Rev. Bras. Reumatol. vol.57 número6

r e v b r a s r e u m a t o l . 2017;57(6):610–612

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

Presence

of

riziform

bodies

in

a

patient

with

juvenile

idiopathic

arthritis:

case

report

and

literature

review

夽

Presenc¸a

de

corpos

riziformes

em

paciente

com

artrite

idiopática

juvenil:

relato

de

caso

e

revisão

de

literatura

Leonardo

Rodrigues

Campos

_,

_Fernanda

_Cardoso

_das

_Neves

_Sztajnbok

_,

_Stélio

_Galvão

_,

Marise

de

Araújo

Lessa

_,

_Ierecê

_Lins

_Aymoré

_,

_Flavio

_Sztajnbok

a_{UniversidadeFederaldoRiodeJaneiro,RiodeJaneiro,RJ,Brazil}

b_{UniversidadedoEstadodoRiodeJaneiro,FaculdadedeCiênciasMédicas,RiodeJaneiro,RJ,Brazil}

c_{HospitalMárioKroeff,LaboratórioCláudioLemosAnatomiaPatológicaLtda.,RiodeJaneiro,RJ,Brazil}

d_{UniversidadedoEstadodoRiodeJaneiro,NúcleodeEstudosdaSaúdedoAdolescente,RiodeJaneiro,RJ,Brazil}

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Articlehistory: Received17April2014 Accepted14September2014 Availableonline28November2014

Introduction

Juvenileidiopathicarthritis(JIA)isthe mostcommon form ofchronicarthritisinpediatricpatients,anditsdiagnosisis madefollowingexclusionofseveralotherconditionsthatmay presentwithprolongedmusculoskeletalmanifestations.The mostcommon form ofJIAis the oligoarticular one,which maypresentasachronicmonoarthritis.1_{Inthesecases,there} aremultiplediagnosestobeinvestigated:tuberculosis, sar-coidosis,villonodularsynovitis,hemarthrosis,hemangioma, synovial osteochondromatosis, arborescent lipoma, malig-nanciesandsomeautoinflammatorydiseases.2–4_Ourgoalis

夽

WorkcarriedoutintheDepartmentofRheumatologyoftheCenterforAdolescentHealthStudies,UniversidadedoEstadodoRiode Janeiro,RiodeJaneiro,RJ,Brazil.

∗ _{Correspondingauthor.}

E-mail:[email protected](F.Sztajnbok).

toreportthecaseofachildwithchronicmonoarthritiswhose biopsyshowedagreatamountofriziformbodiesthatare sel-domdescribedinthisagegroup.Thisisthe9thcasereported inliteratureaboutthepresenceofriziformbodiesinpatients withJIAand,toourknowledge,thefirstcasereportedinBrazil.

Case

report

Amale8-year-oldchild,2ndtwin,borninthecityofRiode Janeiro,hadahistoryofdifficultypracticingexercises,with limitedrangeofmotionoftheleftknee,approximatelyfour monthsbeforethe1stconsultation.Afteramonth,theparents

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rev bras reumatol.2017;57(6):610–612

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noticedagreatincreaseinvolumethat remaineduntilthe dayofconsultation.Withinthisperiod,therewasnofever, skin lesions or any signs or symptoms of involvement of other organs. There was no previous history oftrauma or infectioninthethreemonthsbeforetheonsetofsymptoms. Hehasahealthy twinbrother,thereisnofamilyhistoryof spondyloarthritis,and the mother had a facialskin lesion about 25 years ago, diagnosed as sarcoidosis, and treated withintralesionalcorticosteroids.Physicalexaminationinthe 1stconsultationwasnormalexceptforthepresenceoflarge swellingoftheleftknee,withheatandslighthyperemia,piano keysignandmotionlimitation(flexionat60◦_andextension

at150◦_).

Withthe syndromic diagnosis ofchronic monoarthritis, testswereordered,theresultsofwhichshowedbloodcount, C-reactiveprotein,erythrocytesedimentationrate,lipid pro-file, blood glucose, calcium, urea, creatinine, complement,

Fig.1–Magneticresonanceofleftkneeshowedbulkyjoint effusionandpresenceofmultiplesmallelongatedimages withhypotensesignalinallsequences,suggestiveof riziformbodies.

Fig.2–Macroscopicaspectofleftkneesynovium,showing moderateamountofwhitishmass,similartoricegrains.

muscle enzymes, proteinelectrophoresis, angiotensin con-verting enzyme(ACE)and urinary sedimentwithinnormal ranges. Therheumatoid factor, antinuclear antibodies and HLAB-27werenegative.SerologicmarkersforhepatitisBand C,toxoplasmosis,HIVandHTLVwerealsonegative.Serology for rubellaand cytomegalovirusshowed negative IgM.The tuberculintestwasnegativeandchestradiographwasnormal. Also,hiselectrocardiogram,echocardiogram,and ophthalmo-logicevaluationwerenormal.LeftkneeX-rayshowedonlysoft tissueswelling,andultrasonography(USG)showedjoint effu-sionwithdebrisandsynovialthickening.Magneticresonance imagingshowedmassivejoint effusionwithheterogeneous signalinthepresenceofmultiplesmallelongatedimageswith hypotensivesignalintensityonallsequences,suggesting riz-iformbodies,andpronouncedenhancementofthesynovium (Fig.1).Leftkneesynovialbiopsywasrequiredand,insurgery, there wasoutput ofminimalamount ofsynovialfluid (SF) andamoderateamountofwhitemass,similartoricegrains (Fig.2).Anatomicpathologyshowed,onmacroscopic exam-ination, the presence ofnumerous oval, friable, white and firmstructuresconsistentwiththediagnosisofriziform bod-ies.Microscopyshowedsynoviumfragmentswithhyperplasia ofliningcells,andextensivedepositsoffibrin,withedema, vascularectasiaandneogenesisseeninthestroma,and lym-phocytic inflammatory infiltrateoccasionally aggregated in follicles,andsomegranulocytes(Fig.2).Thematerial repre-sentedbyriziformbodiesconsistedoforganizedfibrinandhad pervadingmononuclearcells,andsomepolymorphonuclear cells.Thehistologicalreportwassuggestiveofchronic inflam-matory synovitis, or JIA. The patient was initially treated, beforesurgery,withnon-steroidalanti-inflammatorydrugs, with no clinical response.After surgery,methotrexate was addedtothetreatmentand,inabouttwomonths,thepatient wasasymptomatic.

Discussion

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outsarcoidosis,notconfirmedbyhistopathology.Atsurgery, a small amount of synovial fluid was found, along with the presence ofa moderate amount of whitish mass that histopathologyshowedtoberiziformbodies.

Riziform bodies are structures which can be found in synovialfluidoradheredtosynovium,andhavethis denom-ination for its similar appearance to the rice grains. They consistoffibrininvolvingmononuclearcells, polymorphonu-clearcellsand red bloodcells,and representa nonspecific responsetosynovialinflammation.5–8_{Theyhavealreadybeen} described in several diseases suchas tuberculous arthritis (where they were originally first reported in 1895), other infectiousarthritis, osteoarthritis,rheumatoid arthritisand JIA.2–11

Itsetiopathogenesisiscontroversial.Theycanarisefrom areas of microinfarctions of inflamed synovium that are releasedinto theSFandencapsulatedbyfibrin,abundantly producedininflammatoryprocessesofthesynovium.7,9,10,12 Thereisalsoanothertheorythatsuggeststhatriziform bod-iesareformeddenovointheSF,quiteinterestingforcasesof osteoarthritisandthepresenceofapatitecrystalsandcalcium pyrophosphate.4,8 _{Rovenska et al. suggestedthat although} thereisaphysiologicallymphoangiogenesisassociatedwith chronicinflammationinordertoimprovedrainageof exces-siveSF,theformationofriziformbodiescanbeassociatedwith thedifficultyoflymphaticdrainageoftheinflamedsynovial fluid.13 _{Theriziformbodiesoccur moreoften inknees and} shouldersand wecould saythattheyare theendproducts ofinflammation,synovialproliferationanddegeneration.9_On theotherhand,thereisadescriptionofriziformbodiesinthe pleuralfluid,tendonsheathsandbursae,suggestinga possi-bilityofnonsynovialorigin.2,8,14

ThefindingofriziformbodiesinJIAwasfirstreportedby Wynne-Robertsetal.in1979inan17-year-oldadolescent12 and, after, we found 7 more cases reported in the litera-ture,withagesrangingfrom2to17years.5–7,9–11_Thisfinding appearstobeindependentfromarthritisseverityandlength ofthedisease.7,9,10,15 _{Inthe casesreportedinchildrenand} adolescents,thetimebetweentheonsetofarthritisandthe findingofriziformbodiesrangedfrom2monthsto5years, butnotallreportscontainthisinformation.Inourcase,we observethattheappearanceoccurredafteronly3monthsof theonsetofarthritis.

Itwouldbeinterestingtoconductsynovialbiopsytocheck forthepresenceofriziformbodieswhenevertheUSGshow thefinding ofdebris.Thedifficulty ofSFsuctioninajoint containinga largeeffusion may bedue tothe presenceof riziformbodiesandthereforetheuseofathickerneedleor asurgicalapproachmaybenecessary.10_{Theperformanceof} synovialbiopsyforcheckingthepresenceofriziformbodiesis important,especiallyincasesofarthritisthatisbulkyand/or unresponsivetoconventionaltreatment,asitswithdrawalcan causesymptomaticreliefforthepatient.7

Ourobjectivewastodescribewhatwebelieveisthe9th casereportedaboutthepresenceofriziformbodiesinJIA,and itspresenceshouldbemorefrequentthanisreportedinthe

literatureandweareusedtofind.9_{Inrheumatoidarthritis,} theycanbefoundin72%ofjoints,whensearched.15_In gen-eral, we donotorder synovialbiopsy inother typesofJIA otherthanthemonoarticularand,withthat,weareprobably under-diagnosingthepresenceofriziformbodies,whichmay beresponsibleforlargearthritisofdifficultclinicaltreatment, butthatrespondwelltosurgicalintervention.

Conflict

of

interest

Theauthorsdeclarenoconflictsofinterest.

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