成人still病如何诊治讲稿.ppt

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1、关于成人Still病如何诊治第一页，讲稿共八十页哦PekingUnionMedicalCollegeHospital2历史1896年，Bannatyne在Lancet上报道首例AOSD病例，但被误诊为“RA”1897年，GeorgeStill报道22例儿童慢性关节炎，即后来的JIA全身型（SystemiconsetofJIA），Still病临床三联征长期间歇性高热一过性特征性皮疹关节炎/痛法、德风湿学家（1943年Wissler，1946年Fanconi）亦报道类似病例，被称为Wissler-Fanconi综合征1964年，亚急性变应性败血症1971年，EricBywaters报道14例临床表

2、现类似的成人患者，标志着AOSD正式做为一种疾病被认识第二页，讲稿共八十页哦PekingUnionMedicalCollegeHospital3现状Still病：JRA中的系统型39Still病特异性一过性皮疹WBC12.0ESR40ANA及RF（）腕骨硬化次要标准（1分）发病年龄35岁关节炎前驱症状：咽痛网状内皮系统活化表现或肝功异常浆膜炎颈椎或跗骨硬化诊断判断疑诊AOSD：10分观察12周确诊AOSD：10分观察6个月诊断困惑？F/21弛张高热3周伴发热的浅红色斑疹双腕轻度疼痛咽痛WBC 2.3ESR 115诊断AOSD？治疗？第四十一页，讲稿共八十页哦PekingUnionMedical

3、CollegeHospital42日本Yamaguchi标准（1992年）主要指标1.间歇发热39，1wks2.关节痛，2wks3.典型皮疹4.WBC10（PMN0.80）次要指标1.咽痛2.淋巴结和/或脾大3.肝功能异常4.RF(-)和ANA(-)排除1.感染性疾病2.恶性肿瘤3.其他风湿病诊断判断：5项（至少2项主要指标）诊断困惑？M/80间歇发热3月固定红色斑丘疹关节肌肉疼痛咽痛，肝脾大WBC 3.0，N 90%ESR 115低血压/低血氧入ICU诊断AOSD？治疗？第四十二页，讲稿共八十页哦PekingUnionMedicalCollegeHospital43法国Bruno标准（200

4、2年）主要标准弛张热39关节痛一过性红斑咽炎PMN80%GF20%次要标准斑丘疹WBC10诊断判断4项主要，或3项主要2项次要第四十三页，讲稿共八十页哦PekingUnionMedicalCollegeHospital44三套标准孰优孰劣？第四十四页，讲稿共八十页哦PekingUnionMedicalCollegeHospital46AOSD新的治疗策略TNF-IL-1IL-6B细胞第四十六页，讲稿共八十页哦PekingUnionMedicalCollegeHospital47依那西普Asherson(2002),首例报道多种DMARDs血浆置换失败Etanercept+MTX+GCs临床表现

5、及实验室指标明显改善SerratriceJ(2003),病例报道AOSD+继发性肾脏淀粉样变引起肾病综合征AOSD改善蛋白尿缓解第四十七页，讲稿共八十页哦PekingUnionMedicalCollegeHospital48依那西普HusniME(2002),openlabeltrial,acohortof12pt基线状况：prednisone,MTX,andNSAIDsET用法：25mg2/周,第8周如无改善增至每周3次随访6个月疗效：压关节数改善67%，肿胀关节数63%第四十八页，讲稿共八十页哦PekingUnionMedicalCollegeHospital49英夫利昔单抗Cavagna

6、L(2001),3例慢性关节型AOSDPred+MTX无效infliximab(3mg/kgwk0,2,6,之后每8wksESR,CRP,铁蛋白，发热均改善第2周PtGA，PGA均改善，并维持至第50周GCs减量：from1530mg/dto712mg/第四十九页，讲稿共八十页哦PekingUnionMedicalCollegeHospital52英夫利昔单抗FautrelB(2005),法国大型观察性研究20pts,平均随访13个月GCMTX无效患者10例IFX,5例ET,5例序贯ETIFXCR:5pt(1ET,4IFX)PR:16/25例次(7/10ET,9/15IFX每组均4例失败（均为

7、JIA，对anti-TNF效果差）85最终停药（失效，或不良反应)第五十二页，讲稿共八十页哦PekingUnionMedicalCollegeHospital53阿那白滞素GodinhoF（2004)，onecasereport难治性AOSD:MTX,SASP,CsA,IVIG,TNF拮抗剂均失败+长期GCs引起严重不良反应Anakinra100mg/dsc+MTX25mg/wk+predl(20mg/d),andnaproxen关节炎和全身症状数天周缓解ESR/CRP正常长期维持MTX+anakinra第五十三页，讲稿共八十页哦PekingUnionMedicalCollegeHospita

8、l55IL-1blockadeNaumann,L(2010),caseseries,8pts大剂量GCs依赖、多种DMARDs及抗TNF-制剂无效Anakira100mg/d，SC随访648m临床症状、炎症指标均改善皮疹和关节炎在数h内显著缓解炎性指标在14周内正常激素减量至小剂量1例停药次日症状复发，恢复用药后好转第五十五页，讲稿共八十页哦PekingUnionMedicalCollegeHospital56托珠单抗IwamotoM（2002)，1ptreportMTX,CsA,GCs无效CRP,发热,关节痛显著改善DeBandt（2009），1ptcaseSabnis,G.R（2011），

9、1ptcase伴无菌性脑膜炎第五十六页，讲稿共八十页哦PekingUnionMedicalCollegeHospital57Rech,J.（2011）3casesreport第五十七页，讲稿共八十页哦PekingUnionMedicalCollegeHospital58利妥昔单抗Ahmadi-Simab,K（2006)，2casesreportsMTX、CsA、LEF、CTX、IVIG无效之后EntanerceptInfliximab，EntanerceptMTX，无效Rituximab375mg/m2，qw4多关节炎等症状缓解，炎症指标下降，激素减至5mg/dMTX/CsA随访6m稳定第五十

10、八页，讲稿共八十页哦PekingUnionMedicalCollegeHospital59Therapeutic algorithm for AOSD（2004）第五十九页，讲稿共八十页哦PekingUnionMedicalCollegeHospital60思考：中国患者的治疗策略？危险分层及时诊断强调规范的基础治疗患者教育和规律随访生物制剂作用机制现有证据安全性可获得性第六十页，讲稿共八十页哦PekingUnionMedicalCollegeHospital61NewstrategeAsageneralapproach,wesuggeststartingtreatmentwithanNSAI

11、Dbutmovingquickly(dayslater)toglucocorticoidsfollowedbybiologicagentsifASDdoesnotcomeundercontrol.PatientswhoareonthesickerendoftheASDdiseasespectrumshouldbetreatedwithglucocorticoidsfromtheoutsetoftherapy,followedbybiologicagentsifthediseaseprovesrefractorywesuggestusingaTNFinhibitorastheinitialbio

12、logicagentinASDnotcontrolledwithNSAIDsandglucocorticoids,andmovingtoanakinraifaresponseisnotevidentwithintwotofourweeks(Grade2C).DMARDsnowgenerallyplayanadjunctiveroleinthetreatmentofASD.Methotrexateisoftenusedinconjunctionwithbiologictherapies.第六十一页，讲稿共八十页哦PekingUnionMedicalCollegeHospital62参考文献1.R

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14、ckerA,KochS,etal.Anakinrainpatientswithtreatment-resistantadult-onsetStillsdisease:fourcasereportswithserialcytokinemeasurementsandareviewoftheliterature.SeminArthritisRheum2007;37:189-97.4.SabnisGR,GokhaleYA,KulkarniUP.Tocilizumabinrefractoryadult-onsetStillsdiseasewithasepticmeningitis-efficacyofi

15、nterleukin-6blockadeandreviewoftheliterature.SeminArthritisRheum2011;40:365-8.5.ColinaM,ZucchiniW,CiancioG,OrzincoloC,TrottaF,GovoniM.Theevolutionofadult-onsetstilldisease:anobservationalandcomparativestudyinacohortof76italianpatients.SeminArthritisRheum2011;41:279-85.6.ElkonKB,HughesGR,BywatersEG,e

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17、taniN.Interleukin-18asanoveldiagnosticmarkerandindicatorofdiseaseseverityinadult-onsetStillsdisease.ArthritisRheum2001;44:1716-7.9.IwamotoM,NaraH,HirataD,MinotaS,NishimotoN,YoshizakiK.Humanizedmonoclonalanti-interleukin-6receptorantibodyfortreatmentofintractableadult-onsetStillsdisease.ArthritisRheu

18、m2002;46:3388-9.10.HusniME,MaierAL,MeasePJ,etal.EtanerceptinthetreatmentofadultpatientswithStillsdisease.ArthritisRheum2002;46:1171-6.11.DhoteR,SimonJ,PapoT,etal.Reactivehemophagocyticsyndromeinadultsystemicdisease:reportoftwenty-sixcasesandliteraturereview.ArthritisRheum2003;49:633-9.12.FitzgeraldA

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20、14.FranchiniS,DagnaL,SalvoF,AielloP,BaldisseraE,SabbadiniMG.Efficacyoftraditionalandbiologicagentsindifferentclinicalphenotypesofadult-onsetStillsdisease.ArthritisRheum2010;62:2530-5.15.MarkusseHM,StolkB,vanderMeyAG,deJonge-BokJM,HeeringKJ.SensorineuralhearinglossinadultonsetStillsdisease.AnnRheumDi

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22、十二页，讲稿共八十页哦PekingUnionMedicalCollegeHospital63参考文献18.GodeauB,LeportC,PerronneC,Salmon-CeronD,VildeJL,KahnMF.LongtermevolutionofadultonsetStillsdiseaseseeninaninfectiousdiseasesdepartment.AnnRheumDis1991;50:968.19.FujiiT,AkizukiM,KamedaH,etal.MethotrexatetreatmentinpatientswithadultonsetStillsdisease

23、-retrospectivestudyof13Japanesecases.AnnRheumDis1997;56:144-8.20.VignesS,LeMoelG,FautrelB,WechslerB,GodeauP,PietteJC.PercentageofglycosylatedserumferritinremainslowthroughoutthecourseofadultonsetStillsdisease.AnnRheumDis2000;59:347-50.21.KraetschHG,AntoniC,KaldenJR,MangerB.Successfultreatmentofasmal

24、lcohortofpatientswithadultonsetofStillsdiseasewithinfliximab:firstexperiences.AnnRheumDis2001;60Suppl3:iii55-7.22.AshersonRA,PascoeL.AdultonsetStillsdisease:responsetoEnbrel.AnnRheumDis2002;61:859-60;authorreply60.23.HamidouMA,DenisM,BarbarotS,BoutoilleD,BeliznaC,LeMoelG.Usefulnessofglycosylatedferr

25、itininatypicalpresentationsofadultonsetStillsdisease.AnnRheumDis2004;63:605.24.VasquesGodinhoFM,ParreiraSantosMJ,CanasdaSilvaJ.RefractoryadultonsetStillsdiseasesuccessfullytreatedwithanakinra.AnnRheumDis2005;64:647-8.25.AarntzenEH,vanRielPL,BarreraP.RefractoryadultonsetStillsdiseaseandhypersensitivi

26、tytonon-steroidalanti-inflammatorydrugsandcyclo-oxygenase-2inhibitors:arebiologicalagentsthesolution?AnnRheumDis2005;64:1523-4.26.Ahmadi-SimabK,LamprechtP,JankowiakC,GrossWL.SuccessfultreatmentofrefractoryadultonsetStillsdiseasewithrituximab.AnnRheumDis2006;65:1117-8.27.ArletJB,LeTH,MarinhoA,etal.Re

27、activehaemophagocyticsyndromeinadult-onsetStillsdisease:areportofsixpatientsandareviewoftheliterature.AnnRheumDis2006;65:1596-601.28.EfthimiouP,PaikPK,BieloryL.DiagnosisandmanagementofadultonsetStillsdisease.AnnRheumDis2006;65:564-72.29.KallioliasGD,GeorgiouPE,AntonopoulosIA,AndonopoulosAP,LiossisSN

28、.Anakinratreatmentinpatientswithadult-onsetStillsdiseaseisfast,effective,safeandsteroidsparing:experiencefromanuncontrolledtrial.AnnRheumDis2007;66:842-3.30.RuizPJ,MasliahE,DohertyTA,QuachA,FiresteinGS.Cardiacdeathinapatientwithadult-onsetStillsdiseasetreatedwiththeinterleukin1receptorinhibitoranaki

29、nra.AnnRheumDis2007;66:422-3.31.DeBandtM,Saint-MarcouxB.Tocilizumabformultirefractoryadult-onsetStillsdisease.AnnRheumDis2009;68:153-4.32.ChenDY,ChenYM,HoWL,ChenHH,ShenGH,LanJL.Diagnosticvalueofprocalcitoninfordifferentiationbetweenbacterialinfectionandnon-infectiousinflammationinfebrilepatientswith

30、activeadult-onsetStillsdisease.AnnRheumDis2009;68:1074-5.33.NaumannL,FeistE,NatuschA,etal.IL1-receptorantagonistanakinraprovideslong-lastingefficacyinthetreatmentofrefractoryadult-onsetStillsdisease.AnnRheumDis2010;69:466-7.34.RechJ,RonnebergerM,EnglbrechtM,etal.Successfultreatmentofadult-onsetStillsdiseaserefractorytoTNFandIL-1blockadebyIL-6receptorblockade.AnnRheumDis2011;70:390-2.第六十三页，讲稿共八十页哦PekingUnionMedicalCollegeHospital64第六十四页，讲稿共八十页哦PekingUnionMedicalCollegeHospital65谢谢 谢谢！第六十五页，讲稿共八十页哦05.04.2023感感谢谢大大家家观观看看第八十页，讲稿共八十页哦