ARD:2021年EULAR/ACR:要考虑诊断、管理和监测白细胞介素1介导的自身炎症性疾病

2022-07-10 MedSci原创 MedSci原创

为冷卟啉相关周期性综合征(CAPS)、肿瘤坏死因子受体相关周期性综合征(TRAPS)、甲羟戊酸激酶缺乏症(MKD)和和IL-1受体拮抗剂缺乏症(DIRA)患者的诊断、治疗和监测建立循证建议。

背景白细胞介素-1IL-1)介导的全身性自身炎症性疾病,包括冷卟啉相关周期性综合征(CAPS)、肿瘤坏死因子受体相关周期性综合征(TRAPS)、甲羟戊酸激酶缺乏症(MKD)和IL-1受体拮抗剂缺乏症(DIRA),属于一组罕见的免疫失调疾病,主要出现在儿童早期,多器官受累。如果未经治疗,具有严重临床表型的患者预后较差,这些患者的诊断和管理可能具有挑战性。然而,已批准的针对促炎细胞因子IL-1的治疗方法已经改变了生活,并显著改善了患者的预后。

目的IL-1介导的自身炎症性疾病患者的诊断、治疗和监测建立循证建议,以规范其管理。

方法成立了一个由医生专家组成的跨国、多学科工作组,包括风湿病学家、患者或护理人员以及相关的医疗保健专业人员。进行了证据综合,包括通过调查进行的系统文献回顾和专家共识(Delphi)。共识方法用于制定声明并对其进行投票,以指导最佳患者护理。

结果该工作组设计了五项总体原则,14项与诊断相关的声明,10项关于治疗的声明,9项侧重于长期监测,这些都是基于证据和/或基于共识的IL-1介导疾病患者的监测。制定了一个大纲,用于对炎症引起的器官损伤进展进行疾病特异性监测,并报告了CAPSTRAPSMKDDIRA的治疗方法。

结论:2021EULAR/美国风湿病学会要点代表基于已发表数据和专家意见的最先进知识,以指导CAPSTRAPSMKDDIRA患者的诊断评估、治疗和监测,并标准化和改善护理、生活质量和疾病结果。

出处:Romano M, Arici ZS, Piskin D, et al. The 2021 EULAR/American College of Rheumatology points to consider for diagnosis, management and monitoring of the interleukin-1 mediated autoinflammatory diseases: cryopyrin-associated periodic syndromes, tumour necrosis factor receptor-associated periodic syndrome, mevalonate kinase deficiency, and deficiency of the interleukin-1 receptor antagonist. Annals of the Rheumatic Diseases 2022;81:907-921.

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[GetPortalCommentsPageByObjectIdResponse(id=2055159, encodeId=f08620551590d, content=<a href='/topic/show?id=c08c8636548' target=_blank style='color:#2F92EE;'>#自身炎症性疾病#</a>, beContent=null, objectType=article, channel=null, level=null, likeNumber=45, replyNumber=0, topicName=null, topicId=null, topicList=[TopicDto(id=86365, encryptionId=c08c8636548, topicName=自身炎症性疾病)], attachment=null, authenticateStatus=null, createdAvatar=, createdBy=b3ad2500191, createdName=12498dd9m11暂无昵称, createdTime=Thu Jan 19 04:57:10 CST 2023, time=2023-01-19, status=1, ipAttribution=), GetPortalCommentsPageByObjectIdResponse(id=1736835, encodeId=a62f1e36835a2, content=<a href='/topic/show?id=56bf6e024d4' target=_blank style='color:#2F92EE;'>#炎症性疾病#</a>, beContent=null, objectType=article, channel=null, level=null, likeNumber=58, replyNumber=0, topicName=null, topicId=null, topicList=[TopicDto(id=67024, encryptionId=56bf6e024d4, topicName=炎症性疾病)], attachment=null, authenticateStatus=null, createdAvatar=null, 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createdTime=Mon Jul 11 22:57:10 CST 2022, time=2022-07-11, status=1, ipAttribution=), GetPortalCommentsPageByObjectIdResponse(id=1620625, encodeId=f8471620625eb, content=<a href='/topic/show?id=25a4e1714f0' target=_blank style='color:#2F92EE;'>#白细胞介素#</a>, beContent=null, objectType=article, channel=null, level=null, likeNumber=53, replyNumber=0, topicName=null, topicId=null, topicList=[TopicDto(id=71714, encryptionId=25a4e1714f0, topicName=白细胞介素)], attachment=null, authenticateStatus=null, createdAvatar=null, createdBy=e1dc20143475, createdName=yxch43, createdTime=Mon Jul 11 22:57:10 CST 2022, time=2022-07-11, status=1, ipAttribution=), GetPortalCommentsPageByObjectIdResponse(id=1231618, encodeId=8c341231618ac, content=<a href='/topic/show?id=8f275032126' target=_blank style='color:#2F92EE;'>#期刊论坛#</a><a href='/topic/show?id=23e3454e353' target=_blank style='color:#2F92EE;'>#学习#</a>, beContent=null, objectType=article, channel=null, level=null, likeNumber=49, replyNumber=0, 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  5. 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createdTime=Mon Jul 11 22:57:10 CST 2022, time=2022-07-11, status=1, ipAttribution=), GetPortalCommentsPageByObjectIdResponse(id=1620625, encodeId=f8471620625eb, content=<a href='/topic/show?id=25a4e1714f0' target=_blank style='color:#2F92EE;'>#白细胞介素#</a>, beContent=null, objectType=article, channel=null, level=null, likeNumber=53, replyNumber=0, topicName=null, topicId=null, topicList=[TopicDto(id=71714, encryptionId=25a4e1714f0, topicName=白细胞介素)], attachment=null, authenticateStatus=null, createdAvatar=null, createdBy=e1dc20143475, createdName=yxch43, createdTime=Mon Jul 11 22:57:10 CST 2022, time=2022-07-11, status=1, ipAttribution=), GetPortalCommentsPageByObjectIdResponse(id=1231618, encodeId=8c341231618ac, content=<a href='/topic/show?id=8f275032126' target=_blank style='color:#2F92EE;'>#期刊论坛#</a><a href='/topic/show?id=23e3454e353' target=_blank style='color:#2F92EE;'>#学习#</a>, beContent=null, objectType=article, channel=null, level=null, likeNumber=49, replyNumber=0, topicName=null, topicId=null, topicList=[TopicDto(id=50321, encryptionId=8f275032126, topicName=期刊论坛), TopicDto(id=45473, encryptionId=23e3454e353, topicName=学习)], attachment=null, authenticateStatus=null, createdAvatar=null, createdBy=b0825417521, createdName=ms6000000960504356, createdTime=Sun Jul 10 11:25:08 CST 2022, time=2022-07-10, status=1, ipAttribution=), GetPortalCommentsPageByObjectIdResponse(id=1231617, encodeId=b65d123161ef6, content=不错的文章, beContent=null, objectType=article, channel=null, level=null, likeNumber=47, replyNumber=0, topicName=null, topicId=null, topicList=[], attachment=null, authenticateStatus=null, createdAvatar=null, createdBy=b0825417521, createdName=ms6000000960504356, createdTime=Sun Jul 10 11:25:01 CST 2022, time=2022-07-10, status=1, ipAttribution=)]
    2022-07-11 10518094zz
  6. 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topicName=null, topicId=null, topicList=[TopicDto(id=50321, encryptionId=8f275032126, topicName=期刊论坛), TopicDto(id=45473, encryptionId=23e3454e353, topicName=学习)], attachment=null, authenticateStatus=null, createdAvatar=null, createdBy=b0825417521, createdName=ms6000000960504356, createdTime=Sun Jul 10 11:25:08 CST 2022, time=2022-07-10, status=1, ipAttribution=), GetPortalCommentsPageByObjectIdResponse(id=1231617, encodeId=b65d123161ef6, content=不错的文章, beContent=null, objectType=article, channel=null, level=null, likeNumber=47, replyNumber=0, topicName=null, topicId=null, topicList=[], attachment=null, authenticateStatus=null, createdAvatar=null, createdBy=b0825417521, createdName=ms6000000960504356, createdTime=Sun Jul 10 11:25:01 CST 2022, time=2022-07-10, status=1, ipAttribution=)]
    2022-07-11 muzishouyi
  7. 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  8. 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  9. 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  10. 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