焉传祝教授：特发性炎症性肌病的诊治进展与挑战：NCN2019

2019-09-23 hosttest MedSci原创

特发性炎症性肌病（IIM）是指非感染性的获得性炎症性肌病，是一个多器官受累的疾病，累及的肌肉组织包括肌肉和肌筋膜，非肌肉组织包括皮肤、肺、关节和血管等。9月20日，在中华医学会第二十二次全国神经病学学术会议上，本届大会主席、山东大学齐鲁医院神经内科主任焉传祝教授针对特发性炎症性肌病的诊治进展与挑战做了专题报告，在此小编整理报告内容与大家分享。IIM的诊断和分类诊断标准IIM的诊断要素条件肌肉受累证

特发性炎症性肌病（IIM）是指非感染性的获得性炎症性肌病，是一个多器官受累的疾病，累及的肌肉组织包括肌肉和肌筋膜，非肌肉组织包括皮肤、肺、关节和血管等。

9月20日，在中华医学会第二十二次全国神经病学学术会议上，本届大会主席、山东大学齐鲁医院神经内科主任焉传祝教授针对特发性炎症性肌病的诊治进展与挑战做了专题报告，在此小编整理报告内容与大家分享。

IIM的诊断和分类诊断标准

IIM的诊断要素条件

肌肉受累证据

自身免疫证据

-肌肉病理：炎细胞侵润、MHC-1、MAC、MxA

-血清自身抗体：肌炎特异性抗体（MSA）、肌炎相关抗体（MAA）、风湿系列以及其他自身抗体

IIM的分型及诊断

IIM的亚型诊断

IIM的亚型包括多发性肌炎、皮肌炎、重叠综合症（Overlap Syndrome）、青少年皮肌炎、伴有肿瘤的肌炎、无肌病皮肌炎（Amyopathic DM）、无皮损皮肌炎（DM sine dermatitis）、抗合成酶抗体综合症（ASS）、免疫介导的坏死性肌病（IMNM/NAM）、非特异性肌炎（NSM）

-PM的最新标准

-皮肌炎

-免疫介导的坏死性肌病（IMNM/NAM）

-SRP阳性的IMNM

-HMGCR阳性的IMNM

-抗体阴性的IMNM

IMNM诊断标准目前尚未形成广泛共识。

-非特异性肌炎（NSM）

-坏死性肌病

-肌束膜或血管周围炎性细胞侵润

-没有单核细胞侵入非坏死纤维现象

-SRP, HMGCR抗体阴性

-传统的“多发性肌炎”

并没有被普遍接受的肌炎类型

-抗磷脂抗体综合症（ASS）

抗合成酶抗体综合症（ASS）：Jo-1、EJ、OJ、PL-7、PL-12、Zo、Ha、KS

常伴有显著的其他组织受累症状：关节炎、间质性肺病、雷诺氏现象、“机械师手”

-包涵体肌炎

50岁以后起病、手指屈肌无力、股四头肌萎缩、镶边空泡、肌内膜炎细胞侵润

IIM的治疗

小结

-IIM诊断的关键是在病理和血清学水平确立自身免疫的证据

-在缺少自身免疫证据的情况下，IIM仍然是一个排除性诊断

-IIM分型诊断目前仍存在很大分歧，血清抗体可能成为IIM重要的分型诊断依据

-IIM作为可治疗的肌病，早期诊断早期治疗至关重要。规范的糖皮质效素治疗可让绝大多数IIM获益。

-少数难治的IIM可尝试利妥昔单抗

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2020-05-27 12498cbcm11(暂无昵称)

#诊治进展#

30 0
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2019-10-30 ms7421025882867189

#NCN#

32 0
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2020-08-08 bbjsj_1990

#特发性炎症性肌病#

29 0
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2019-10-30 xinxin088

#特发性#

19 0
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2019-09-25 12498dd9m15暂无昵称

#炎症性#

23 0
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2019-09-25 爆笑小医

#肌病#

23 0

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焉传祝教授：特发性炎症性肌病的诊治进展与挑战：NCN2019

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