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特发性炎性肌病相关间质性肺疾病诊断和治疗中国专家共识
中国研究型医院学会呼吸病学专业委员会
作者及单位信息
·
DOI： 10.3760/cma.j.cn112147-20220108-00025
Chinese expert-based consensus statement on diagnosis and treatment of idiopathic inflammatory myopathy associated interstitial lung disease
Respiratory Council of Chinese Research Hospital Association
Cai Hourong
Xu Zuojun
Authors Info & Affiliations
Respiratory Council of Chinese Research Hospital Association
Cai Hourong
Department of Respiratory and Critical Care Medicine, Nanjing Drum Tower Hospital, Nanjing 210008, China
Xu Zuojun
Department of Respiratory and Critical Care Medicine, Peking Union Medical College Hospital, Chinese Academy of Medical Sciences & Peking Union Medical College, Beijing 100730, China
·
DOI： 10.3760/cma.j.cn112147-20220108-00025
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摘要

特发性炎性肌病（idiopathic inflammatory myopathy，IIM）是以四肢近端肌肉慢性炎症为特征性表现的一组异质性疾病。与IIM相关的间质性肺疾病（IIM-associated interstitial lung disease，IIM-ILD）是IIM患者致残和致死的重要原因之一。IIM-ILD患者的起病方式、临床表现，发展过程、治疗反应和疾病预后呈异质性。由于IIM-ILD诊断与病情评估需要多学科共同参与，免疫抑制治疗药物前瞻性研究资料有限，治疗方案的临床选择差异较大，因此亟需制定IIM-ILD诊断和治疗的共识，供临床医师在临床诊治实践中借鉴。中国研究型医院学会呼吸病学专业委员会组织国内从事IIM-ILD基础与临床研究的呼吸病学、风湿免疫学，放射学等多学科的专家，基于现有文献资料，结合专家经验，共同制定本共识。本共识主要对IIM-ILD流行病学、临床特征、诊断与病情评估、治疗药物和治疗方案等方面做了较为系统阐述。经专家讨论和投票，共形成18条推荐意见，其中7条是关于IIM-ILD诊断和评估的推荐意见，11条是关于IIM-ILD治疗药物和方案选择的推荐意见，以期规范化诊断和治疗我国IIM-ILD患者，推动开展相关的临床和基础研究工作。

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特发性炎性肌病是以四肢近端肌肉慢性炎症为特征性表现的一组异质性疾病。本共识主要对IIM⁃ILD流行病学、临床特征、诊断与病情评估、治疗药物和治疗方案等方面做了较为系统阐述。
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ABSTRACT

Idiopathic inflammatory myopathies (IIM), a group of heterogenous autoimmune diseases, are characterized by proximal, skeletal muscle involvement of chronic immunological pathogenesis. IIM associated interstitial lung diseases (IIM-ILDs) are the most important causes of morbidity or mortality for IIM patients. Patients with IIM-ILDs always exhibit diversity of diseases manifestations including onset pattern, diseases progress, therapeutic respond and prognosis. Hence, the diagnosis and evaluation of IIM-ILDs usually need multidisciplinary consultations. And we have to face some dilemmas, such as sophisticated choice of immunosuppressants due to limited prospective research data, and personnel and different responses to therapeutic remedy. It is an urgent task to develop a consensus on the diagnosis and treatment of IIM-ILDs for guiding the routine practice of clinicians. Experts from pulmonology, rheumatology, and radiology were organized by Respiratory Council of Chinese Research Hospital Association, and developed a consensus based on previous studies and the experts ′ experiences. This consensus mainly illustrated the epidemiology, clinical characteristics, diagnosis and evaluation, therapeutic remedy for IIM-ILDs. After full discussion and voting by the expert group, a total of 18 recommendations were formed, including 7 recommendations on diagnosis and evaluation, and 11 recommendations on the choice of therapeutic strategies. This consensus is intended to facilitate the standardization of diagnosis and treatment of patients with IIM-ILD and promote the development of relevant clinical and basic research in China.

Cai Hourong,Department of Respiratory and Critical Care Medicine, Nanjing Drum Tower Hospital, Nanjing 210008, China, Email: mocdef.3ab613102gnoruohiac
Xu Zuojun,Department of Respiratory and Critical Care Medicine, Peking Union Medical College Hospital, Chinese Academy of Medical Sciences & Peking Union Medical College, Beijing 100730, China, Email: mocdef.labiamtohjzux
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引用本文

中国研究型医院学会呼吸病学专业委员会. 特发性炎性肌病相关间质性肺疾病诊断和治疗中国专家共识[J]. 中华结核和呼吸杂志,2022,45(07)：635-650.

DOI:10.3760/cma.j.cn112147-20220108-00025

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特发性炎性肌病（idiopathic inflammatory myopathy，IIM），简称肌炎（myositis），是一组以四肢近端肌肉受累和慢性炎症为突出表现的异质性疾病。目前IIM分为：皮肌炎（dermatomyositis，DM）、抗合成酶抗体综合征（antisynthetase syndrome，ASS）、包涵体肌炎（inclusion body myositis，IBM）、免疫介导坏死性肌炎（Immune-mediated necrotizing myopathy，IMNM）和多发性肌炎（polymyositis，PM） ［ 1 , 2 , 3 , 4 , 5 ］ 。其中DM和ASS更为常见 ［ 4 , 5 ］ ，以往研究中的PM包括了现在分类中的ASS和IMNM。IIM可累及皮肤、肺脏、关节和心脏等多个系统及器官，而间质性肺疾病（interstitial lung disease，ILD）是IIM累及肺脏的最常见临床表现。特发性炎性肌病相关间质性肺疾病（idiopathic inflammatory myopathy-associated interstitial lung disease，IIM-ILD）是IIM患者致残和致死的重要原因 ［ 6 , 7 , 8 ］ 。
近年来随着肌炎抗体谱的使用和对IIM-ILD认识的提高，在ILD的临床实践中观察到IIM-ILD患者明显增多，但迄今国内外尚无IIM-ILD临床诊断及治疗相关的指南或共识，亟需由IIM-ILD诊疗相关的专家制定IIM-ILD的诊治共识，以规范IIM-ILD的诊断和治疗，改善患者预后，提高本领域的临床和科研水平。为此，中国研究型医院学会呼吸病学专业委员会组织国内呼吸科、风湿免疫科、放射科等多学科的专家，基于国际及我国现有临床证据和专家的诊治经验制定了本共识；同时，本共识提出了IIM-ILD临床诊治和研究中若干尚不明确、有待解决的问题，期望通过建立我国IIM-ILD多学科、多中心合作，开展相关临床和基础研究，积累研究证据，为今后制定诊治指南奠定基础。
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备注信息
A
蔡后荣，南京鼓楼医院呼吸与危重症医学科，南京210008，Email： mocdef.3ab613102gnoruohiac
B
徐作军，中国医学科学院北京协和医院呼吸与危重症医学科，北京100730，Email： mocdef.labiamtohjzux
C

中国研究型医院学会呼吸病学专业委员会. 特发性炎性肌病相关间质性肺疾病诊断和治疗中国专家共识[J]. 中华结核和呼吸杂志, 2022, 45(7): 635-650. DOI: 10.3760/cma.j.cn112147-20220108-00025.

D
所有作者声明无利益冲突
E
国家重点研发计划精准医学研究重点专项 (2016YFC0905700)
江苏省科技厅社会发展重点项目 (BE2016611)
中国医学科学院中央级公益性科研院所基本科研业务费临床与转化医学研究基金 (2019XK320037)
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