B-Cell Depletion Therapy in Systemic Sclerosis Experimental Rationale and Update on Clinical Evidence英文文献资料.docVIP

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B-Cell Depletion Therapy in Systemic Sclerosis Experimental Rationale and Update on Clinical Evidence英文文献资料.doc

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B-Cell Depletion Therapy in Systemic Sclerosis Experimental Rationale and Update on Clinical Evidence英文文献资料

HindawiPublishingCorporation InternationalJournalofRheumatology Volume2011,ArticleID214013,7pages doi:10.1155/2011/214013 ReviewArticle B-CellDepletionTherapyinSystemicSclerosis: ExperimentalRationaleandUpdateonClinicalEvidence DimitriosDaoussis,Stamatis-NickC.Liossis, GeorgiosYiannopoulos,andAndrewP.Andonopoulos DivisionofRheumatology,DepartmentofInternalMedicine,PatrasUniversityHospital,UniversityofPatrasMedicalSchool, Rion,26504Patras,Greece CorrespondenceshouldbeaddressedtoDimitriosDaoussis,jimdaoussis@ Received24February2011;Revised4June2011;Accepted7June2011 AcademicEditor:LauraK.Hummers Copyright?2011DimitriosDaoussisetal.ThisisanopenaccessarticledistributedundertheCreativeCommonsAttribution License,whichpermitsunrestricteduse,distribution,andreproductioninanymedium,providedtheoriginalworkisproperly cited. Systemicsclerosis(SSc)isasystemicrheumaticdiseasewithpoorprognosissincetherapeuticoptionsarelimited.Recentevidence fromanimalmodelssuggeststhatB-cellsmaybeactivelyinvolvedinthe?broticprocess.B-cellsfromtightskinmice,ananimal model of scleroderma, display a “hyperresponsive” phenotype; treatment with rituximab (RTX) signi?cantly attenuates skin ?brosisinthisanimalmodel.Inhumans,B-cellin?ltrationisaprominent?ndinginmostlungbiopsiesobtainedfrompatients withSSc-associatedinterstitiallungdisease.Severalopenlabelstudieshaveassessedtheclinicale?cacyofRTXinSSc.Inmost patientsskin?brosisimproved;lungfunctioneitherimprovedorremainedstable.De?niteconclusionsregardingtheclinical e?cacyofRTXinSSccannotbedrawnbutfurtherexplorationwithamulticenter,randomizedstudyiswarranted. 1.Introduction question to some extent, reporting encouraging results. In thispaperweprovidetheexperimentalevidencesupporting the active role of B-cells in ?brosis and summarize all theavailableclinicalevidenceregardingtheuseofRTXin patientswithSSc. Systemic sclerosis (SSc) is a systemic rheumatic disease characterizedbyvasculopathy,autoimmunity,and?brosis. Theavailabletherapeuticoptionsareextremely