成人still’s病0.ppt

成人Still's病AdultOnsetStill'sDisease，AOSDStill’s病概述定义流行病学病因及发病机制病因-感染因素病因-遗传因素病因-免疫异常发病机制临床特征症状和体征（1）发热（2）皮疹皮疹特征（3）关节痛及关节炎肌痛关节受累-特征关节受累（4）咽痛（5）其他临床表现临床表现实验室检查实验室检查铁蛋白（SF）铁蛋白IL-18组织病理学检查影像学检查诊断诊断标准（美国Cush标准）诊断标准（日本1992年）诊断要点AOSD的临床量表注意事项鉴别诊断治疗NSAIDs糖皮质激素DMARDs常用的DMARDs如下：1)MTX：口服、肌注或静注均有效口服60%吸收多采用每周一次给药常用剂量为7.5~15mg/周不良反应：常见恶心、口腔炎、腹泻、脱发、皮疹少数出现骨髓抑制、听力损害和肺间质变可引起流产、畸胎和影响生育力服药期间，应定期查血常规和肝功能2)来氟米特（LEF）：剂量为：10~20mg/d主要不良反应：有腹泻、瘙痒、高血压、皮疹肝酶增高、脱发和一过性白细胞下降等有致畸作用服药期间应定期查血常规和肝功能6）环孢素A（CsA）：口服:起始量为3~5mg/(kg•d)维持量为2~3mg/(kg•d)常见毒副作用：高血压、肝肾毒性、神经系统损害继发感染、肿瘤及胃肠道反应、齿龈增生、多毛等生物制剂其他转归预后预后相关因素参考文献7.ZouYQ,LuLJ,LiSJ,etal.Thelevelsofmacrophagemigrationinhibitoryfactorasanindicatorofdiseaseactivityandseverityinadult-onsetStill’sdisease.ClinicalBiochemistry.2008;41(7-8):519–524.8.JungSY,ParkYB,HaYJ,LeeKH,LeeSK.Serumcalprotectinasamarkerfordiseaseactivityandseverityinadult-onsetstill’sdisease.JournalofRheumatology.2010;37(5):1029–1034（治疗）9.FautrelB.Adult-onsetStilldisease.BestPracticeandResearch.2008;22(5):773–792.10.SabnisGR,GokhaleYA,KulkarniUP.TocilizumabinrefractoryAdult-OnsetStill’sDiseasewithasepticmeningitis-efficacyofinterleukin-6blockadeandreviewoftheliterature.SeminarsinArthritisandRheumatism.2011;40(4):365–36811.BagnariV,ColinaM,CiancioG,GovoniM,TrottaF.Adult-onsetStill’sdisease.RheumatologyInternational.2010;30(7):855–862.12.WoutersJMGW,ReekersP,VandePutteLBA.Adult-onsetStill’sdisease:DiseasecourseandHLAassociations.ArthritisandRheumatism.1986;29(3):415–418.（非甾）13.EfthimiouP,PaikPK,BieloryL.DiagnosisandmanagementofadultonsetStill’sdisease.AnnalsoftheRheumaticDiseases.2006;65(5):564–572.（激素）14.FranchiniS,DagnaL,SalvoF,AielloP,BaldisseraE,SabbadiniMG.Efficacyoftraditionalandbiologicagentsindifferentclinicalphenotypesofadult-onsetStill’sdisease.ArthritisandRheumatism.2010;62(8):2530–2535.（甲氨蝶呤）15.MaeshimaK,IshiiK,IwakuraM,etal.Adult-onsetStill’sdiseasewithmacrophageactivationsyndromesuccessfullytreatedwithacombinationofmethotrexateandetanercept.ModernRheumatology.2012;22(1):137–141.16.F