EULARBD治疗建议.ppt

EULARrecommedationsforthemanagementofBehcetdisease AnnRheumDis2008 67 1656 1662 目的和方法 基于循证医学证据 建立治疗BD的EULAR建议专家委员会 风湿病专家9人 眼病专家3人 内科学专家1人 皮肤病专家1人 神经病学专家1人 文献检索 MedlineandCochraneLibrarydatabase 1966 2006 1 Eyeinvolvement AnypatientwithBDandinflammatoryeyediseaseaffectingtheposteriorsegmentTreatmentregimeincludesazathioprine RCT 2 5mg kg d andsystemiccorticosteroids 白内障 青光眼 2 Refractoryeyeinvolvement Patienthassevereeyediseasedefinedas 2linesofdropinvisualacuityona10 10scaleand orretinaldisease retinalvasculitisormacularinvolvement RecommendedthateitherciclosporineA 2 5mg kg d 3RCT Hypertensionandnephrotoxicity orinfliximab relapse Tb beusedincombinationwithazathioprineandcorticosteroids AlternativelyIFNa depression cytopenia withorwithoutcorticosteroidscouldbeusedinstead 3 Majorvesseldisease ThereisnofirmevidencetoguidethemanagementofmajorvesseldiseaseinBD Forthemanagementofacutedeepveinthrombosis 血管壁炎症 inBDimmunosuppressiveagentssuchascorticosteroids azathioprine cyclophosphamideorciclosporineAarerecommended Forthemanagementofpulmonaryandperipheralarterialaneurysms cyclophosphamide 静脉冲击 andcorticosteroidsarerecommended 4 Anticoagulation Similarlytherearenocontrolleddataon orevidenceofbenefitfromuncontrolledexperiencewithanticoagulants antiplateletorantifibrinolyticagentsinthemanagementofdeepveinthrombosisorfortheuseofanticoagulationforthearteriallesionsofBD rarePE avoidfatalbleeding 不能减少DVT复发 5 Gastrointestinalinvolvement Thereisnoevidence basedtreatmentthatcanberecommendedforthemanagementofgastrointestinalinvolvementofBD Agentssuchassulfasalazine corticosteroids azathioprine TNFaantagonistsandthalidomideshouldbetriedfirstbeforesurgery exceptinemergencies 6 Jointinvolvement InmostpatientswithBD arthritis mildandtransient nonerosive largejoints canbemanagedwithcolchicine 1 2mg d 2RCT 7 Neurologicalinvolvement TherearenocontrolleddatatoguidethemanagementofCNSinvolvementinBD Forparenchymalinvolvementagentstobetriedmayincludecorticosteroids MP静脉冲击1g d 3 7d IFNa azathioprine cyclophosphamide methotrexateandTNFaantagonists Forduralsinusthrombosiscorticosteroidsarerecommended 8 CyclosporineAneurotoxicity CyclosporineAshouldnotbeusedinBDpatientswithcentralnervoussysteminvolvementunlessnecessaryforintraocularinflammation 3casecontrolstudies 9 Mucocutaneousinvolvement Thedecisiontotreatskinandmucosainvolvementwilldependontheperceivedseveritybythedoctorandthepatient Mucocutaneousinvolvementshouldbetreatedaccordingtothedominantorcodominantlesionspresent Topicalmeasures ie localcorticosteroids shouldbethefirstlineoftreatmentforisolatedoralandgenitalulcers 9 Mucocutaneousinvolvement Acne likelesionsareusuallyofcosmeticconcernonly Thus topicalmeasuresasusedinacnevulgarisaresufficient Colchicineshouldbepreferredwhenthedominantlesioniserythaemanodosum Legulcers post thrombotic vasculitic inBDmighthavedifferentcauses Treatmentshouldbeplannedaccordingly Azathioprine IFNa 1RCT andTNFaantagonists 1RCTforetanercept maybeconsideredinresistantcases Thalidomide Categoryofevidence strengthofrecommendationsandlevelofagreementofrecommendations Recommendationno CategoryofevidStrengthofrecomm1EyeinvolvementIbA D2RefractoryeyeinvolvementIb IIbC D3MajorvesseldiseaseI