rev bras reumatol.2015;55(6):528–530
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
Rheumatoid
vasculitis
–
Case
report
夽
Inah
Maria
Drummond
Pecly
a,∗,
Juan
Felipe
Ocampo
b,
Guillermo
Pandales
Ramirez
b,
Hedi
Marinho
de
Melo
Guedes
de
Oliveira
b,
Claudia
Guerra
Murad
Saud
c,
Milton
dos
Reis
Arantes
baUniversidadeFederaldoRiodeJaneiro,RiodeJaneiro,RJ,Brazil
bSantaCasadaMisericórdiadoRiodeJaneiro,RiodeJaneiro,RJ,Brazil
cUniversidadeFederalFluminense,Niterói,RJ,Brazil
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Articlehistory: Received23June2013 Accepted18July2014
Availableonline27November2014
Keywords: Vasculitis
Rheumatoidarthritis Rheumatoidvasculitis
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b
s
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c
t
Rheumatoidarthritis(RA)isachronicsystemicinflammatoryautoimmune diseaseand itsmainmanifestationispersistentsynovitisaffectingperipheraljointssymmetrically,In spiteofitsdestructivepotential,theevolutionofRAishighlyvariable.Somepatientsmay haveonlyashort-termprocessoligoarticularwithminimumlesion,whileotherssuffera polyarthritisevolvingwithprogressiveandcontinuousinvolvementofotherorgansystems suchasskin,heart,lungs,musclesandbloodvesselsrarelyleadingtorheumatoidvasculitis. Theaimofthisstudywastodescribeacaseofrheumatoidvasculitisarareandsevere condition.
©2014ElsevierEditoraLtda.Allrightsreserved.
Vasculite
reumatoide
–
Relato
de
caso
Palavras-chave: Vasculite
Artritereumatoide Vasculitereumatoide
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e
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Aartritereumatoide(AR)éumadoenc¸acrônicaautoimuneinflamatóriasistêmicaesua principalmanifestac¸ãoéasinovitepersistente,quecomprometearticulac¸õesperiféricas deformasimétrica.Apesardoseupotencialdestrutivo,aevoluc¸ãodaARémuitovariável. Algunspacientespodemterapenasumprocessodecurtadurac¸ãooligoarticularcomlesão mínima,enquantooutrossofremumapoliartriteprogressivaecontínuaeevoluemcom acometimentodeoutrosórgãosesistemas,comopele,corac¸ão,pulmões,músculosemais raramentevasossanguíneos,quelevaàvasculitereumatoide.Oobjetivodesteestudofoi descreverumcasodevasculitereumatoide,umacondic¸ãoraraegrave.
©2014ElsevierEditoraLtda.Todososdireitosreservados.
夽
WorkconductedatSantaCasadaMisericórdiadoRiodeJaneiro–ProfessorMiltondosReisArantes4thand20thwardsService. ∗ Correspondingauthor.
E-mailaddresses:[email protected],[email protected](I.M.D.Pecly). http://dx.doi.org/10.1016/j.rbre.2014.07.006
rev bras reumatol.2015;55(6):528–530
529
Introduction
Systemic vasculitis hasbeen adiagnostic challenge inthe areasofclinicalmedicineandrheumatologyformanyyears. Therheumatoidvasculitis,rare andsevere complicationof rheumatoid arthritis, is one of them. The most common cutaneouspresentationofrheumatoidvasculitisisthe devel-opmentofpalpablepurpurainthelowerextremitiesthatifnot treatedearlyenoughcanevolveintoamulti-organsystemic vasculitis.1
Case
report
64-year old,non-Caucasian,femalepatient, withRA, with-outtreatmentfor25 years.Sixyearsago,developededema inherleftlowerlimb,erythemaandhardlumpsthat ulcer-atedinthelaterallegregionwithmoderateserosanguineous secretion.Shewastreatedwithmultipletopicalandsystemic antibiotics,butshowednoimprovement.Thesizeofthelesion evolveduntilitinvolvedthedistaltwo-thirdsoftheleftlower limb.Twoyearslater,shepresentedsimilarulcerationinthe rightleg, forwhich shereceivedseveral topical, unspecific treatments,butdidnotobtainagoodresponse.Duetothe impairmentofhergeneralcondition andworsening ofthe lesions,shesoughtahealthcareunit,whereshewas eval-uatedandreferredforhospitaltreatment.Uponadmissionto thehospital,thepatientwasinregular generalcondition– emaciated,presentingastheniaandanemia,claiming morn-ingstiffnessforoveranhour,showingulnardeviationofthe fingersinsymmetricalhandswithbonyprominencesbothin themetacarpophalangealand theproximalinterphalangeal joints(Fig.1),impairmentofbilateralelbowsandknees.She alsopresentedshallow-edgedulceratedlesionswith moder-ateserosanguineoussecretioninthedistaltwo-thirdsofher lowerlimbs(Fig.2).
Fig.1–Handdeformities.Ulnardeviationofthefingers withbonyprominencesbothinthemetacarpophalangeal andtheproximalinterphalangealjoints,secondaryto rheumatoidarthritis.
Fig.2–Vasculitisinlowerlimbs.Shallow-edgedulcerated vasculiticlesionswithmoderateserosanguineoussecretion inthedistaltwo-thirdsofthelowerlimbs,secondaryto rheumatoidarthritis.
ShewasdiagnosedwithseropositiveRAaccordingtothe criteriaofthe1987AmericanCollegeofRheumatology, pre-sentingoverfourcriteria(morningstiffness,arthritisof3or morejointareas,arthritisofhandjoints,symmetricarthritis andpositiverheumatoidfactor)andscoringgreaterthan6in thenewclassificationcriteriaforrheumatoidarthritis,2010 ACR/EULAR(jointinvolvementscore5,serologyscore3, dura-tionofsymptomsscore1),DASdiseaseactivityscore28:4.83 (moderate activity), complicated by rheumatoid vasculitis. Thediagnosticwasmadeaccordingtoclinicalevaluationof therheumatologyservice.Shewastreatedwithmethotrexate 15mg/weekaftersystemicantibioticsandtopicaltreatment oftheulcers,showingsignificantimprovementofthelesions in the lower limbs and of her generalcondition. She was dischargedfromhospitaltocontinuethetreatmentas out-patient.
Laboratory
tests
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rev bras reumatol.2015;55(6):528–530Discussion
Rheumatoid arthritis (RA) is a relatively common chronic inflammatory autoimmune disease that affects men and womenofallages,withworldwidepresence.Itaffects0.3–2.1% oftheworldpopulation,butinsome,suchastheblack pop-ulationintheCaribbeanandruralpopulationinSub-Saharan Africa,thediseaseislessrecurrentandlesssevere.2InBrazil,
it isestimatedthataround 1.3millionpeople are affected, beingmorecommonintheagegroupbetween30and50years old,mostlywomenthanman,inaproportionof3:1.3Its
clin-icalpresentationischaracterizedbythejointinvolvement,in aninsidiousprocessthatoftenresultsindeformities. How-ever, it isimportantto acknowledgethatRA isasystemic disease.Becauseofthat,inadditiontojointmanifestations, severalorgansandspecificsystemscanbeaffected,enabling the onset ofvarious extra-articular manifestations.4 These
manifestationsareusuallyobservedinindividualswithhigh titersofrheumatoid factorand anti-CCP.5 Ourpatient
pre-sentedrheumatoidfactorof94UI/mL(referencelimitupto 20UI/mL),threetimesgreaterthantheupperlimitofnormal. Rheumatoidvasculitis is aform ofcutaneous presenta-tion with annual incidence of 12.5/1 million that typically affects small and medium-sized vessels, with associated peripheralneuropathy (often motor), digital gangrene,nail bed infarcts and palpable purpura.6 The spectrumof
clin-icallesions reported inthe rheumatoid vasculitis is broad andvarieswiththesizeandthelocationoftheaffected ves-selsandtheextentofthedisease.7Thisisarareandsevere
complicationthatmustbediagnosedearlyenough,giventhe largeinterferenceintheevolution,treatmentandprognosis ofthe underlying disease.Our patientdid notundergo an anatomopathologicalexamination(biopsy)as it isnot eas-ilyaccessibleintheinstitution.Thediagnosiswasbasedon theclinicalpresentationandtheexperienceoftheHospital’s rheumatologyservice.
Duetothepresenceofneutropenia,weinvestigatedthe possibility of Felty’s syndrome (rheumatoid arthritis, neu-tropenia and splenomegaly). However, the patient did not showsignsofsplenomegalyinthephysicalexaminationor intheabdominalultrasound,removingthishypothesis.
The patient presented microcytic, hypochromic anemia withlowironlevels and highRDW, lowtransferrin satura-tionindexcompatiblewithiron-deficiencyanemia,associated tolowingestionofspecificfoods,accordingtodietaryrecall, associatedtoanemiaofchronicdisease.
Oneoftheaspectsthatmakethiscaseparticularly interest-ingisthefactthatthisdiseaseevolvedformanyyearswithout anykindoftreatmentfortheunderlyingdisease(rheumatoid arthritis)orthevasculitis,whichhighlightstheimportanceof properdiagnosisandearlytreatmentofthedisease.
Conclusion
Despite being arare condition,it isimportantto beaware ofthe developmentofrheumatoid vasculitisinthe clinical courseofrheumatoidarthritissotheappropriatetreatment canbepromptlyinstituted,thusavoidingtheprogressionof lesionsandpossibleassociatedcomplications.
Conflicts
of
interest
Theauthorsdeclarenoconflictofinterest.
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5.MonganES,CassRM,JacoxRF,VaughenJH.Astudyofthe relationofseronegativeandseropositiverheumatoidarthritis toeachotherandtonecrotizingvasculitis.AmJMed (Rochester,NY).1969;47:23–35.
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