Rev. Bras. Reumatol. vol.57 número6

r e v b r a s r e u m a t o l . 2017;57(6):623–625

w w w . r e u m a t o l o g i a . c o m . b r

REVISTA

BRASILEIRA

DE

REUMATOLOGIA

Case

report

Relapsing

polychondritis

and

lymphocytic

meningitis

with

varied

neurological

symptoms

Policondrite

recidivante

e

meningite

linfocitária

com

sintomas

neurológicos

variados

Renata

Dal-Prá

Ducci

_,

_Francisco

_Manoel

_Branco

_Germiniani

,

Letícia

Elizabeth

Augustin

Czecko

_,

_Eduardo

_S.

_Paiva

_,

_Hélio

_Afonso

_Ghizoni

_Teive

a_{UniversidadeFederaldoParaná,HospitaldasClínicas,DepartamentodeMedicinaInterna,Servic¸odeNeurologia,Curitiba,PR,Brazil} b_{UniversidadeFederaldoParaná,HospitaldeClínicas,DepartamentodeMedicinaInterna,Servic¸odeReumatologia,Curitiba,PR,Brazil}

a

r

t

i

c

l

e

i

n

f

o

Articlehistory:

Received14March2015

Accepted25September2015

Availableonline17March2016

Introduction

Relapsingpolychondritisisarareautoimmunedisease,1,2_with

afemale–maleratioof2:4,3_{withsymptom’sonsetbetween20}

and60yearsofage(peakincidencearound40y/o),4

charac-terizedbybilateralauricularandnasalchondritis,vestibular

involvementand varied systemic symptoms due to

recur-rentandprogressiveinflammationofcartilaginoustissueand

proteoglycan-richstructuresinvarioussitesofthebody.1,2_We

reportthecaseofapatientwithrelapsingpolychondritiswith

severalneurologicalmanifestations.

Case

report

A69year-oldmalewithdiabetes,hypothyroidismand

dysli-pidemiahadatwo-monthhistoryofswellingandpainofboth

earlobesandedemaandarthralgiaofmetacarpophalangeal

∗ _{Correspondingauthor.}

E-mail:frankgerminiani@hotmail.com(F.M.Germiniani).

joints and ankles,aswell asgeneralized painwith a

wax-ingandwaningcourse.Twentydayspriortohisadmissionin

ourhospitalhestartedwithataxia,paraparesis,tinnitus,

ver-tigoandconfusion.Hehadbeenpreviouslyseenatanother

hospital,aroundthetimeofconfusiononset,wherehewas

treatedforherpeticencephalitisfollowingalumbarpuncture

which disclosed elevated leukocytes, witha predominance

oflymphocytes.Althoughatfirsthehadanimprovementof

confusion,hisparaparesisremainedunaffected.Onphysical

examinationhehadnystagmusinthedownwardgaze,rigidity

ofupperlimbs,paraparesis,absentreflexes,tactile

hypoesthe-sia,dysmetricmovements,grossposturalandactiontremor,

bradykinesia and truncal ataxia. He alsohad swelling and

a purplish erythemaofboth earlobes and arthritisin the

metacarpophalangealjointsofthesecondandthirdfingers

oftherighthand(Fig.1).BrainandcervicalMRIdiscloseda

mildthickeningofthedura(Fig.1).Anewlumbarpuncture

confirmed the presenceof elevatedleukocytes and

labora-toryexamsdisclosedaugmentedinflammatoryactivity and

http://dx.doi.org/10.1016/j.rbre.2016.02.005

2255-5021/©2016ElsevierEditoraLtda.ThisisanopenaccessarticleundertheCCBY-NC-NDlicense(http://creativecommons.org/

624

Fig.1–Rheumatologicalclinicalfindings(upperimages):(A)edemaofmetacarpophalangealjoints;(B)earlobechondritis. BrainMRIfindings(lowerimages):(C)axialdiffusionwiththickeningoftheduraofbothfrontallobes;(D)sagittalT1FSE FATSATwithincreasedmeningealsignaladjacenttothecerebellum;(E)T2FSEshowingmildcerebellaratrophy.

iron-deficientanemia(Table1).Adiagnosisofrelapsing

poly-chondritiswasmadebasedontheassociationofchondritis,

arthritisandvestibularataxiawithpredominant

neurologi-calsymptoms.FollowingacourseofPrednisone1mg/kgqd

therewasmajorimprovementofchondritis,arthritis,ataxia

and paraparesis, but the tremor remained unchanged. On

a one-year follow-up visit he had developed several

com-plicationsofchroniccorticosteroiduse,suchasosteopenia,

hypertension,Cushing’ssyndrome,worseningofobstructive

apneasyndromeandoneepisodeofbilateralcutaneous

Her-pes Zoster of the trunk. These complications warranted a

changeofimmunosuppressivetreatmentfromPrednisoneto

Methotrexate.In spiteofthis,hehad no newneurological

symptomsandremainedwithmoderateataxia.

Discussion

Relapsingpolychondritisisararemultisystemicautoimmune

disease that affects cartilaginous tissue, especially hyaline

cartilages at multiple sites, most often compromising the

antihelixofbothearlobes,withsparingofthelobule.2,5

Sero-negative polyarthritis and systemic compromise of other

organsmayalsooccur(includingocularinflammation,

audio-vestibularimpairment,vasculitis,skininvolvement,valvular

insufficiency and neurological symptoms) due to

compro-miseofproteoglycan-richtissues.6,7_{Around30%ofthecases}

are associated with concurrent autoimmune disease,

sys-temicvasculitisandmyelodysplasticsyndrome.5,8_Diagnosis

ismadeonclinicalgrounds,occasionallywithpathology

dis-closing inflammatory compromiseof affected cartilaginous

tissue.5,7 _{Currently, the diagnosis is made on the basisof}

demonstrationofeitherchondritisintwoofthreesites

(auric-ular, nasal,laryngotracheal); or oneofthese sitesand two

additional features, including ocular inflammation,

audio-vestibulardamage,orsero-negativeinflammatoryarthritis.1,9

There are no specific laboratory findings.5,7 _Neurological

symptomsoccurinaminorityofcases(3%)andmayrange

from compromiseofcranialnervestoamoreovert

presen-tation with cerebellar compromise, seizures or other focal

findings suggestive ofcorticalcompromise. These are

vas-culitic in nature. Aseptic meningitis, with thickening of

themeninges,lymphocyticmeningoencephalitis,

rhomboen-cephalitisandcerebralaneurysmscanalsooccur.1,10–12_Inour

casewemadethediagnosissolelyonclinicalgrounds,asthere

wasevidenceonphysicalexaminationofchondritisofboth

earlobes,sero-negativepolychondritisandneurological

com-promisewithasepticmeningitis.About25%ofpatientsdiein

uptofiveyearsfollowingdiagnosis;laryngotracheal

rev bras reumatol.2017;57(6):623–625

625

Table1–Complementaryexams.

Exams Results

BilateralX-raysofhandsandknees Normal

Angiotomographyofchest,abdomenand pelvis

Normal

Uppergastrointestinalendoscopy Normal

Colonoscopy Smallpolyp

Echocardiogram Normal

Proteinelectrophoresis(blood) Normal

Calciumandinorganicphosphorus Normal

Electroneuromyography Normal

Anti-HIV,syphilis,hepatitisBandC Negative

Rheumatoidfactor,antinuclearfactor, anticentromerefactor

Negative

Erythrocytesedimentationrate 53

Serumiron 16.3(normal

range:59–158)

Totalironbindingcapacity 252.8(normal range:250–450)

Ironsaturationratio 6.4%(normal

range:20–50)

Ferritin 327.54␮g/l

(normalrange: 4.63–204)

Reticulocytes 11.8

Hemoglobin 11.8

VG 32.1

VCM 72

C3andC4 Normal

ANCA Negative

Spinalfluid

Erythrocytes 0.3

Leucocytes 71(97%

lymphocytes,3% monocytes)

Glucose 68

Protein 69.7

GRAM Negative

BAAR Negative

HTLV1and2 Negative

ofdeath.1,4 _{Factorsthathaveanegativeimpactonsurvival}

atthetimeofdiagnosisincludeoldage,anemiaand

laryn-gotracheal stricture.1,2 _{Oralnonsteroidal anti-inflammatory}

drugs may be used to treat patients with arthralgias and

mildarthritis.Standardimmunosuppressivetreatmentstarts

with high doses of corticosteroids(Prednisone 1mg/kgqd),

which islater tapered off to a smaller dosage in patients

withmoderatetoseverecompromise.Methotrexatemaybe

used asasecond-line drugtoavoidside effectsofchronic

corticosteroid treatment. Azathioprine, cyclophosphamide,

cyclosporine,mycophenolatemofetilandTNF-antagonistsare

otheroptions.5,11

Conflicts

of

interest

Theauthorsdeclarenoconflictsofinterest.

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6.ArnaudL,MathianA,HarocheJ,GorochovG,AmouraZ. Pathogenesisofrelapsingpolychondritis:a2013update. AutoimmunRev.2014;13:90–5.

7.WangZJ,PuCQ,WangZJ,ZhangJT,WangXQ,YuSY,etal. Meningoencephalitisormeningitisinrelapsing

polychondritis:fourcasereportsandaliteraturereview.JClin Neurosci.2011;18:1608–15.

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