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

Chinese expert-based consensus statement on diagnosis and treatment of idiopathic inflammatory myopathy associated interstitial lung disease

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。

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.