Rev. Bras. Reumatol. vol.57 número6

r e v b r a s r e u m a t o l . 2017;57(6):613–615

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

Nephrotic

syndrome

as

the

first

manifestation

of

juvenile

systemic

scleroderma

Síndrome

nefrótica

como

a

primeira

manifestac¸ão

da

esclerodermia

sistêmica

juvenil

Saulo

B.

Couto

_,

_Adriana

_M.

_Sallum

_,

_Luciana

_S.

_Henriques

_,

_Denise

_M.

_Malheiros

_,

Clovis

A.

Silva

_,

_Maria

_H.

_Vaisbich

a_{UniversidadedeSãoPaulo(USP),FaculdadedeMedicina,HospitaldasClínicas,InstitutodaCrianc¸a,UnidadedeNefrologiaPediátrica,}

SãoPaulo,SP,Brazil

b_{UniversidadedeSãoPaulo(USP),FaculdadedeMedicina,HospitaldasClínicas,DepartamentodePatologia,SãoPaulo,SP,Brazil}

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o

Articlehistory:

Received27February2014 Accepted17August2014

Availableonline12December2014

Introduction

Juvenilesystemicsclerosis(JSSc)isanautoimmunedisease, characterizedbydisorderedcollagen accumulation,leading todisseminatedvascularlesionsinskinandinternalorgans fibrosis,includingthekidneys.1–3

Worthyofnote,renalinvolvementisararemanifestation and occurs in 1–12%of JSSc patients, mainly with arterial hypertensionand/orproteinuria.1,3–5_{Sclerodermarenalcrisis}

wasalsorarelyreported5,6 _{andtoourknowledge,nephrotic}

syndrome(NS)wasdescribedinonlyoneJSScpatientwith membranousnephropathy.7

WereporthereinapatientwhopresentedNSasthefirst manifestationofJSScwithfocalsegmentalglomerulosclerosis (FSGS).

∗ _{Correspondingauthor.}

E-mail:[email protected](M.H.Vaisbich).

Case

report

Afemale patientpresentedvomiting,malaiseand general-izededemaattheageof12years.Shehadsystemicarterial hypertension,hypoalbuminemia(<2.5mg/dL)andproteinuria (urineprotein/creatinineratio≥2.0mg/g).Shewasdiagnosed

withNSandtreatedwithprednisone(60mg/m2_/day)with

pro-gressivereduction.Duringfollow-upshehadseveralrelapses ofNS,oneofthemassociatedtotheupperrespiratorytract infectionsandanotheronewithspontaneousbacterial peri-tonitis,andwasreferredtoourUniversityHospitalattheage of14.On the first admissionatourservice, shepresented orbital and lowerlimbsedema, systemicarterial hyperten-sion, sclerodactyly and proximal skin sclerosis.No muscle weaknessand vasculitiswereobserved.Laboratoryfindings

http://dx.doi.org/10.1016/j.rbre.2014.08.004

614

A

B

A

Fig.1–Nailfoldcapillaroscopyshowingmoderatecapillary dilation(arrowA)andmildfocaldevascularization(arrow B)inpatientwithjuvenilesystemicsclerosis.

revealedserumalbumin0.9g/dL(normallevels3.8–5.6),total cholesterol637mg/dL(normal<199),lowdensitylipoprotein (LDL) 439mg/dL (normal <110), serum urea 18mg/dL (nor-mal 15–45), serum creatinine 0.53mg/dL (normal 0.6–0.9), 25-hydroxyvitamin D 5ng/mL (normal 30–100ng/mL) and proteinuria7.8g/day. HandsX-rayrevealed no bone abnor-malities. Antinuclear antibodies (ANA), rheumatoid factor, andanti-Scl-70(anti-topoisomeraseI),anti-Sm,Anti-SS-A/Ro, Anti-SS-B/Laandanti-smoothmuscle(anti-SMA)antibodies werenegative.C3andC4fractionsofthecomplement sys-temwerenormal.SerologyforhepatitisA,BandC,human immunodeficiencyvirus(HIV),cytomegalovirus,Epstein–Barr virusandsyphiliswerenegative.Echocardiogramwasnormal. Moderatecapillarydilationandmildfocaldevascularization were observed in nailfold capillaroscopy, compatible with early stage of scleroderma (scleroderma pattern) (Fig. 1). Percutaneous renal biopsy guided by ultrasound revealed focalsegmentalglomerulosclerosisand direct immunofluo-rescencewerenegative(Fig.2AandB).Therefore,shefullfilled the provisional classification criteriafor JSSc.6 _Thepatient

was treated with oral 25-hydroxyvitamin D (800IU/day),

methotrexate (0.5mg/kg/week) and amlodipin (0.15mg/kg). Prednisone (60mg/m2_{/day) was administered for 4}

consec-utive weeks, followed by alternate-day (40mg/m2_{) for 2}

consecutive months,with tapering for4months and then stoppingthismedication.Currently,sheisbeingtreatedwith methotrexate15mg/week,withoutedemaandproteinuria.

Discussion

Toourknowledge,thiswasthefirstcasereportedwith steroid-sensitiveNSandFSGSasthefirstmanifestationofJSSc.

The mostimportantsystemic manifestationof JSSc are cutaneouslesions,speciallyindurationproximalto metacar-pophalangealjoints,sclerodactlyandedema,1,3,4_asobserved

inthepresentcase.Thecapillaroscopycanbeusedto eval-uate changes of microcirculation in the capillaries of the nailbed.8_{Thesefindingsstronglysuggestsystemicsclerosis}

diagnosis,8_{asevidencedherein,andnotfoundincutaneous}

scleroderma.9

Kidneyinvolvementwasrarelydescribedinpediatric scle-rodermapopulation.Themostfrequentrenalmanifestations were arterial hypertension and proteinuria. Renal crisis in JSSc patients ranges from 0.7% to 4%.1,3–5 _{This is a}

life-threatening complication with abrupt onset of malignant arterialhypertension,proteinuriaand/orhematuria, throm-boticmicroangiopathyandpotentiallycanresulttoendstage kidneydisease.6,10,11

AdultsclerodermaNShasagreatspectrumvaryingfrom minimal changes disease and secondary amyloidosis to rapidly progressive renal failure and proliferative changes lesions with crescents,and membranous nephropathy. NS wasonlyreportedinone12-year-oldfemalepatientwho pre-sentedwithsclerodermafiveyearsaftertheonsetofthisrenal involvementwithmembranousnephropathy.7

Importantly,ourpatienthad steroidsensitive NSwitha severehistologicalpatternofFSGS.Thisglomerulopathyisan importantcauseofproteinuriaandchronicrenaldiseasein childrenandadolescents,accountingin5–15%ofallcasesof idiopathicNS.12,13

ThetreatmentofJSCc isperformedaccording toorgans andsystemsinvolvement.Methotrexatehasbeenshownto improveskinscoresinearlydiffuseSSc.14_{Additionally,renal}

rev bras reumatol.2 0 1 7;57(6):613–615

615

treatmentofFSGSincludesglucocorticoidand antihyperten-sivedrugs,asusedinourpatient.12_{Interestingly,theabsence}

ofproteinuriawithcompleteremissionaftertreatment sug-gestsadequaterenallong-termoutcome.13

Inconclusion,wereportedararecaseofNSwithFSGSas thefirstmanifestationofscleroderma.Therefore,renalbiopsy ismandatoryinJSScpatientswithsustainedproteinuriaorNS.

Conflicts

of

interest

Theauthorsdeclarenoconflictsofinterest.

Acknowledgments

ThisstudywassupportedbyFundac¸ãodeAmparoàPesquisa doEstadodeSãoPaulo–FAPESP(grant#08/58238-4toCAS), Conselho Nacional de Desenvolvimento Científico e Tec-nológico–CNPq(302724/2011-7toCAS),FedericoFoundation toCASandNúcleodeApoioàPesquisa“SaúdedaCrianc¸ae doAdolescente”daUSP(NAP-CriAdtoCAS).

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