Journal article

S2K Guideline for Diagnosis of Idiopathic Pulmonary Fibrosis


Authors listBehr, Jurgen; Guenther, Andreas; Bonella, Francesco; Dinkel, Julien; Fink, Ludger; Geiser, Thomas; Geissler, Klaus; Glaser, Sven; Handzhiev, Sabin; Jonigk, Danny; Koschel, Dirk; Kreuter, Michael; Leuschner, Gabriela; Markart, Philipp; Prasse, Antje; Schoenfeld, Nicolas; Schupp, Jonas Christian; Sitter, Helmut; Muller-Quernheim, Joachim; Costabel, Ulrich

Publication year2021

Pages238-270

JournalRespiration

Volume number100

Issue number3

ISSN0025-7931

eISSN1423-0356

Open access statusBronze

DOI Linkhttps://doi.org/10.1159/000512315

PublisherKarger Publishers


Abstract
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.



Citation Styles

Harvard Citation styleBehr, J., Guenther, A., Bonella, F., Dinkel, J., Fink, L., Geiser, T., et al. (2021) S2K Guideline for Diagnosis of Idiopathic Pulmonary Fibrosis, Respiration, 100(3), pp. 238-270. https://doi.org/10.1159/000512315

APA Citation styleBehr, J., Guenther, A., Bonella, F., Dinkel, J., Fink, L., Geiser, T., Geissler, K., Glaser, S., Handzhiev, S., Jonigk, D., Koschel, D., Kreuter, M., Leuschner, G., Markart, P., Prasse, A., Schoenfeld, N., Schupp, J., Sitter, H., Muller-Quernheim, J., ...Costabel, U. (2021). S2K Guideline for Diagnosis of Idiopathic Pulmonary Fibrosis. Respiration. 100(3), 238-270. https://doi.org/10.1159/000512315



Keywords


ACUTE EXACERBATIONBRONCHOALVEOLAR LAVAGECLINICAL-USEFULNESSFLEISCHNER-SOCIETYGUIDELINEHigh-resolution computed tomographyIdiopathic pulmonary fibrosisINTERSTITIAL LUNG-DISEASEOCCUPATIONAL-EXPOSURERESOLUTION COMPUTED-TOMOGRAPHYSURGICAL BIOPSYTRANSBRONCHIAL CRYOBIOPSYVIDEO-ASSISTED THORACOSCOPY

Last updated on 2025-10-06 at 11:21