Rev. Bras. Reumatol. vol.56 número2

r e v b r a s r e u m a t o l . 2016;56(2):181–184

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

Acute

acalculous

cholecystitis

in

systemic

lupus

erythematosus:

a

rare

initial

manifestation

夽

Valdano

Manuel

,

Gertrudes

Maria

Pedro

,

Lemuel

Bornelli

Cordeiro

,

Sandra

Maria

da

Rocha

Neto

de

Miranda

a_{EducationOffice,Post-GraduationandResearch,ClínicaGirassol,Luanda,Angola}

b_{DepartmentofInternalMedicine,ClínicaGirassol,Luanda,Angola}

a

r

t

i

c

l

e

i

n

f

o

Articlehistory:

Received20December2013 Accepted26March2014 Availableonline25October2014

Keywords:

Systemiclupuserythematosus Acuteacalculouscholecystitis Abdominalcomputedtomography Initialmanifestation

a

b

s

t

r

a

c

t

Acuteacalculouscholecystitisisaveryraregastrointestinalmanifestationinsystemiclupus erythematosusandbecomesrarerasaninitialmanifestation.Thereareonlytwocases reported.Theauthorsreporta20-year-oldblackwomanthatpresentedacuteacalculous cholecystitisrevealedbyabdominalcomputedtomography.Duringhospitalization,shewas diagnosedsystemiclupuserythematosus.Conservativetreatmentwithantibioticswas per-formedwithcompleteremissionofthesymptoms.Corticosteroidwasstartedinambulatory. Cholecystectomyhasbeenthetreatmentofchoiceinacuteacalculouscholecystitisasa complicationofsystemiclupuserythematosus.Thepatientrespondedwelltoconservative treatment,andsurgerywasnotrequired.Thiscaseisuniqueinthewaythatcorticosteroid wasstartedinambulatorycare.Weshouldnotforgetthattheacuteacalculouscholecystitis canbetheinitialpresentationofsystemiclupuserythematosusalthoughitsoccurrenceis veryrare.Conservativetreatmentshouldbeconsidered.Abdominalcomputedtomography wasadeterminantexamforbetterassessmentofacuteacalculouscholecystitis.

©2013ElsevierEditoraLtda.Allrightsreserved.

Colecistite

aguda

acalculosa

no

lúpus

eritematoso

sistêmico:

uma

manifestac¸ão

inicial

rara

Palavras-chave:

Lúpuseritematososistêmico Colecistiteagudaacalculosa Tomografiacomputadorizadado abdome

Manifestac¸ãoinicial

r

e

s

u

m

o

Acolecistiteagudaacalculosaéumamanifestac¸ãogastrointestinalraranolúpus eritem-atoso sistêmicoe ainda mais rara como manifestac¸ão inicial. Foramdescritos apenas doiscasosatéomomento.Osautoresrelatamocasodeumamulhernegrade20anos, com quadrode colecistiteaguda acalculosa reveladapelatomografiacomputadorizada do abdome. Durantea hospitalizac¸ão, a paciente foi diagnosticada com lúpus eritem-atososistêmico.Houveremissãocompletadossintomasapóstratamentoconservadorcom

夽

ThestudywasoriginatedatClínicaGirassol,Luanda,Angola.

∗ _{Correspondingauthor.}

E-mail:valdanympub@gmail.com(V.Manuel).

http://dx.doi.org/10.1016/j.rbre.2014.03.027

182

antibióticos.Iniciou-setratamentocomcorticosteroidesnoambulatório.Emboraa cole-cistectomiasejaotratamentodeescolhaemcasosdecolecistiteagudaacalculosacomo complicac¸ãodolúpuseritematososistêmico,apacienterespondeubemaotratamento con-servador;logo,acirurgianãofoinecessária.Estecasoéúnicoemrazãodomodocomo ocorticosteroidefoiiniciadonoatendimentoambulatorial.Éimportantelembrarquea colecistiteagudaacalculosapodeseramanifestac¸ãoinicialdolúpuseritematososistêmico, emborasuaocorrênciasejarara.Deve-seconsiderararealizac¸ãodetratamento conser-vador.Atomografiacomputadorizadadoabdomefoideterminante paraquefossefeita umamelhoravaliac¸ãodacolecistiteagudaacalculosa.

©2013ElsevierEditoraLtda.Todososdireitosreservados.

Introduction

Systemiclupuserythematosus(SLE)isanautoimmune dis-ease found predominantly in female gender1–3 _{in which}

almostallorgans canbeinvolvedwithwide rangeof clini-calmanifestations.Gastrointestinalmanifestationisusually mild, but gallbladder involvementis an uncommon event. Acuteacalculouscholecystitis(AAC)isveryrareasa compli-cationofSLEandbecomesrarerasaninitialpresentation.1–9

SeveralcasesofAACasacomplicationofSLEwerereported; nevertheless only two cases have been reported as initial manifestation.5,9

Case

report

A 20-year-old black woman with history ofmigratory pol-yarthralgia was admitted with fever, nausea and vomit, acute abdominal pain in the right upper quadrant and referred loss of appetite. Physical examination evidenced febrile (38.6◦_{C), tachycardia (102 beats per minute),}

dehy-dratedandsubictericmucousmembranes.Abdominalpain intherightupperquadrantwithhepatomegalyof8cmbelow thecostalmarginwasfound.Cardiacauscultation,revealed agrade-II/VIprotosystolicmurmurinall cardiacfocus. Ini-tiallaboratorydatashowedhemoglobin7.6g/dL,leucocytes 3300/L, albumin 2.2g/dL, alanine aminotransferase 86U/L, aspartateaminotransferase127U/L,totalbilirubin1.8mg/dL; lactatedehydrogenase1719U/Landtestforplasmodium neg-ative. Abdominal ultrasound confirmed hepatomegaly and revealedsplenomegaly,gallbladderslightlydistendedwithout wallthickening.During hospitalization,the patientevolved withretrosternalpain,orthopnea,surfeitsensation,increased pain in the right upper quadrant, nausea, vomiting, diar-rheaand fever. Pulmonary auscultation revealed abolished in both bases. Cardiac auscultation was the same. The abdomenwas distended withhepatomegaly of8cm below the right costal margin; liquid wave signal and murphy

werepresent.Lowerlimbspresentedmalleolaredema.The main laboratory findings are shown (Table 1). Teleradiog-raphy of chest showed bilateral pleural effusion of small volume. Echocardiography revealed pericardial effusion of 1.5cm.Abdominalcomputedtomographyrevealedincreased wall thickness without any evidence of stone and edema aroundthegallbladder(Fig.1),andconfirmedsplenomegaly,

hepatomegaly andascites. Basedonthephysical examina-tion,laboratoryandimagefindings,adiagnosisofinaugural systemiclupuserythematosustriggeredbyacuteacalculous cholecystitis was established. The patient initiated treat-ment with ceftriaxone and metronidazole, furosemide and albumin. Within10 days, therewas complete remissionof symptoms. The corticosteroid was initiated in ambulatory of rheumatology.Surgical interventionwas not performed. Accordingtotherheumatologists’feedback,shelookedwell and did not have any symptoms until four months after discharge.

Table1–Laboratoryfindingsduringadmissioncourse.

Exam Results Referencevalues

TestforPlasmodium Negative Negative Hemoglobin 6.9g/dL 11.5–15.5 Leukocytes 3.2×109/L 04–11 Platelets 342×109/L 150–400 Totalproteins 5.2mg/dL 6.5–8.1 Proteinuria-24h 174mg/dia <150 Albumin 2.3mg/dL 3.5–5.0

ALT 80U/L <40

AST 112U/L <40

LDH 1557U/L 230–460

Alkalinephosphatase 155U/L 32–92

GGT 256U/L <50

Creatinine 0.4mg/dL 0.6–1.3

Urea 4mg/dL 0.8–26

Amylase 28U/L 36–128

Anti-HBs 0.44S/CO <0.90 Anti-HCV 0.8S/CO <0.9 Toxoplasmosis 0.2S/CO <0.8

ANAa _{6IU/mL >1.1positive}

Anti-dsDNAa _{80IU/mL >60positive}

C3a _{45.2mg/dL 79–152}

C4a _{7.1mg/dL 16–38}

AnticardiolipinIgMa _{7.9UMPL/mL 0.0–7.0}

Anti-␤2glycoprotein1IgMa _{12.1UMPL/mL 0.0–5.0}

Anti-␤2glycoprotein1IgGa _{5.5UGPL/mL 0.0–5.0}

Smoothmuscleantibodya _{1:80 <1:20}

ALT,alanineaminotransferase;AST,aspartateaminotransferase; LDH, lactatedehydrogenase;GGT, gamma-glutamyltransferase; ANA,antinuclearantibody.

rev bras reumatol.2016;56(2):181–184

183

Fig.1–AbdominalCTshowgallbladderwallthickness withoutstonewithedemaaround(arrow).

Discussion

Abdominal pain is always a challenge for diagnosis and treatment in patients with SLE; it may be caused by the disease itself, other comorbidities or drug effects.1–3 _The

involvement of gastrointestinal and hepatobiliary systems sparinggallbladderhavebeenreported.1–9_{Thedepositionof}

immunecomplexesinbloodvesselwallsresultsinacute vas-culitis; these events result in ischemia and fibrosis in the targetorgan.4–9_{Thromboemboliceventsaremostfrequently}

observedin patients withSLE; this risk isincreasedwhen anticardiolipinantibodyispositive,10_{asinthecaseof}

anticar-diolipinIgM,anti-␤2glycoprotein1positive,althoughitwas alowtiter.Thepatientdoesnothavecriteriafordiagnosis ofantiphospholipidsyndrome.10_{Histologically,}

antiphospho-lipidsyndrome ischaracterizedbymultiplethrombi inthe vesselsofthegallbladderwithoutevidenceofvasculite.5_The

gallbladder is rarely involved in patients with SLE.1–9

Sev-eral casesofAAC as a complicationof SLEwere reported, butonlytwocasesofAACasaninitialmanifestationofSLE were described,onein apediatric patientand other in an adult.5,9_{Themostwidelyusedclassificationcriteriafor}

diag-nosis of SLE are those proposed by the American College ofRheumatologyand SystemicLupusInternational Collab-oratingClinicsthatrequirefour ormoreitemstodiagnose SLE(cutaneous manifestation,joints, serositis, renal disor-der,hematologicdisorder,immunologicabnormality).11,12_In

thiscase,thepatientpresentedwithpainintheperipheral joints,pleuralandpericardialeffusion,proteinuria,hemolytic anemia,leukopenia,positiveANA,positiveanti-dsDNA, posi-tiveanti-␤2glycoprotein1,positiveSmandlowcomplement. Whenthereisasuspicionofinvolvementofthegallbladder asthe cause ofabdominal paininthese patients, abdomi-nalultrasoundandcomputedtomography(CT)areindicated forbetterassessmentofAAC.13_{Inthiscase,theabdominal}

ultrasound performed at admission was inconclusive. The abdominalCTperformedwasessentialforrevealingthe gall-bladder wall thickness withedema aroundthe gallbladder without the presenceofstones,important findingsfor the diagnosisofAACcasesthathavenotbeendisclosedbythe ultrasoundmadeintheadmission.Nootherriskfactorsfor cholecystitiswerefound.Inthiscase,AACmaybedueto mul-tiple thrombi in thegallbladder vessels.8 _{Thetreatmentof}

AAChasbeencontroversial.Therearefourcasesreportedof patientswithAACinSLE,whichdidnothavecholecystectomy, and responded well to medicaltreatment with high doses of corticosteroid.4,5,7,9 _{In this case, due to major}

improve-ment ofthe symptoms withantibiotics, corticosteroidwas started inoutpatientfollow-up.Surgerywasnotperformed whichjustifynohistologicaldescription.Highdosesof cor-ticosteroids are usuallysuggestedas first-line treatment if patientshaveagoodgeneralconditionwithoutother chole-cystitisriskfactors,noserioushealthcomplicationsandno infections.7

Conclusions

Despitetherarity,weshouldnotforgetthatacuteacalculous cholecystitis,besidesbeingacomplication,canbetheinitial manifestationofSLE.Conservativetreatmentshouldbe con-sidered.Inthiscase,corticosteroidwasstartedafterpatient discharge;itmadethiscaseunique.Computedtomographyis agoodexamforbetterassessmentofAAC.

Conflicts

of

interest

Theauthorsdeclarenoconflictsofinterest.

Acknowledgments

WewouldliketoacknowledgeDr.HumbertoMoraisandDra BrunaDavidforsuggestions.

r

e

f

e

r

e

n

c

e

s

1.TuY-T,YehK-W,ChenL-C,YaoT-C,OuL-S,LeeW-I,etal. Differencesindiseasefeaturesbetweenchildhood–onsetand adult–onsetinsystemiclupuserythematosuspatients presentingwithAcuteAbdominalPain.SeminArthritis Rheum.2011;40:447–54.

2.RicherO,UlinskiT,LemelleI,RanchinB,LoiratC,PietteJC, etal.Abdominalmanifestationsinchildhood–onsetsystemic lupuserythematosus.AnnRheumDis.2007;66:

174–8.

3.EbertEC,HagspielKD.Gastrointestinalandhepatic manifestationsofsystemiclupuserythematosus.JClin Gastroenterol.2011;45:436–41.

4.KamimmuraT,MimoriA,TakedaA,MasuyamaJ,YoshioT, OkazakiH,etal.Acuteacalculuoscholecystitisinsystemic lupuserythematosus.Lupus.1998;7:361–3.

5.Mendonc¸aJA,Marques-NetoJ,PrandoP,AppenzellerS.Acute acalculuoscholecystitisinjuvenilsystemiclupus

184

6. BasiratniaM,VaseiM,BahadorA,EbrahimE,DarakhshanA. Acuteacalculouscholecystitisinachildwithsystemiclupus erythematosus.PediatrNephrol.2006;21:873–6.

7. ShinSJ,NaKS,JungSS,BaeSC,YooDH,KimSY,etal.Acute acalculouscholecystitisassociatedwithsystemiclupus erythematosuswithSjogren’ssyndrome.KoreanJIntern Med.2002;17:61–4.

8. SwanepoelCR,FloydA,AllisonH,LearmonthGM,CassidyMJ, PascoeMD.Acuteacalculouscholecystitiscomplicating systemiclupuserythematosus:casereportandreview.Br MedJClinResEd.1983;286:251–2.

9. GhorbelIB,SouabniL,LamloumM,KhanfirM,BrahamA, MiledM,etal.Unecholécystiteaiguëalithiasiquerévélantun lupusérythémateuxsystémique.GastroenterolClinBiol. 2009;33:1175–8.

10.WilsonAW,GharaviAE,KoikeT,LockshinMD,BranchDW, PietteJC,etal.Internationalconsensusstatementon

preliminaryclassificationcriteriafordefinite

antiphospholipidsyndrome.Reportonaninternational workshop.ArthritisRheum.1999;42:

1309–11.

11.HochbergMC.UpdatingtheAmericanCollegeof Rheumatologyrevisedcriteriafortheclassificationof systemicLupuserythematosus.ArthritisRheum. 1997;40:1725.

12.PetriM,OrbaiAM,AlarconGS,GordonC,MerrillJT,FortinPR, etal.Derivationandvalidationofthesystemiclupus internationalcollaboratingclinicsclassificationcriteriafor systemiclupuserythematosus.ArthritisRheum.

2012;64:2677e86.