Abstract：
BACKGROUND: Since the latest 2017 French guidelines, knowledge about idiopathic pulmonary fibrosis has evolved considerably.
METHODS: Practical guidelines were drafted on the initiative of the Coordinating Reference Center for Rare Pulmonary Diseases, led by the French Language Pulmonology Society (SPLF), by a coordinating group, a writing group, and a review group, with the involvement of the entire OrphaLung network, pulmonologists practicing in various settings, radiologists, pathologists, a general practitioner, a health manager, and a patient association. The method followed the \"Clinical Practice Guidelines\" process of the French National Authority for Health (HAS), including an online vote using a Likert scale.
RESULTS: After a literature review, 54 guidelines were formulated, improved, and then validated by the working groups. These guidelines addressed multiple aspects of the disease: epidemiology, diagnostic procedures, quality criteria and interpretation of chest CT scans, lung biopsy indication and procedures, etiological workup, methods and indications for family screening and genetic testing, assessment of the functional impairment and prognosis, indication and use of antifibrotic agents, lung transplantation, management of symptoms, comorbidities and complications, treatment of chronic respiratory failure, diagnosis and management of acute exacerbations of fibrosis.
CONCLUSIONS: These evidence-based guidelines are intended to guide the diagnosis and practical management of idiopathic pulmonary fibrosis.
METHODS: Practical guidelines were drafted on the initiative of the Coordinating Reference Center for Rare Pulmonary Diseases, led by the French Language Pulmonology Society (SPLF), by a coordinating group, a writing group, and a review group, with the involvement of the entire OrphaLung network, pulmonologists practicing in various settings, radiologists, pathologists, a general practitioner, a health manager, and a patient association. The method followed the \"Clinical Practice Guidelines\" process of the French National Authority for Health (HAS), including an online vote using a Likert scale.
RESULTS: After a literature review, 54 guidelines were formulated, improved, and then validated by the working groups. These guidelines addressed multiple aspects of the disease: epidemiology, diagnostic procedures, quality criteria and interpretation of chest CT scans, lung biopsy indication and procedures, etiological workup, methods and indications for family screening and genetic testing, assessment of the functional impairment and prognosis, indication and use of antifibrotic agents, lung transplantation, management of symptoms, comorbidities and complications, treatment of chronic respiratory failure, diagnosis and management of acute exacerbations of fibrosis.
CONCLUSIONS: These evidence-based guidelines are intended to guide the diagnosis and practical management of idiopathic pulmonary fibrosis.
摘要：
背景：自最新的2017年法国指南以来，关于特发性肺纤维化的知识已经有了很大的发展。
方法：实践指南是在罕见肺部疾病协调参考中心的倡议下起草的，由法国语言肺病学会（SPLF）领导，由一个协调小组，一个写作小组，和一个审查小组，在整个OrphaLung网络的参与下，肺科医师在各种环境中练习，放射科医生,病理学家,全科医生,一个健康经理,和患者协会。该方法遵循法国国家卫生管理局（HAS）的“临床实践指南”流程，包括使用李克特量表的在线投票。
结果：经过文献综述，制定了54条准则，改进,然后由工作组验证。这些指南解决了该疾病的多个方面：流行病学，诊断程序,胸部CT扫描的质量标准和解释，肺活检指征和程序，病因学检查，家庭筛查和基因检测的方法和适应症，评估功能障碍和预后，抗纤维化药物的适应症和使用，肺移植,症状的管理，合并症和并发症，慢性呼吸衰竭的治疗，纤维化急性加重的诊断和治疗。
结论：这些循证指南旨在指导特发性肺纤维化的诊断和实际治疗。
方法：实践指南是在罕见肺部疾病协调参考中心的倡议下起草的，由法国语言肺病学会（SPLF）领导，由一个协调小组，一个写作小组，和一个审查小组，在整个OrphaLung网络的参与下，肺科医师在各种环境中练习，放射科医生,病理学家,全科医生,一个健康经理,和患者协会。该方法遵循法国国家卫生管理局（HAS）的“临床实践指南”流程，包括使用李克特量表的在线投票。
结果：经过文献综述，制定了54条准则，改进,然后由工作组验证。这些指南解决了该疾病的多个方面：流行病学，诊断程序,胸部CT扫描的质量标准和解释，肺活检指征和程序，病因学检查，家庭筛查和基因检测的方法和适应症，评估功能障碍和预后，抗纤维化药物的适应症和使用，肺移植,症状的管理，合并症和并发症，慢性呼吸衰竭的治疗，纤维化急性加重的诊断和治疗。
结论：这些循证指南旨在指导特发性肺纤维化的诊断和实际治疗。
