinducethrombosisofsmallbloodvesselscould leadtocellularnecrosisofanumberofosteocytes.Thisnecrosis mightbefollowedbylocalizeddemineralizationoftheboneand fracturescanoccurinthisweakenedbone[11].Inourcase,sys-

temicinﬂammationmarkedbyelevatedleukocyteandC-reactive protein.

Corticosteroids (CS) are the most common cause of non- traumaticONaswellasthemostconsistentfactorassociatedwith thedevelopmentofONinSLE.AlthoughtheeffectofCSonON seemstobeclearlyestablished,thepathophysiologyisnotfully understood.1Theeffectofcorticosteroidsatthecellularleveland theirinﬂuenceontheimmunesystemisoneoftheprincipallypro- posedmechanismsforthedevelopmentofON[1].Glucocorticoid receptorshavebeenfoundincartilage,osteoblasts,osteoclasts,and osteocytes.Bindingofglucocorticoidstothesereceptorshasbeen shownto induceananti-inﬂammatoryresponse through apop- toticpathwayswithin theimmunogenic cells.Thus, osteoclasts andosteoblastscanundergoapoptosisafterprolongedtreatment withglucocorticoids[2].Apoptosisoftheosteoblastsandosteo- clasts, a decreased bone turnover and a decreased survival of osteocytesappeartobeimportantmechanismsforinducingON [1]. ItalsohasbeenhypothesizedthatchronicCSusepromotes intraosseousadipocytehypertrophyanddepositionoffatwithin theintramedullarytissue,whichcauseselevationofintracortical pressureandcompromisesperfusion.Anotherpostulatedmecha- nismisthatCSalterlipidmetabolism,leadingtofatmicroemboli insubchondralvessels[10].Thedurationofcorticosteroidtherapy, total cumulative dose and highest daily dose have been inde- pendentlyassociatedwiththedevelopmentofON.4Themedian durationofglucocorticosteroid usepriortothedevelopmentof osteonecrosiswas3.4years,showingthatashortexposureofCS maybeassociatedwithosteonecrosis[5].

TheclinicalpresentationofONcanbemanifestasasymptomatic orpresent withgradual-onsetpain that canprogress tosevere pain,bonecollapse,andjointdamage.Theseconditionscanleadto restrictionoftherangeofmotioninvolvedjoints[10].Bilateralhip involvementoccursinupto90%ofSLEpatientswithON.Themost commonlyaffectedotherjointsincludethekneeandshoulder,with incidenceof10%–20%.Lesscommonly,theankleandelbowcan beinvolved[11].MultifocalONaffectingmorethanthreeofthese jointsisnotinfrequentinSLEpatients,withapproximately3%incidence[8].Inthisstudy,ourpatientalreadyunderwentrighttotal hiparthroplastyduetoAVNandbegansufferingpainintheleft anklewithswollenandhyperemicsofttissues.

DiagnosisofosteonecrosisinSLEcanbemadebyradiography ﬁndingsbutalwaysbeginswithathoroughassessmentforcom- monrisk factorsassociated withON andphysical examination.

Radiographicevaluationshouldbeginwithstandardradiographs ofallsymptomaticjoints.Inaddition,patientswithONshouldhave theirfemoralheadsevaluatealthoughsymptomsoccurredinother jointsbecauseofthehighincidenceoffemoralheadinvolvement (greaterthan80%)inmultifocaldisease[11].Themostcommonly usedradiographicstagingmethodistheFicatandArletsystem, whichwasoriginallyappliedtothefemoralheadbutcanbeapplied toanyinvolved bone.Plainradiographscanassessforevidence ofarticular-surfacecollapse.Magneticresonanceimaging(MRI)is thegoldstandardandcanbeusedfordiagnosingearly-stageON lesions(sensitivityandspeciﬁcity>99%)[8].FromMRIexamina- tion,ourpatienthasmultifocalappearanceofosteonecrosisinthe distalepimethaphysisofthetibia,talus,calcaneusandleftﬁrstshaft metatarsal.Tothebestofourknowledge,thisisaveryrarecase.

Conservativemedicalmanagementisthefirstlineofnontrau- maticONtreatment, whichismosteffectiveintheearlystages of the disease before bone collapse. The medical management approachistoreducesystemicinflammationanduseofglucocor- ticoids,asbothareriskfactorsforosteonecrosis,andtoimprove thevasculature totheaffected bone area[6]. Theconservative managementofONincludedpharmacologicaltreatment,modifi- cationofactivitywithrestrictedweightbearing,andtheusedof ultrasoundorelectricsignalstostimulatesrepair.Medicationsthat 127

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I.Oesman,D.H.Adhimukti InternationalJournalofSurgeryCaseReports77(2020)126–128

havebeeninvestigatedinseveralstudiesandhavebeenusedin patientswithONincludedbisphosphonates(inhibitionofosteo- clastactivity),statins(pro-osteoblasticandanti-adipogeniceffects onbonemarrowstromalcells),heparin(stabilizationthebalance betweentheﬁbrinolyticandthromboticcascades),antimalarials (antithromboticandlipid-loweringproperties)andsupplementa- tionwithfolicacid(reducehomocysteinelevels)[2,10].

In general, joint-preserving procedures are the ﬁrst line of operative treatment for pre-collapse lesions. Several operative treatmentsintheearlystagesfornontraumaticONoftheankle included coredecompression,vascularized andnonvascularized bonegrafting,tibiotalarfusion,andtalectomywithtibiocalcaneal fusion.Theseprocedureshavebeenusedasatreatmentmethod torelieveintraosseuspressure,increasebloodﬂowtothenecrotic subchondralbone,andinsomecasesprovidestructuralsupportfor theunderlyingsubchondralbone.Inend-stageofnontraumaticON ofankle,whensubchondralcollapsehasoccurred,itusuallyleads toanklearthrodesis[2,8].

4. Conclusion

AlthoughONisoftensecondarytotrauma,therearesomeother etiologies includingSLE.Despiteadvancementsinthediagnosis andtreatmentofSLE,symptomaticosteonecrosiscontinuestobe a signiﬁcantcomorbidity.Strategies todetectandmanageearly stages ofONisnecessary topreventprogressionofthis serious complication.

Declarationofcompetinginterest None.

Funding None.

Ethicalapproval Notrequired.

Consent

Writteninformedconsentwasobtainedfromthepatientfor publicationofthiscasereportandaccompanyingimages.Acopy ofthewrittenconsentisavailableforreviewbytheEditor-in-Chief ofthisjournalonrequest.

Authorcontribution

IhsanOesman:performingtheprocedure,datacollection.

DanartoHariAdhimukti:performingtheprocedure,datacollection,writingthepaper.

Registrationofresearchstudies Notrequired.

Guarantor IhsanOesman.

Provenanceandpeerreview

Notcommissioned,externallypeer-reviewed.

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