JAMA neurology:重症肌无力患者不同激素治疗方案的效果比较

2021-02-13 MedSci原创 MedSci原创

对于需要大剂量强的松与硫唑嘌呤治疗的中重度MG患者,选择强的松快速减量治疗不仅耐受性好,而且临床结局更佳。

重症肌无力(MG)是一种由神经-肌肉接头处传递功能障碍所引起的自身免疫性疾病,临床主要表现为部分或全身骨骼肌无力和易疲劳,活动后症状加重,经休息后症状减轻。患病率为77~150/100万,年发病率为4~11/100万。女性患病率大于男性,约3:2,各年龄段均有发病,儿童1~5岁居多。

MG患者发病初期往往感到眼或肢体酸胀不适,或视物模糊,容易疲劳,天气炎热或月经来潮时疲乏加重。随着病情发展,骨骼肌明显疲乏无力,显著特点是肌无力于下午或傍晚劳累后加重,晨起或休息后减轻,此种现象称之为“晨轻暮重”。

通常,MG的治疗包括胆碱酯酶抑制剂、免疫抑制剂、血浆置换、静脉注射免疫球蛋白等。其中,肾上腺皮质类固醇激素如强的松、甲强龙等是最为常用的免疫抑制剂。

然而,激素治疗MG后如何减量却仍是一个临床难题。同时,许多指南中推荐的方案也尚未得到大型研究的炎症。为此,来自国际MYACOR研究小组的专家们针对泼尼松标准缓速减量及快速减量的疗效进行了全面的比较。结果发表在最新的《美国医学会杂志》子刊JAMA Neurology杂志上。

该研究小组在200961日至2013731日期间,进行了一项多中心、平行、单盲随机试验,旨在比较上述2种激素减量方案。参与者为从法国7个专业中心招募的共2291名确诊为中重度MG的患者。

将两组患者随机分配至缓慢减量组(强的松剂量逐渐增加至1.5mg/kg,每隔一天一次,一旦达到MG缓解状态,则缓慢减少)与快速治疗组(确诊后立即每天高剂量强的松0.75mg/kg给药,一旦达到MG改善状态,则提前快速减少剂量)。所有参与者同时使用最高剂量为3mg/kg/d的硫唑嘌呤治疗。

该研究的主要重点事件是12个月时达到无激素治疗的缓解状态,且15个月时无临床复发。 在评估的2291名患者中,2086名患者不符合纳入标准,87名患者拒绝参与。因此,共117名患者(慢速组58名,快速组59名)最终纳入该研究并进行随机分组。

参与者包括62名男性(53%);中位年龄为65岁(四分位数范围,35-69岁)。在调整混杂因素后结果显示,与慢速组相比,快速组达到主要结果的患者比例较高(23 [39%] vs 5 [9%]),HR为3.61(95% CI,1.64-7.97;P < .001)。

进一步分析显示,选用快速治疗方案的患者比慢速组1年内减少1898mg(95%CI,-3121~-461mg)泼尼松(即每位患者5.3mg/d,P = .03)的摄入。此外,两组严重不良事件的数量之间没有显著差异(13 [22%] vs 21 [36%]P = 0.15)。

综上,该研究提示,对于需要大剂量强的松与硫唑嘌呤治疗的中重度MG患者,选择强的松快速减量治疗不仅耐受性好,而且临床结局更佳。

 

参考文献:

Sharshar T, et al. Comparison of Corticosteroid Tapering Regimens in Myasthenia Gravis: A Randomized Clinical Trial. JAMA Neurol. Published online February 08, 2021. doi:10.1001/jamaneurol.2020.5407

 

 

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    2021-07-14 静心

    学习了

    0

  4. 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    2021-02-13 旺医

    顶刊就是顶刊,谢谢梅斯带来这么高水平的研究报道,我们科里同事经常看梅斯,分享梅斯上的信息

    0

  8. 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topicName=null, topicId=null, topicList=[], attachment=null, authenticateStatus=null, createdAvatar=null, createdBy=f0620, createdName=CHANGE, createdTime=Sat Feb 13 20:42:39 CST 2021, time=2021-02-13, status=1, ipAttribution=), GetPortalCommentsPageByObjectIdResponse(id=924474, encodeId=601f9244e4f3, content=认真学习了, beContent=null, objectType=article, channel=null, level=null, likeNumber=64, replyNumber=0, topicName=null, topicId=null, topicList=[], attachment=null, authenticateStatus=null, createdAvatar=https://wx.qlogo.cn/mmopen/NUyjXTCJjo6EF3hKja8nER8RMzxQdria9eCYFhgLTLsDOeqnvIYdeLDz5eP6EnicLuE5gibiawgno4zUA0jAR7Nbtp2llQ9fs3Mk/0, createdBy=278f1986883, createdName=医鸣惊人, createdTime=Sat Feb 13 18:19:10 CST 2021, time=2021-02-13, status=1, ipAttribution=), GetPortalCommentsPageByObjectIdResponse(id=924472, encodeId=92009244e267, content=<a href='/topic/show?id=910e959e0c9' target=_blank style='color:#2F92EE;'>#重症肌无力#</a>, beContent=null, objectType=article, channel=null, level=null, 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    2021-02-13 CHANGE

    疗效只是效果的众多方面之一,还要看对患者的获益,包括生活质量等因素共同决定效果的

    0

  9. 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topicName=null, topicId=null, topicList=[], attachment=null, authenticateStatus=null, createdAvatar=null, createdBy=f0620, createdName=CHANGE, createdTime=Sat Feb 13 20:42:39 CST 2021, time=2021-02-13, status=1, ipAttribution=), GetPortalCommentsPageByObjectIdResponse(id=924474, encodeId=601f9244e4f3, content=认真学习了, beContent=null, objectType=article, channel=null, level=null, likeNumber=64, replyNumber=0, topicName=null, topicId=null, topicList=[], attachment=null, authenticateStatus=null, createdAvatar=https://wx.qlogo.cn/mmopen/NUyjXTCJjo6EF3hKja8nER8RMzxQdria9eCYFhgLTLsDOeqnvIYdeLDz5eP6EnicLuE5gibiawgno4zUA0jAR7Nbtp2llQ9fs3Mk/0, createdBy=278f1986883, createdName=医鸣惊人, createdTime=Sat Feb 13 18:19:10 CST 2021, time=2021-02-13, status=1, ipAttribution=), GetPortalCommentsPageByObjectIdResponse(id=924472, encodeId=92009244e267, content=<a href='/topic/show?id=910e959e0c9' target=_blank style='color:#2F92EE;'>#重症肌无力#</a>, beContent=null, objectType=article, channel=null, level=null, likeNumber=110, replyNumber=0, topicName=null, topicId=null, topicList=[TopicDto(id=95970, encryptionId=910e959e0c9, topicName=重症肌无力)], attachment=null, authenticateStatus=null, createdAvatar=https://img.medsci.cn/20220519/c2ab253484ee4527a2d4e9589a4821ac/45de9bf494a54becb2ea4369c9d11e85.jpg, createdBy=7a3710, createdName=健康达人, createdTime=Sat Feb 13 18:01:57 CST 2021, time=2021-02-13, status=1, ipAttribution=)]
    2021-02-13 医鸣惊人

    认真学习了

    0

  10. 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