S2K Guideline for Diagnosis of Idiopathic Pulmonary Fibrosis

Jürgen Behr, Andreas Günther, Francesco Bonella, Julien Dinkel, Ludger Fink, Thomas Geiser, Klaus Geissler, Sven Gläser, Sabin Handzhiev, Danny Jonigk, Dirk Koschel, Michael Kreuter, Gabriela Leuschner, Philipp Markart, Antje Prasse, Nicolas Schönfeld, Jonas Christian Schupp, Helmut Sitter, Joachim Müller-Quernheim, Ulrich Costabel

Research output: Journal article (peer-reviewed)Journal article

18 Citations (Scopus)


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.

Original languageEnglish
Pages (from-to)238-271
Number of pages34
Issue number3
Publication statusPublished - Mar 2021


  • Biopsy/methods
  • Bronchoalveolar Lavage/methods
  • Bronchoscopy/methods
  • Diagnosis, Differential
  • Humans
  • Idiopathic Pulmonary Fibrosis/diagnosis
  • Interdisciplinary Communication
  • Lung/diagnostic imaging
  • Lung Diseases, Interstitial/diagnosis
  • Patient Selection
  • Serologic Tests/methods
  • Tomography, X-Ray Computed/methods


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