[German Guideline for Idiopathic Pulmonary Fibrosis].

Behr, J; Günther, A; Bonella, F; Dinkel, J; Fink, L; Geiser, T.; Geißler, K; Gläser, S; Handzhhiev, S; Jonigk, D; Koschel, D; Kreuter, M; Leuschner, G; Markart, P; Prasse, A; Schönfeld, N; Schupp, J C; Sitter, H; Müller-Quernheim, J and Costabel, U (2020). [German Guideline for Idiopathic Pulmonary Fibrosis]. Pneumologie, 74(5), pp. 263-293. Thieme 10.1055/a-1120-3531

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Idiopathic pulmonary fibrosis (IPF) is a severe and often fatal disease. Diagnosis of IPF requires considerable expertise and experience. Since publication of the international IPF guideline in the year 2011 and Update 2018 several studies and technical advances occurred, which made a new assessment of the diagnostic process mandatory. In view of the antifibrotic drugs which have been approved for the treatment of IPF patients, the goal of this guideline is to foster early, confident and effective diagnosis of IPF. The guideline focusses on the typical clinical setting of an IPF patient and provides tools to exclude known causes of interstitial lung disease including standardised questionnaires, serologic testing and cellular analysis of bronchoalveolar lavage. High resolution computed tomography remains crucial in the diagnostic work-up. If it is necessary to obtain specimen 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 bronchoscopic diagnosis did not provide the information needed. Despite considerable progress, IPF remains 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:

04 May 2020 10:47

Last Modified:

31 Aug 2020 09:41

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