S2K Guideline for Diagnosis of Idiopathic Pulmonary Fibrosis.

Behr, Jürgen; Günther, Andreas; Bonella, Francesco; Dinkel, Julien; Fink, Ludger; Geiser, Thomas; Geissler, Klaus; Gläser, Sven; Handzhiev, Sabin; Jonigk, Danny; Koschel, Dirk; Kreuter, Michael; Leuschner, Gabriela; Markart, Philipp; Prasse, Antje; Schönfeld, Nicolas; Schupp, Jonas Christian; Sitter, Helmut; Müller-Quernheim, Joachim and Costabel, Ulrich (2021). S2K Guideline for Diagnosis of Idiopathic Pulmonary Fibrosis. Respiration, 100(3), pp. 238-271. Karger 10.1159/000512315

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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.

Item Type:

Journal Article (Review Article)


04 Faculty of Medicine > Department of Gastro-intestinal, Liver and Lung Disorders (DMLL) > Clinic of Pneumology

UniBE Contributor:

Geiser, Thomas


600 Technology > 610 Medicine & health








Heidi Lobsiger

Date Deposited:

16 Feb 2021 11:14

Last Modified:

13 Mar 2021 01:34

Publisher DOI:


PubMed ID:


Uncontrolled Keywords:

Biopsy Bronchoalveolar lavage Diagnosis Guideline High-resolution computed tomography Idiopathic pulmonary fibrosis Serology





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