进展性肺纤维化表现的结缔组织病相关间质性肺疾病患者临床管理(四川省)专家共识

Expert consensus on clinical management of CTD-ILD patients with PPF presentation(Sichuan Province)Multidisciplinary

结缔组织病相关间质性肺疾病(connective tissue diseases associated with interstitial lung disease,CTD-ILD)很容易出现进展性肺纤维化(progressive pulmonary fibrosis,PPF)表现而预后不良。然而,如何管理PPF表现的CTD-ILD患者的临床认识仍然非常有限;如何规范化治疗这部分患者也是经常面临的临床问题。为此,四川省医疗卫生与健康促进会呼吸与危重症医学专业委员会间质性肺疾病学组邀请了省内ILD相关领域专家组成了共识编写组,根据临床收集问题,组织专家讨论,最终确定纳入了5个方面的内容。相关内容主要包括CTD-ILD出现PPF的高危因素、临床表现与诊断、病情评估、随访管理和治疗等方面。基于国内外指南、临床研究数据等循证证据,经过多次讨论和投票表决,形成9条推荐意见,共同制定了《PPF表现的CTD-ILD患者临床管理(四川省内)专家共识》,旨在提升出现PPF表现的CTD-ILD的临床认识,为临床决策及管理提供依据,提高临床救治水平。

Connective tissue diseases associated with interstitial lung disease(CTD-ILD)is often found to be prone to progressive pulmonary fibrosis(PPF).However,clinical understanding of how to manage PPF in CTD-ILD patients is still limited.How to standardize the treatment of this group of patients is also a common clinical problem that doctors are often faced.Therefore,Interstitial Lung Disease Group,Committee on Respiratory and Critical Care Medicine of Sichuan Medical and Health Care Promotion Institute invited experts in ILD related fields to form a consensus compilation group.Five aspects of content were finally determined according to clinical collection problems and expert discussions.The relevant contents mainly include the high risk factors of PPF in CTD-ILD,clinical manifestations and diagnosis,disease evaluation,follow-up management and treatment.Based on evidence-based evidence such as domestic and foreign guidelines and clinical research data,after many discussions and voting,9 recommendations were formed.The“Expert Consensus on Clinical Management of CTD-ILD patients with PPF Performance in Sichuan Province”was jointly formulated.The aim is to improve the clinical understanding of CTD-ILD with PPF manifestations,provide the basis for clinical decision-making and management,and improve the level of clinical treatment.