国际IgG4相关疾病管理与治疗的共识出台

2016-09-06 康进陈进伟中华风湿病学杂志

IgG4相关疾病(IgG4-RD)是一种免疫介导的纤维炎症反应，它可以破坏组织正常结构，累及多个器官，导致器官肿胀，甚至衰竭。 在第二届IgG4-RD国际会议筹备过程中，42位来自不同国家和地区的专家经过系统的调查及讨论，最终达成了IgG4-RD管理与治疗的7条国际共识(见表1)，并发表在2015年7月的Arthritis&Rheumatology。 这是首次公开发表的

IgG4相关疾病(IgG4-RD)是一种免疫介导的纤维炎症反应，它可以破坏组织正常结构，累及多个器官，导致器官肿胀，甚至衰竭。

在第二届IgG4-RD国际会议筹备过程中，42位来自不同国家和地区的专家经过系统的调查及讨论，最终达成了IgG4-RD管理与治疗的7条国际共识(见表1)，并发表在2015年7月的Arthritis&Rheumatology。

这是首次公开发表的IgG4-RD治疗策略的专家共识，旨在IgG4-RD的研究初期，对IgG4-RD相关专家的经验进行总结归纳，以期为医师进行临床决策提供参考。

《共识》中强调血清Igc4浓度测量仍然是诊断IgG4-RD的重要方法，但必须结合临床特征及病理结果，严格排除临床表现及组织学特征与IgG4-RD相仿的疾病：ANCA相关血管炎的肉芽肿性血管炎；显微镜下多血管炎，嗜酸性肉芽肿血管炎，腺癌和鳞状细胞癌，瘤周浸润，Castleman病(多发性或局限性)；皮肤浆细胞增多症；Erdheimchester病，炎性肌纤维母细胞瘤，炎性肠病，淋巴组织增生性疾病，结外边缘区淋巴瘤，淋巴浆细胞性淋巴瘤；滤泡性淋巴瘤，穿通性胶原病，原发性硬化性胆管炎，鼻窦炎，Rosai．Dorfman病，结节病，SS，脾脏硬化性血管瘤样结节性转化，黄色肉芽肿。组织活检及IgG4+浆细胞免疫组织化学有一定诊断意义，轮辐状纤维化及闭塞的静脉炎可提高诊断特异性。

《共识》同意糖皮质激素仍为]gG4-RD活动期诱导缓解的一线治疗药物，但在减停过程中疾病易复发。很多日本临床医生推荐低剂量糖皮质激素维持治疗3年。大部分专家认为免疫抑制剂可作为疾病活动度很高时诱导缓解的药物、也可作为激素减量过程中的助减剂及无法减量时的维持缓解的联合治疗药物。利妥昔单抗能去除B细胞，靶向性抑制IgG4的表达，有效缓解病情，也可维持治疗。当疾病进展在某些脏器会产生不可逆的损害时需要紧急治疗。

需进行紧急治疗的IgG4-RD临床表现：①主动脉炎：炎症性腹主动脉瘤可进行性增大，有破裂的风险；②腹膜后纤维化：疾病进行性发展可导致不可逆的神经损害/疼痛、尿路梗阻、肾衰竭；③近端胆道狭窄：不治疗可导致胆囊炎的重叠感染，最终产生不可逆的纤维化或肝硬化；④肾小管间质肾炎：不治疗可导致不可逆的慢性肾脏疾病；⑤硬脑膜炎：不治疗可导致神经功能缺损及癫痫；⑥胰腺肿大：不治疗可导致不可逆的胰腺内外分泌失调；⑦心包炎：不治疗可导致心包填塞或缩窄性心包炎。

该共识的实用性、有效性和局限性有待于进一步验证和评估。不同国家和地区对疾病诱导缓解和维持治疗的方法也会受到地方医疗政策的限制。该共识可为我国临床医生诊疗决策提供参考；也提醒我国医疗工作者，应充分利用我国病源优势，总结制定出适合我国国情的IgG4-RD诊疗指南。

中华风湿病学杂志2016年6月20卷6期

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2016-12-09 licz0423

#疾病管理#

88 0
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2016-10-11 1e10c84am36(暂无匿称)

好文章，受益

2 0
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replyNumber=0, topicName=null, topicId=null, topicList=[], attachment=null, authenticateStatus=null, createdAvatar=https://wx.qlogo.cn/mmopen/jW482SpianMayicTRbRZ5RzfPkmqRTH5rHib5mcib8Wb3QdXjhfOK1UubQL2lictjJFdFJ6Lc3cnnCOSWqxXZ4Kk6y5GdzDoy764R/0, createdBy=156e1644785, createdName=doctorJiangchao, createdTime=Wed Sep 14 11:01:00 CST 2016, time=2016-09-14, status=1, ipAttribution=), GetPortalCommentsPageByObjectIdResponse(id=1482962, encodeId=a0ce148296276, content=<a href='/topic/show?id=5d52950839' target=_blank style='color:#2F92EE;'>#IgG4#</a>, beContent=null, objectType=article, channel=null, level=null, likeNumber=106, replyNumber=0, topicName=null, topicId=null, topicList=[TopicDto(id=9508, encryptionId=5d52950839, topicName=IgG4)], attachment=null, authenticateStatus=null, createdAvatar=, createdBy=bcbb7863836, createdName=ms5106640509429053, createdTime=Thu Sep 08 01:18:00 CST 2016, time=2016-09-08, status=1, ipAttribution=), GetPortalCommentsPageByObjectIdResponse(id=1598942, 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createdTime=Thu Sep 08 01:18:00 CST 2016, time=2016-09-08, status=1, ipAttribution=), GetPortalCommentsPageByObjectIdResponse(id=112216, encodeId=3e8b11221684, content=继续关注, beContent=null, objectType=article, channel=null, level=null, likeNumber=67, replyNumber=0, topicName=null, topicId=null, topicList=[], attachment=null, authenticateStatus=null, createdAvatar=https://wx.qlogo.cn/mmopen/jW482SpianMayicTRbRZ5RzfPkmqRTH5rHib5mcib8Wb3QdXjhfOK1UubQL2lictjJFdFJ6Lc3cnnCOSWqxXZ4Kk6y5GdzDoy764R/0, createdBy=156e1644785, createdName=doctorJiangchao, createdTime=Tue Sep 06 13:44:00 CST 2016, time=2016-09-06, status=1, ipAttribution=)]
2016-09-15 doctorJiangchao

继续学习

104 0
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2016-09-15 doctorJiangchao

继续关注

96 0
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2016-09-14 doctorJiangchao

继续学习

116 0
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createdAvatar=https://wx.qlogo.cn/mmopen/jW482SpianMayicTRbRZ5RzfPkmqRTH5rHib5mcib8Wb3QdXjhfOK1UubQL2lictjJFdFJ6Lc3cnnCOSWqxXZ4Kk6y5GdzDoy764R/0, createdBy=156e1644785, createdName=doctorJiangchao, createdTime=Thu Sep 15 16:12:00 CST 2016, time=2016-09-15, status=1, ipAttribution=), GetPortalCommentsPageByObjectIdResponse(id=126428, encodeId=9410126428c9, content=继续学习, beContent=null, objectType=article, channel=null, level=null, likeNumber=116, replyNumber=0, topicName=null, topicId=null, topicList=[], attachment=null, authenticateStatus=null, createdAvatar=https://wx.qlogo.cn/mmopen/jW482SpianMayicTRbRZ5RzfPkmqRTH5rHib5mcib8Wb3QdXjhfOK1UubQL2lictjJFdFJ6Lc3cnnCOSWqxXZ4Kk6y5GdzDoy764R/0, createdBy=156e1644785, createdName=doctorJiangchao, createdTime=Wed Sep 14 11:01:00 CST 2016, time=2016-09-14, status=1, ipAttribution=), GetPortalCommentsPageByObjectIdResponse(id=126430, encodeId=d092126430f7, content=继续关注, beContent=null, objectType=article, channel=null, level=null, likeNumber=86, 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2016-09-14 doctorJiangchao

继续关注

86 0
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2016-09-08 ms5106640509429053

#IgG4#

106 0
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2016-09-08 jiekemin

#IgG4相关疾病#

75 0
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createdTime=Thu Sep 08 01:18:00 CST 2016, time=2016-09-08, status=1, ipAttribution=), GetPortalCommentsPageByObjectIdResponse(id=112216, encodeId=3e8b11221684, content=继续关注, beContent=null, objectType=article, channel=null, level=null, likeNumber=67, replyNumber=0, topicName=null, topicId=null, topicList=[], attachment=null, authenticateStatus=null, createdAvatar=https://wx.qlogo.cn/mmopen/jW482SpianMayicTRbRZ5RzfPkmqRTH5rHib5mcib8Wb3QdXjhfOK1UubQL2lictjJFdFJ6Lc3cnnCOSWqxXZ4Kk6y5GdzDoy764R/0, createdBy=156e1644785, createdName=doctorJiangchao, createdTime=Tue Sep 06 13:44:00 CST 2016, time=2016-09-06, status=1, ipAttribution=)]
2016-09-08 ms5768352621950538

#IgG#

120 0
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createdName=1e10c84am36(暂无匿称), createdTime=Tue Oct 11 22:28:46 CST 2016, time=2016-10-11, status=1, ipAttribution=), GetPortalCommentsPageByObjectIdResponse(id=127887, encodeId=55fa12e88750, content=继续学习, beContent=null, objectType=article, channel=null, level=null, likeNumber=104, replyNumber=0, topicName=null, topicId=null, topicList=[], attachment=null, authenticateStatus=null, createdAvatar=https://wx.qlogo.cn/mmopen/jW482SpianMayicTRbRZ5RzfPkmqRTH5rHib5mcib8Wb3QdXjhfOK1UubQL2lictjJFdFJ6Lc3cnnCOSWqxXZ4Kk6y5GdzDoy764R/0, createdBy=156e1644785, createdName=doctorJiangchao, createdTime=Thu Sep 15 16:12:00 CST 2016, time=2016-09-15, status=1, ipAttribution=), GetPortalCommentsPageByObjectIdResponse(id=127888, encodeId=ab7112e888f8, content=继续关注, beContent=null, objectType=article, channel=null, level=null, likeNumber=96, replyNumber=0, topicName=null, topicId=null, topicList=[], attachment=null, authenticateStatus=null, 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2016-09-06 doctorJiangchao

继续关注

67 0

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