ERS/EULAR clinical practice guidelines for connective tissue disease-associated interstitial lung diseaseDeveloped by the task force for connective tissue disease-associated interstitial lung disease of the European Respiratory Society (ERS) and the European Alliance of Associations for Rheumatology (EULAR)Endorsed by the European Reference Network on rare respiratory diseases (ERN-LUNG).

IF 21 1区 医学 Q1 RESPIRATORY SYSTEM
Katerina M Antoniou,Oliver Distler,Ana-Maria Gheorghiu,Catharina C Moor,Jens Vikse,Nikoleta Bizymi,Ilaria Galetti,Graham Brown,Elena Bargagli,Yannick Allanore,Tamera J Corte,Philippe Dieudé,Vincent Cottin,Benjamin A Fisher,Aurelie Fabre,Jon T Giles,Michael Kreuter,Ingrid E Lundberg,Venerino Poletti,Britta Maurer,Elisabetta A Renzoni,Ulf Müller-Ladner,Mary E Strek,Nicola Sverzellati,Paul Studenic,Jibril Mohammed,Blin Nagavci,Tanja Stamm,Thomy Tonia,Bruno Crestani,Anna-Maria Hoffmann-Vold
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Abstract

BACKGROUND Interstitial lung disease (ILD) is a frequent manifestation of connective tissue diseases (CTDs) and is associated with high morbidity and mortality. Clinical practice guidelines to standardise screening, diagnosis, treatment and follow-up for CTD-ILD are of high importance for optimised patient care. METHODS A European Respiratory Society and European Alliance of Associations for Rheumatology task force committee, composed of pulmonologists, rheumatologists, pathologists, radiologists, methodologists and patient representatives, developed recommendations based on PICO (Patients, Intervention, Comparison, Outcomes) questions with grading of the evidence according to the GRADE (Grading of Recommendations, Assessment, Development and Evaluations) methodology and complementary narrative questions agreed on by both societies. For both PICO and narrative questions, the Evidence to Decision framework was used to formulate the recommendations. RESULTS The task force committee concluded with recommendations for 25 PICO and 28 narrative questions, regarding ILD in the context of systemic sclerosis, rheumatoid arthritis (RA), idiopathic inflammatory myopathies, Sjögren disease (SjD), systemic lupus erythematosus (SLE) and mixed connective tissue disease (MCTD). In four narrative questions, regarding screening and assessment of risk for ILD progression in MCTD, SjD and SLE and one PICO question regarding pirfenidone in CTD-ILD other than RA-ILD, the task force had insufficient evidence to support recommendations. Screening, diagnostic, monitoring and treatment algorithms were developed based on the recommendations and usual clinical practice. CONCLUSIONS We provide practical guidance by evidence-based recommendations to clinicians for each of the CTDs. In many cases there is low certainty or absence of evidence and we encourage further research to fill these gaps.
结缔组织病相关间质性肺病的ERS/EULAR临床实践指南由欧洲呼吸学会(ERS)和欧洲风湿病协会联盟(EULAR)的结缔组织病相关间质性肺病工作组制定,并得到欧洲罕见呼吸疾病参考网络(ERN-LUNG)的认可。
背景:间质性肺疾病(ILD)是结缔组织疾病(CTDs)的常见表现,具有较高的发病率和死亡率。规范CTD-ILD的筛查、诊断、治疗和随访的临床实践指南对于优化患者护理具有重要意义。方法欧洲呼吸学会和欧洲风湿病协会联盟工作组委员会,由肺科医生、风湿病学家、病理学家、放射科医生、方法学家和患者代表组成,根据PICO(患者、干预、比较、结果)问题制定建议,并根据GRADE(建议、评估的分级)对证据进行分级。发展和评价)的方法和互补的叙述问题由两个社会商定。对于PICO和叙述性问题,采用了决策证据框架来制定建议。结果工作组委员会总结了关于系统性硬化症、类风湿性关节炎(RA)、特发性炎症性肌病、Sjögren疾病(SjD)、系统性红斑狼疮(SLE)和混合性结缔组织病(MCTD)背景下ILD的25个PICO和28个叙述性问题的建议。在四个叙述性问题中,关于MCTD、SjD和SLE中ILD进展风险的筛查和评估,以及一个PICO问题中关于吡非尼酮在CTD-ILD(非RA-ILD)中的应用,工作组没有足够的证据来支持建议。筛查、诊断、监测和治疗算法是根据建议和通常的临床实践制定的。结论我们通过循证建议为临床医生提供每一种CTDs的实用指导。在许多情况下,证据的确定性很低或缺乏证据,我们鼓励进一步的研究来填补这些空白。
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来源期刊
European Respiratory Journal
European Respiratory Journal 医学-呼吸系统
CiteScore
27.50
自引率
3.30%
发文量
345
审稿时长
2-4 weeks
期刊介绍: The European Respiratory Journal (ERJ) is the flagship journal of the European Respiratory Society. It has a current impact factor of 24.9. The journal covers various aspects of adult and paediatric respiratory medicine, including cell biology, epidemiology, immunology, oncology, pathophysiology, imaging, occupational medicine, intensive care, sleep medicine, and thoracic surgery. In addition to original research material, the ERJ publishes editorial commentaries, reviews, short research letters, and correspondence to the editor. The articles are published continuously and collected into 12 monthly issues in two volumes per year.
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