Neisseria meningitidis presenting as acute abdomen and recurrent reactive pericarditis

brazjinfectdis2016;20(6):641–644

w w w . e l s e v i e r . c o m / l o c a t e / b j i d

The

Brazilian

Journal

of

INFECTIOUS

DISEASES

Case

report

Neisseria

meningitidis

presenting

as

acute

abdomen

and

recurrent

reactive

pericarditis

Karolina

Akinosoglou

_,

_Angelos

_Alexopoulos

_,

_Nikolaos

_{Koutsogiannis}

_,

Charalampos

Gogos

_,

_Aleksandra

_Lekkou

a_{MedicalSchoolofPatras,UniversityHospital,DepartmentofInternalMedicine,Rio,Greece}

b_{MedicalSchoolofPatras,UniversityHospital,DepartmentofCardiology,Rio,Greece}

c_{MedicalSchoolofPatras,UniversityHospital,DepartmentofInfectiousDiseases,Rio,Greece}

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Articlehistory:

Received16April2016 Accepted2August2016

Availableonline12September2016

Keywords:

Reactivepericarditis

Neisseriameningitidis

Meningococcalmeningitis

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Meningococcalmeningitisisawellestablishedpotentialfatalinfectioncharacterizedby fever,headache,petechialrash,andvomitinginthemajorityofcases.However,protean manifestationsincludingabdominalpain,sorethroat,diarrheaandcough,eventhough rare,shouldnotbeoverlooked.Similarly,althoughdisseminatedinfectioncouldpotentially involvevariousorgan-targets,secondaryimmunerelatedcomplicationsincludingjointsor pericardiumshouldbedealtwithcaution,sincetheyremainunresponsivetoappropriate antibioticregimens.Weherebyreporttherarecaseofanotherwisehealthyadultfemale, presentingwithacute abdominalpain maskingNeisseriameningitidisserotypeB menin-gitis,latercomplicatedwithrecurrentreactivepericarditisdespiteappropriateantibiotic treatment.Therefollowsareviewofcurrentliterature.

©2016SociedadeBrasileiradeInfectologia.PublishedbyElsevierEditoraLtda.Thisisan openaccessarticleundertheCCBY-NC-NDlicense(http://creativecommons.org/licenses/ by-nc-nd/4.0/).

Introduction

Meningococcal meningitisrepresents asevere –potentially fatal–infection characterizedbyfever,headache,petechial rash,andvomiting.Increasedclinicalsuspicionandprompt diagnosisispivotaltoensurefavorableoutcomes.However, uncommonmanifestationsincludingabdominalpain,cough, arthritis,vasculitis,orpericarditiscanmisleadtheattending physician,whilerequiringcombinedtreatmentwithagents otherthanappropriateantibiotics.Acuteabdominalpainas

∗ _{Correspondingauthor.}

E-mailaddress:akin@upatras.gr(K.Akinosoglou).

initialmanifestationofmeningococcalinfectionisextremely uncommon, typically located around the right abdomen – commonlyaroundtherightiliacfossa.Itcanbecommonly mistakenforacutecholecystitis,appendicitis,ormesenteric adenitis.Therefore,patientstendtoinitiallypresentto surgi-calemergencydepartments.Pericarditisisalsoanuncommon (3–19%) but well-recognizedcomplicationofmeningococcal disease.1 _{Presenceofmultiplefactorsdifferentiatebetween} directinvasionbytheorganism(disseminatedmeningococcal diseasewithpericarditisorisolatedmeningococcal pericardi-tis), fromanimmunemediatedreactivepericarditis(RMP).1

http://dx.doi.org/10.1016/j.bjid.2016.08.005

1413-8670/©2016SociedadeBrasileiradeInfectologia.PublishedbyElsevierEditoraLtda.ThisisanopenaccessarticleundertheCC BY-NC-NDlicense(http://creativecommons.org/licenses/by-nc-nd/4.0/).

642

A

B

C

Fig.1–(A)ChestCTscan;(B)cardiacultrasound(parasternallongaxisview)revealedthepresenceofmild-to-moderate

amountofpericardialeffusionwithnohemodynamicderangement(yellowarrows);and(C)nopericardialeffusionwas

noted,following9daysofcorticosteroidtherapy.Ao,aorta;LV,leftventricle;RV,rightventricle;LA,leftatrium.

Weherebyreportthecaseofa28-year-oldotherwisehealthy female presenting in our surgical department with acute abdomenmasking meningococcalmeningitis,later compli-catedbyrecurrentepisodesofreactive pericarditis,despite appropriateantibiotictreatment.

Case

report

A 28-year-old Caucasian female presented at our hospi-tal complainingoffever, rigors,and severe epigastricpain, notsubsidingfollowingnonsteroidanti-inflammatorydrug administration, during the last 24h. Upon admission the patient was in poor condition, BP:120/80mmHg, T: 38.8◦C, GCS: 15/15, while physical examination revealed severe rebound tenderness along right upper quadrant and epi-gastrium. Blood tests came back to show WBC: 19.51K/␮L (92.5/2.7/4.7%),PLT:117.00K/␮L,PT:18.4s,INR:1.59,andCRP: 26IU/L.Electrocardiogram(EKG)andchest-X-ray(CXR)were unremarkable.Anemergencyabdominalultrasoundandlater CTscandid notreveal any causeofacuteabdomen. Inter-estingly,thepatientstartedcomplainingofheadacheduring herstayintheemergencydepartment.Atthetime,patient reassessment revealed increasednuchal rigidity and Kern-ing’ssignsuggestiveofcentralnervoussysteminvolvement. Lumbarpuncturerevealed15,200cellsofpolymorphonuclear predominance, Glu<5mg/dl and protein530mg/dl in cere-brospinalfluid (CSF).CSFlatex agglutinationtest andlater CSFandbloodculturesresultsshowedNeisseria meningitidis

groupBsensitivetoarangeofantibiotics,hencethepatient (followingpriorempirictherapyofvancomycin,ceftriaxone and dexamethasone) was put on ceftriaxone 4 grqd. The patientpresenteddramaticclinicalimprovementaweek fol-lowingIVtherapywithnearnormalizationofinflammatory markerswhileserologyforcommonviruses, including HIV andconsecutivebloodculturescamebacknegative.C3and C4complementconcentrationswerealsonormal.Tendays post-admissionthepatientstartedcomplainingofasharp ret-rosternalpainradiatingtothe leftscapula,associatedwith pericardial friction rub along the lower left sterna border. Noalterationsinhemodynamic,ABG,orotherblood param-eters including serum troponin I and creatine kinase MB were noted. However, EKG showed raised ST segments in

leads V2–V6, indicative of pericarditis. Chest CT scan and cardiacultrasoundconfirmeddevelopmentofmoderate peri-cardial effusion (Fig. 1Aand B).In the context ofprevious clinical improvement, negative serology for infectious and autoimmune diseases, and presence of medication – sen-sitive meningococcus strain, we decided that pericarditis was immune-mediatedandacombination of methylpred-nisoloneandcolchicineat24mgand0.5mgqd,respectively, was initiated. The patient showed clinical and radiologic improvementandwasdischarged19dayspostadmissionon atapering schemeofcorticosteroids(Fig.1C).Interestingly, approximatelyoneandahalfmonthslater–atthetimeon 4mgofmethylprednisolone –thepatientstarted complain-ingagainofretrosternalpain.Shevisitedatertiaryhospital whererecurrenceofmoderatepericardialfluidwasconfirmed, whilereinstitutionofmethylprednisolone8mg/dand ibupro-fen 600mg/tid was followed by gradual improvement and dischargeshortlyafter.Sincethen,thepatienthasagain pre-sented inour department twicewithrecurrentpericarditis whileonmethylprednisolonetapering.Aftereightmonthsof follow up andslow taperingscheme ofcorticosteroidsand NSAIDsthepatientremainsinexcellent condition,without symptomsandoutoftreatment.

Discussion

Acuteabdominalpainasaninitialmanifestationof meningo-coccal infection is extremely uncommon, and can present bothasanisolatedentity,aswellasinthecontextof meningo-coccalsepsis.Includingours,wehavetrackednomorethan 19 casesofsharp abdominalpainasinitialpresentationof invasivemeningococcaldiseaseingloballiterature(Table1). Despiteequallyinvolvingadultsandchildren,morethanhalf (60%)ofchildhoodcasesareundersixyearsofage.2–7_Based on available data, Neisseria meningitidis serotype C was the mostfrequentlyisolatedpathogen(∼48%ofcases).3,4,7–13_Two casesofserotypeB,similartoourcase,havealsobeen iden-tified, even though the former involving children.5,6 _Fever wasthemostfrequentaccompanyingsymptomwhilea sur-gical procedure following suspicionofacute abdomen was conductedin42%ofthesepatients.3–5,7,8,11,14 _{Theetiologyof} abdominalpainremainsobscure.Severaltheoriesattemptto explaintheunderlyingpathophysiologyassociatedwiththis

brazj infect dis.2016;20(6):641–644

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Table1–Casesofmeningococcemiapresentingasacuteabdomensince1974.

Reference Year Age(years) Clinicalmanifestation Serogroup Siteofisolation Surgery

Ourcase 2016 28 Febrile B CSF,bloodculture No

Austin21 _{2015 33 Febrile,vomiting&diarrhea Nodata Bloodculture No}

sanAlvarez2 _{2011 10month Febrile A Bloodculture No}

Hsia9 _{2009 13 Febrile,agitation C Bloodculture No}

Tomezzoli5 _{2008 4 Febrile B Bloodculture Yes}

deSouza22 _{2006 6 Febrile,myalgia C Peritonealfluid Yes}

Herault8 _{2006 14 Meningealsyndrome C Peritonealfluid,bloodculture Yes}

Kelly11 _{2004 28 Nodata C Peritonealfluid Yes}

Demeter23 _{1999 37 Febrile Nodata Bloodculture No}

1999 34 Febrile Nodata Bloodculture No

Winrow6 _{1999 3 Febrile B Bloodculture No}

1999 12 Febrile Nodata Bloodculture No

Schmid12 _{1998 21 Meningealsyndrome,exanthema C Bloodculture No}

Grewal10 _{1993 16 Meningealsyndrome,exanthema C Bloodculture No}

Kunkel4 _{1984 4 Febrile C Peritonealfluid Yes}

BarMeir14 _{1978 42 Febrile Nodata Peritonealfluid,bloodculture Yes}

1978 65 Febrile Nodata Peritonealfluid Yes

Bannatyne3 _{1977 4 Febrile C Peritonealfluid Yes}

Weintraub13 _{1974 32 Meningealsyndrome C CSF No}

Thistableillustratesreportedcasesofmeningococcemiapresentingasacuteabdomeningloballiteraturesince1974.Thetableisdividedinto 7verticalcolumnsindicatingreportedcase,yearofpublication,patient’sage,clinicalmanifestationuponpresentation,pathogenserotype,site ofNeisseriaisolationandwhethersurgerywascarriedoutrespectively.

CSF,cerebrospinalfluid.

clinical entity including, mesenteric hypoperfusion, septic epiploicmicroinfarctions,splanchnicinvasionvia hematoge-nousspreadorascendinginfectionfromtheurogenitaltract, orimmunecomplexdeposition.2

Contrary topurulent pericarditis, RMPrepresents alate complication and very few cases have been reported in literature.1,15–19 _{Itdevelopsmostfrequently6–15 daysafter} onset of illness and is characterized by a type 3 hyper-sensitivity reaction, either againstthe specific serotype of

the N. meningitidis or newly antigenic, damaged

pericar-dial tissue because of molecular mimicry with microbial antigens.20_{Severedisease,age(adultsandyoungteenagers),} and serogroup C seems to predispose to post-infectious

immune associated complications including arthritis, vas-culitis, pleuritis, or pericarditis.15,16,20 _{In line with these} observations, ourpatient was ayoung adult,presentingin poor clinical condition, with highly elevated inflammatory markers suggestive of severe disease, even though inter-estingly serogroup B(and not C) was finallyisolated. The pericardial fluid inRMP isserous and sterile,and isoften associatedwithpolyserositisnotresponsivetoantibioticsbut toNSAIDs.1,18_{RMPmaybemoreseverethanpurulent} peri-carditis and cardiac tamponade can be relatively frequent requiringhighdosagesofsteroidsand/orpericardiocentesis.20 Recurrentpericarditisisexceptionallyrareafterthe meningo-coccalinfection(Table2),whilethereasonsofitsrecurrence

Table2–Casesofrecurrentreactivemeningococcalpericaditisinliteraturesince1969. Reference Patient age Timeof pericarditis diagnosis Siteof isolation Neisseria meningitidis serogroup Clinical presentation Therapy Outcome

Chiappini15 _{10y/o 7d CSF C Meningitis Prednisone+Aspirin Recurrence}

ElBashir16 _{13y/o 7d Blood C Meningitis Dexamethasone+Ibuprofen,}

laterdiclofenacsodium

Recurrence

Dupont17 _{14y/o 3d CSF C Meningitis ASA Recurrence}

Lanchemayer18 _{45y/o 8d CSF Nodata Meningitis Hydrocortisone Recurrence}

Stange19 a _{Nodata Nodata CSF B Nodata Nodata Recurrence}

47y/o 7d CSF Nodata Meningitis ASA+NSAID+Pericardiocentesis Recurrence

Stephani24 _{14y/o 9d CSF,blood C Meningitis,}

endopthalmitis

Prednisone+Antibiotics Recurrence FuglsangHansen25a _{Nodata 11d Nodata Nodata Meningitis Steroid+Pericardiocentesis Recurrence}

Thistableillustratesreportedcasesofmeningococcalrecurrentreactivepericarditisingloballiteraturesince1969Thetableisdividedinto8 verticalcolumnsindicatingreportedcase,patient’sage,timeofpericarditisdiagnosisfollowingonsetofsymptoms,siteofNeisseriaisolation, pathogenserotype,clinicalmanifestationuponinitialpresentation,therapeuticschemeandoutcomerespectively.

d,days;CSF,cerebrospinalfluid;ASA,acetylsalicylicacid;NSAID,nonsteroidantiinflammatorydrug.

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remain unknown, even though genetic factors have been proposed.15–18_{Inthesecases,thecourseofthediseasemay} bechronicandunpredictable,regardlessofthetherapygiven orthetriggeringcause,whilecorticosteroiduse caninduce severedependence.

Conclusion

Itwould beintriguingtohypothesize thatsevere disease– commonlyassociatedwithhigherantigenicloads–couldhave triggeredovertimmunecomplexformationandlater deposi-tiontoabdominalvascularbedandpericardium,responsible forinitialpresentationandsecondary complication respec-tively.Carefulinitialexamination,closeobservationandhigh clinical suspicionmay berequiredso thatan atypical pre-sentation,aswellas,manifestationduringthecourseofthe disease isnotoverlooked,even afterappropriateantibiotic treatmentofmeningococcalmeningitishasoccurred.

Consent

Writteninformedconsentwasobtainedfromthepatientfor publicationofthiscasereportandanyaccompanyingimages. Acopy ofthewrittenconsentisavailableforreviewbythe Editorofthisjournal.

Conflicts

of

interest

Theauathorsdeclarenoconflictsofinterest.

Authors’

contributions

KA performed literaturereview, wrotethe manuscriptand designed figureand tables. AAwas the attending internal medicine resident, NK performed cardiologic assessments andconsultsandprovidedimages.CGoversawpatient’s man-agement.ALcriticallycorrectedthemanuscriptandwasthe attendinginfectiousdiseasesspecialist.

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