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Ann Rheum Dis：免疫介导的坏死性肌病中抗SRP或抗HMGCR自身抗体阳性患者IgG的体内致病性

2018-10-15 xiangting MedSci原创

坏死性肌病抗SRP抗HMGCRIMNM
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本研究表明，来自患者的抗SRP+和抗HMGCR+ IgG 通过补体介导的机制在体内对肌肉具有致病性，最终确定了IMNM的自身免疫特征。

在自身免疫中，自身抗体（aAb）可能是疾病的简单生物标志物或真正的致病效应物。与抗信号识别颗粒（SRP）或抗-3-羟基-3-甲基CoA还原酶（HMGCR）aAb相关的特发性炎性肌病的一种类型已经被个体化，并被称为免疫介导的坏死性肌病（IMNM）。aAb水平与IMNM的活动性相关，并且疾病对免疫抑制治疗有反应，这表明aAb是致病性的。这项研究目的是通过建立IMNM的首个小鼠模型，评估抗SRP或抗HMGCR aAb阳性患者的IgG在体内的致病性。

将抗SRP或抗HMGCR相关性IMNM患者的IgG被动转移至野生型、Rag2-/-或补体C3-/-小鼠。通过电刺激和握力测试的肌肉力量来评估肌肉缺乏。苏木精/伊红染色后或通过免疫荧光或免疫组化分析进行组织学分析。通过可寻址激光珠测定（ALBIA）来定量抗体水平。

将IMNM患者的IgG被动转移至C57BL/6或Rag2-/-小鼠，以诱发肌肉缺乏。C3-/-小鼠中aAb的致病性降低，而通过补充人类补体致病性升高。通过SRP或HMGCR主动免疫破坏耐受性可以引起疾病。

本研究表明，来自患者的抗SRP+和抗HMGCR+ IgG 通过补体介导的机制在体内对肌肉具有致病性，最终确定了IMNM的自身免疫特征。这些数据支持使用血浆置换，并主张在IMNM中评估补体靶向疗法。

原始出处：

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    2019-01-29 智慧医人

    #抗体阳性#

    0

  2. [GetPortalCommentsPageByObjectIdResponse(id=1948104, encodeId=4f6a1948104d4, content=<a href='/topic/show?id=3aae55212db' target=_blank style='color:#2F92EE;'>#抗体阳性#</a>, beContent=null, objectType=article, channel=null, level=null, likeNumber=75, replyNumber=0, topicName=null, topicId=null, topicList=[TopicDto(id=55212, encryptionId=3aae55212db, topicName=抗体阳性)], attachment=null, authenticateStatus=null, createdAvatar=, createdBy=a12645, createdName=智慧医人, createdTime=Tue Jan 29 12:43:00 CST 2019, time=2019-01-29, status=1, ipAttribution=), GetPortalCommentsPageByObjectIdResponse(id=1718842, encodeId=95221e18842b2, content=<a href='/topic/show?id=d3e9864e750' target=_blank style='color:#2F92EE;'>#致病性#</a>, beContent=null, objectType=article, channel=null, level=null, likeNumber=103, replyNumber=0, topicName=null, topicId=null, topicList=[TopicDto(id=86477, encryptionId=d3e9864e750, topicName=致病性)], attachment=null, authenticateStatus=null, createdAvatar=null, createdBy=d36132650190, createdName=cnxcy, createdTime=Wed Mar 20 06:43:00 CST 2019, time=2019-03-20, status=1, ipAttribution=), GetPortalCommentsPageByObjectIdResponse(id=2012836, encodeId=67b8201283641, content=<a href='/topic/show?id=7d69598468' target=_blank style='color:#2F92EE;'>#Dis#</a>, beContent=null, objectType=article, channel=null, level=null, likeNumber=72, replyNumber=0, topicName=null, topicId=null, topicList=[TopicDto(id=5984, encryptionId=7d69598468, topicName=Dis)], attachment=null, authenticateStatus=null, createdAvatar=, createdBy=c689267, createdName=cmj8wellington, createdTime=Tue Oct 23 19:43:00 CST 2018, time=2018-10-23, status=1, ipAttribution=), GetPortalCommentsPageByObjectIdResponse(id=1362915, encodeId=407913629157a, content=<a href='/topic/show?id=b723414321c' target=_blank style='color:#2F92EE;'>#坏死#</a>, beContent=null, objectType=article, channel=null, level=null, likeNumber=75, replyNumber=0, topicName=null, topicId=null, topicList=[TopicDto(id=41432, encryptionId=b723414321c, topicName=坏死)], attachment=null, authenticateStatus=null, createdAvatar=, createdBy=c0e3195, createdName=millore, createdTime=Wed Oct 17 05:43:00 CST 2018, time=2018-10-17, status=1, ipAttribution=), GetPortalCommentsPageByObjectIdResponse(id=1615190, encodeId=87511615190b6, content=<a href='/topic/show?id=010c9506a3' target=_blank style='color:#2F92EE;'>#IgG#</a>, beContent=null, objectType=article, channel=null, level=null, likeNumber=82, replyNumber=0, topicName=null, topicId=null, topicList=[TopicDto(id=9506, encryptionId=010c9506a3, topicName=IgG)], attachment=null, authenticateStatus=null, createdAvatar=null, createdBy=6c4f19588882, createdName=ms5768352621950538, createdTime=Wed Oct 17 05:43:00 CST 2018, time=2018-10-17, status=1, ipAttribution=), GetPortalCommentsPageByObjectIdResponse(id=1622351, encodeId=5a771622351a7, content=<a href='/topic/show?id=b9578094833' target=_blank style='color:#2F92EE;'>#肌病#</a>, beContent=null, objectType=article, channel=null, level=null, likeNumber=70, replyNumber=0, topicName=null, topicId=null, topicList=[TopicDto(id=80948, encryptionId=b9578094833, topicName=肌病)], attachment=null, authenticateStatus=null, createdAvatar=, createdBy=395f25, createdName=爆笑小医, createdTime=Wed Oct 17 05:43:00 CST 2018, time=2018-10-17, status=1, ipAttribution=)]
    2019-03-20 cnxcy

    #致病性#

    0

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    2018-10-23 cmj8wellington

    #Dis#

    0

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    2018-10-17 millore

    #坏死#

    0

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    2018-10-17 ms5768352621950538

    #IgG#

    0

  6. [GetPortalCommentsPageByObjectIdResponse(id=1948104, encodeId=4f6a1948104d4, content=<a href='/topic/show?id=3aae55212db' target=_blank style='color:#2F92EE;'>#抗体阳性#</a>, beContent=null, objectType=article, channel=null, level=null, likeNumber=75, replyNumber=0, topicName=null, topicId=null, topicList=[TopicDto(id=55212, encryptionId=3aae55212db, topicName=抗体阳性)], attachment=null, authenticateStatus=null, createdAvatar=, createdBy=a12645, createdName=智慧医人, createdTime=Tue Jan 29 12:43:00 CST 2019, time=2019-01-29, status=1, ipAttribution=), GetPortalCommentsPageByObjectIdResponse(id=1718842, encodeId=95221e18842b2, content=<a href='/topic/show?id=d3e9864e750' target=_blank style='color:#2F92EE;'>#致病性#</a>, beContent=null, objectType=article, channel=null, level=null, likeNumber=103, replyNumber=0, topicName=null, topicId=null, topicList=[TopicDto(id=86477, encryptionId=d3e9864e750, topicName=致病性)], attachment=null, authenticateStatus=null, createdAvatar=null, createdBy=d36132650190, createdName=cnxcy, createdTime=Wed Mar 20 06:43:00 CST 2019, time=2019-03-20, status=1, ipAttribution=), GetPortalCommentsPageByObjectIdResponse(id=2012836, encodeId=67b8201283641, content=<a href='/topic/show?id=7d69598468' target=_blank style='color:#2F92EE;'>#Dis#</a>, beContent=null, objectType=article, channel=null, level=null, likeNumber=72, replyNumber=0, topicName=null, topicId=null, topicList=[TopicDto(id=5984, encryptionId=7d69598468, topicName=Dis)], attachment=null, authenticateStatus=null, createdAvatar=, createdBy=c689267, createdName=cmj8wellington, createdTime=Tue Oct 23 19:43:00 CST 2018, time=2018-10-23, status=1, ipAttribution=), GetPortalCommentsPageByObjectIdResponse(id=1362915, encodeId=407913629157a, content=<a href='/topic/show?id=b723414321c' target=_blank style='color:#2F92EE;'>#坏死#</a>, beContent=null, objectType=article, channel=null, level=null, likeNumber=75, replyNumber=0, topicName=null, topicId=null, topicList=[TopicDto(id=41432, encryptionId=b723414321c, topicName=坏死)], attachment=null, authenticateStatus=null, createdAvatar=, createdBy=c0e3195, createdName=millore, createdTime=Wed Oct 17 05:43:00 CST 2018, time=2018-10-17, status=1, ipAttribution=), GetPortalCommentsPageByObjectIdResponse(id=1615190, encodeId=87511615190b6, content=<a href='/topic/show?id=010c9506a3' target=_blank style='color:#2F92EE;'>#IgG#</a>, beContent=null, objectType=article, channel=null, level=null, likeNumber=82, replyNumber=0, topicName=null, topicId=null, topicList=[TopicDto(id=9506, encryptionId=010c9506a3, topicName=IgG)], attachment=null, authenticateStatus=null, createdAvatar=null, createdBy=6c4f19588882, createdName=ms5768352621950538, createdTime=Wed Oct 17 05:43:00 CST 2018, time=2018-10-17, status=1, ipAttribution=), GetPortalCommentsPageByObjectIdResponse(id=1622351, encodeId=5a771622351a7, content=<a href='/topic/show?id=b9578094833' target=_blank style='color:#2F92EE;'>#肌病#</a>, beContent=null, objectType=article, channel=null, level=null, likeNumber=70, replyNumber=0, topicName=null, topicId=null, topicList=[TopicDto(id=80948, encryptionId=b9578094833, topicName=肌病)], attachment=null, authenticateStatus=null, createdAvatar=, createdBy=395f25, createdName=爆笑小医, createdTime=Wed Oct 17 05:43:00 CST 2018, time=2018-10-17, status=1, ipAttribution=)]
    2018-10-17 爆笑小医

    #肌病#

    0

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