Department of Internal Medicine V, Ludwig-Maximilians-University (LMU) of Munich, Comprehensive Pneumology Center, Member of the German Center for Lung Research (Deutsches Zentrum für Lungenforschung, DZL), Munich, Germany,
Section of Fibrotic Lung Diseases, University Hospital Giessen and Marburg, Giessen Campus, Justus Liebig University Giessen, Agaplesion Pneumological Clinic Waldhof-Elgershausen, University of Giessen Marburg Lung Center, Member of the German Center for Lung Research (Deutsches Zentrum für Lungenforschung, DZL), Giessen, Germany.
Respiration. 2021;100(3):238-271. doi: 10.1159/000512315. Epub 2021 Jan 22.
Idiopathic pulmonary fibrosis (IPF) is a severe and often fatal disease. Diagnosis of IPF requires considerable expertise and experience. Since the publication of the international IPF guideline in the year 2011 and the update 2018 several studies and technical advances have occurred, which made a new assessment of the diagnostic process mandatory. The goal of this guideline is to foster early, confident, and effective diagnosis of IPF. The guideline focusses on the typical clinical context of an IPF patient and provides tools to exclude known causes of interstitial lung disease including standardized questionnaires, serologic testing, and cellular analysis of bronchoalveolar lavage. High-resolution computed tomography remains crucial in the diagnostic workup. If it is necessary to obtain specimens for histology, transbronchial lung cryobiopsy is the primary approach, while surgical lung biopsy is reserved for patients who are fit for it and in whom a bronchoscopic diagnosis did not provide the information needed. After all, IPF is a diagnosis of exclusion and multidisciplinary discussion remains the golden standard of diagnosis.
特发性肺纤维化（IPF）是一种严重且常常致命的疾病。IPF 的诊断需要相当的专业知识和经验。自 2011 年国际 IPF 指南发布以及 2018 年更新以来，已经有多项研究和技术进展，这使得对诊断过程进行新的评估变得必要。本指南的目的是促进 IPF 的早期、有信心和有效的诊断。该指南侧重于 IPF 患者的典型临床情况，并提供工具来排除已知的间质性肺疾病的病因，包括标准化问卷、血清学检测和支气管肺泡灌洗的细胞分析。高分辨率计算机断层扫描在诊断工作中仍然至关重要。如果需要获取组织学标本，经支气管肺冷冻活检是主要方法，而外科肺活检则保留给适合该方法的患者，并且支气管镜诊断未能提供所需的信息。毕竟，IPF 是一种排除性诊断，多学科讨论仍然是诊断的黄金标准。
