在日本患者ANCA-相关显微镜下血管炎中环磷酰胺的长期疗效:一项回顾性研究

翻译：李变变 校对：刘素苗审核：张莉芸

Long-termoutcomeandefficacyofcyclophosphamidetherapyinJapanesepatientswithANCA-associatedmicroscopicpolyangiitis:aretrospectivestudy

-IVE:Theaimofthisstudywastoelucidatetheefficacyofcyclophosphamide(CY)inJapanesepatientswithantineutrophilcystoplasmicantibody(ANCA)-associated microscopicpolyangiitis (MPA).METHODS:Sixty-fourpatients,newlydiagnosedwithANCA-associatedMPAwereincludedinthisretrospectivestudy.Thepatientsweredividedintotwogroups-donwhethertheyreceivedcombinationtherapyofCYandcorticosteroid(CS)(CYgroup,n=29)orCSalone(CSgroup,n=35)forremissioninduction.Theprimaryoutcomewasall-causemortality.RESULTS:MostpatientsintheCYgroupweretreatedwithoralCY.Betweenthetwogroups,therewerenodifferencesinthe-linecharacteristicsexceptforahigherproportionofmalepatientsintheCYgroup.Theremissionratewasnotsubstantiallydifferentbetweenthetwogroups(86.2%intheCYgroupvs.91.4%intheCSgroup).ThesurvivalratewasslightlyhigherintheCYgroupthanintheCSgroup(notstatisticallysignificant;0.86vs.0.77at1yearand0.73vs.0.64at5years,p=0.648).IntheCYgroup,thehazardratioafteradjustingforage,sex,Birminghamvasculitisactivityscorevalues,serumalbuminlevelsandC-reactiveprotein(CRP)levelswas0.657(95%CI,0.254-1.699;p=0.386).CONCLUSION:WeobservednoincreasedefficacyofCYinANCA-positiveMPAintheJapanesepatients,andhence,itsefficacymaybelimitedinthesepatients.

目的：本研究的目的是阐明环磷酰胺(CY)在日本患者ANCA-相关显微镜下血管炎中的有效性。方法:这项回顾性研究由六十四例新诊断患有ANCA-相关显微镜下血管炎（ MPA)的患者构成。根据他们是否接受联合治疗分为2组：CY（环磷酰胺）和皮质类固醇(CS)(CY组,n=29)或单独CS(CS组,n=35)。观察指标是全因死亡率。

结果:大多数CY组的病人口服CY。两组之间在基准特征上没有差异除了较高比例的CY组男性患者之外。两组之间的缓解率没有明显的不同（CY组86.2%vs.91.4%CS组)。生存率CY组略高于CS组(不显著;在1年0.86和0.77,0.73和0.64在5年,p=0.648)。在调整了年龄、性别之后CY组的危险比、伯明翰血管炎活动评分值、血清白蛋白水平和C反应蛋白(CRP)水平为0.657(95%CI,0.254--1.699;p=0.386)。结论:在CY组ANCA（+）MPA病人中我们并没有观察到其增加的疗效,因此,环磷酰胺的效果可能是有限。

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