Rev. Bras. Reumatol. vol.56 número1

r e v b r a s r e u m a t o l . 2016;56(1):79–81

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

Pyomyositis

in

childhood-systemic

lupus

erythematosus

Gabriela

Blay

_,

_Mariana

_P.L.

_Ferriani

_,

_Izabel

_M.

_Buscatti

_,

_Camila

_M.P.

_Franc¸a

_,

Lucia

M.A.

Campos

_,

_Clovis

_A.

_Silva

a_{PediatricRheumatologyUnit,PediatricDepartment,FaculdadedeMedicinadaUniversidadeSãoPaulo,SãoPaulo,SP,Brazil}

b_{PediatricAllergyandImmunologyUnit,PediatricDepartment,FaculdadedeMedicinadaUniversidadeSãoPaulo,SãoPaulo,SP,Brazil}

c_{DivisionofRheumatology,FaculdadedeMedicinadaUniversidadeSãoPaulo,SãoPaulo,SP,Brazil}

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Articlehistory:

Received16December2013 Accepted7April2014

Availableonline27November2014

Keywords:

Pyomyositis

Childhood-systemiclupus erythematosus

Infection

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Pyomyositisisapyogenicinfectionofskeletalmusclethatarisesfromhematogenousspread andusuallypresentswithlocalizedabscess.Thismuscleinfectionhasbeenrarelyreported inadult-onsetsystemiclupuserythematousand,tothebestofourknowledge,hasnotbeen diagnosedinpediatriclupuspopulation.Amongourchildhood-onsetsystemiclupus ery-thematouspopulation,including289patients,onepresentedpyomyositis.Thispatientwas diagnosedwithchildhood-onsetsystemiclupuserythematousattheageof10years-old. Aftersixyears,whilebeingtreatedwithprednisone,azathioprineandhydroxychloroquine, shewashospitalizedduetoa30-dayhistoryofinsidiouspainintheleftthighandno appar-enttraumaorfeverwerereported.Herphysicalexaminationshowedmuscletenderness andwoodyinduration.Laboratorytestsrevealedanemia,increasedacutephasereactants andnormalmuscleenzymes.Computertomographyoftheleftthighshowedcollection on themiddlethirdofthevastusintermedius,suggestingpurulentstageofpyomyositis. Treatment with broad-spectrum antibioticwasinitiated, leadingto a completeclinical resolution.Inconclusion,wedescribedthefirstcaseofpyomyositisduringchildhoodin pediatriclupuspopulation.Thisreportreinforcesthatthepresenceoflocalizedmuscle paininimmunocompromisedpatients,evenwithoutelevationofmuscleenzymes,should raisethesuspicionofpyomyositis.Apromptantibiotictherapyisstronglyrecommended.

©2013ElsevierEditoraLtda.Allrightsreserved.

Piomiosite

no

lúpus

eritematoso

sistêmico

juvenil

Palavras-chave:

Piomiosite

Lúpuseritematososistêmicojuvenil Infecc¸ão

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e

s

u

m

o

A piomiosite é uma infecc¸ão piogênica da musculatura esquelética, decorrente da disseminac¸ãohematogênicaegeralmenteacompanhadadeformac¸ãodeabscesso local-izado.Estainfecc¸ãodamusculaturaéraramentedescritaemadultoscomlúpuseritematoso sistêmico(LES)e,atéondesesabe,aindanãoofoiempacientescomLESjuvenil(LESJ).

∗ _{Correspondingauthor.}

E-mail:clovisaasilva@gmail.com(C.A.Silva).

http://dx.doi.org/10.1016/j.rbre.2014.04.005

80

Denossos289pacientescomLESJ,umaapresentoupiomiosite.DiagnosticadacomLESJ aos10anosdeidadeeapósseisanosdetratamentocomprednisona,azatioprinae hidroxi-cloroquina,apacientefoihospitalizadaemrazãodeumhistóricode30diasdedorinsidiosa nacoxaesquerda,semrelatoalgumdetraumaaparenteoufebre.Oexamefísicomostrou músculossensíveisecomendurecimentolenhoso.Osexameslaboratoriaisrevelaram ane-mia,aumentodereagentesdefaseagudaeenzimasmuscularesnormais.Atomografia computadorizadadacoxaesquerdamostroucolec¸ãonoterc¸omédiodovastointermédio, sugerindoestágiopurulentodepiomiosite.Iniciou-setratamentocomantibióticodelargo espectro,quelevouàresoluc¸ãoclínicacompleta.Emsuma,descreveu-seoprimeirocaso depiomiositeempacientescomLESJencontradonesteservic¸o.Esterelatoreforc¸aquea presenc¸adedormuscularlocalizadaempacientesimunocomprometidos,aindaquesem aumentodeenzimasmusculares,devesugerirodiagnósticodepiomiosite.Recomenda-se tratamentoimediatocomantibióticos.

©2013ElsevierEditoraLtda.Todososdireitosreservados.

Introduction

Pyomyositis isapyogenic infection ofskeletal musclethat arises from hematogenous spread and usually presents with localized abscess.1 _{Infectious myopathy is associated}

with immunocompromised conditions, especially human immunodeficiency virus (HIV) infection, diabetes melli-tus, malignancy,immunosuppressive drugs and rheumatic diseases.1–7

Ofnote,infectionsareoneofthemaincausesof morbid-ityandmortalityinadultsystemiclupuserythematosus(SLE) andchildhood-onsetSLE(C-SLE)patients,8,9_{andmayinvolve}

theskeletalmuscle.Pyomyositishasbeenrarelyreportedin adultSLE,3–7_{andtothebestofourknowledge,ithasnotbeen}

diagnosedduringchildhoodinpediatriclupuspopulation. WereviewedourdatafromJanuary1983toJuly2013and included5593patientsofPediatricRheumatologyUnitof Insti-tutodaCrianc¸adaFaculdadedeMedicinadaUniversidadede SãoPaulo.Weidentified289(5.1%)C-SLEpatientsthatfulfilled the American Collegeof Rheumatology(ACR) classification criteria.10_{One(0.34%)ofthemhadapyomyositisepisodeafter}

C-SLEdiagnosiswasestablishedandwasdescribedherein.

Case

report

A10-year-oldgirlwasdiagnosedwithC-SLEbasedondiscoid skin rash, pericarditis, psychosis, lymphopenia, thrombo-cytopenia,proteinuria, antinuclear antibodies (ANA)1/1280 (nuclearhomogenouspattern),andpresenceof anti-double-stranded DNA (anti-dsDNA) antibody, fulfilling the ACR classification criteria for SLE.11 _{At that moment, the SLE}

Disease Activity Index 2000 (SLEDAI-2K) score11 _{was 28}

basedonthefollowingfindings:psychosis,proteinuria, cel-lular casts, alopecia, pleuritis, pericarditis, low levels of C3 and C4, presenceof anti-dsDNA, fever and thrombocy-topenia. She was treated with prednisone (2.0mg/kg/day) thatwasprogressivelytaperedto2.5mg/day, hydroxychloro-quine (5.0mg/kg/day) and azathioprine (2.0mg/kg/day). At the age of 16 years, she was hospitalized in our service with a 30-day history of insidious pain in the middle

of left thigh, without cramps, apparent trauma or fever. Her physical examination showed localized muscle ten-derness and woody induration. She did not present skin erythema and local heat. At that time, she was on pred-nisone (0.95mg/kg/day), azathioprine (2.25mg/kg/day) and hydroxychloroquine (5.0mg/kg/day) dueto severe alopecia, discoid skin rash, photosensitivity and oral ulcers. Labo-ratory tests revealedhemoglobin 9.3mg/L,hematocrit29%, whitebloodcellcount8700mm3_{(71%neutrophils,20%}

lym-phocytes and 8% monocytes), platelet count 202,000mm3_,

C-reactiveprotein46.9mg/L(referencevalue<5mg/L), eryth-rocyte sedimentation rate 54mm/1st hour (normal range 0–20mm/h), proteinuria 0.12g/24h, urea 41mg/dL (normal range15–45mg/dL)andcreatinine0.36mg/dL(normalrange 0.6–0.9mg/dL),C375mg/dL(normalrange90–180mg/dL)and C4 9.9mg/dL (normal range 10–40mg/dL). Muscle enzymes tests performedshowed: aspartateaminotransferase 31U/L (normal range 10–34U/L), alanine aminotransferase 34U/L (normalrange10–44U/L),creatinekinase64U/L(normalrange 24–204U/L).Anti-dsDNAwaspositive,andSLEDAI-2Kwas4. Computedtomography(CT)oftheleftthighshowed collec-tion on the middle third of the vastus intermedius muscle, dimensions 5.0cm×3.0cm×2.0cm, strongly suggestive of

purulentstageofpyomyositis.Thepatientwassubmittedto an ultrasound-guidedpuncture,but no fluidwas obtained. Bloodculturedidnotidentifyanyorganisms.Treatmentwith intravenousantibiotics,suchascefepime(150mg/kg/day)and clindamycin (40mg/kg/day), was administered fora seven-dayperiod.Shewasthen dischargedwithoralclindamycin (40mg/kg/day)formoresevendays,resultinginacomplete clinicalresolution.

Discussion

PyomyositishadneverbeenreportedinC-SLEpopulation fol-lowedinourtertiaryUniversityHospitalinthelast30years. Thepresenceoflocalizedmusclepainwithindurationsinan immunosuppressedpatientsuggestedthisdiagnosis.

Thisinfectious myopathywas firstdescribed in1885by Scribba12_{andisararepyogenicinfectionofmuscletissue.In}

rev bras reumatol.2016;56(1):79–81

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ItisusuallycausedbyStaphylococcusaureus,Streptococcussp. andEscherichiacoli.2,7_{However,inmorethantwothirdsofthe}

cases,theetiologicalagentisnotidentifiedinbloodcultures,14

asobservedinourpatient.

Pyomyositis comprises three clinical stages: invasive (insidiousandnonspecificsymptoms);purulent(deepabscess formation and inflammatory signs); and late stage (high fever,severepain,musclefluctuationandoccasionallyseptic shock).2,7_{Purulentstageisobservedinupto90%ofpatients}

atdiagnosis,15_{asevidencedherein.}

Ofnote,theclinicalpresentationandthelaboratoryexams ofpyomyositisarenonspecific.Whitebloodcellcountcanbe elevatedin50–60%ofcases;acutephasereactantsareusually high15 _{andmuscleenzymesareoftennormal,}2_asobserved

inthiscase.Therefore,earlyimagingtestsareimperativefor the diagnosis ofpyomyositis.X-raystudies can beused to ruleoutbonelesions.Ultrasonographyisanaccessible, low-cost and quick tool, but not sensitive enough, particularly attheonsetoftheinfection.14 _{Importantly,CTormagnetic}

resonanceimagingconfirms thediagnosis, andmay reveal muscleenlargement,abscessextension,jointeffusionsand fluidcollections.15

Thisdiseasecanalsooccurinhealthypatientsafterlocal mechanical trauma,13,15 _{and can be associated with}

para-sitosisanddesnutrition,alsoknownastropicalpyomyositis. Theatypicalornonspecificpresentationofpyomyositismay delay the diagnosis and treatment in immunosuppressive and/or chronic diseases, such as HIV infeccion,13–15 _HTLV

infeccion,7 _leukemia,13–15 _diabetes,13–15 _scleroderma,13 _and

dermatomyositis.15 _{To the best of our knowledge, there}

are only five cases described of pyomyositis in adult SLE patients3–7 _{and only one with childhood-onset lupus who}

presented pyomyositis at the age of 32.4 _{Therefore, the}

firstcase ofpyomyositisinpediatric lupuspopulationwas describedherein.

Differentialdiagnosis of pyomyositisaffectingthe thigh includes hematoma, osteomyelitis, septic arthritis, deep venousthrombosis,cellulitisandmalignanttumors.2,15

Thetreatmentdependsonthestageofdisease.Inthe puru-lentandlatestages,intravenousbroad-spectrumantibiotics are the main therapy. Moreover, percutaneous drainage or surgicalincisionmaybenecessaryinpatientsrefractoryto antibiotics.2_{Ourpatienthadcompleterecoveryafterclinical}

treatment.

Inconclusion, wedescribed acaseofpyomyositisin C-SLEpopulation.This reportreinforcesthatthe presenceof localizedmusclepaininimmunocompromisedpatients,even withoutelevationofmuscleenzymes,shouldraisethe sus-picionofpyomyositis.Apromptantibiotictherapyisstrongly recommended.

Fundings

ThisstudywassupportedbyFundac¸ãodeAmparoàPesquisa doEstadodeSãoPaulo(FAPESP,Brazil-grants2008/58238-4to

CAS and 2011/12471-2 to CAS), by Conselho Nacional do Desenvolvimento Científico e Tecnológico (CNPQ, Brazil – grant302724/2011-7toCAS),byFedericoFoundation, Switzer-land toCASandbyNúcleo deApoio àPesquisa“Saúde da Crianc¸aedoAdolescente”daUSP(NAP-CriAd),Brazil.

Conflicts

of

interest

Theauthorsdeclarenoconflictofinterest.

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