Idiopathic pulmonary fibrosis in a Swiss interstitial lung disease reference centre.

Guler, Sabina; Zumstein, Pascal; Berezowska, Sabina Anna; Pöllinger, Alexander; Geiser, Thomas; Funke-Chambour, Manuela (2018). Idiopathic pulmonary fibrosis in a Swiss interstitial lung disease reference centre. Swiss medical weekly, 148(w14577), w14577. EMH Schweizerischer Ärzteverlag 10.4414/smw.2018.14577

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BACKGROUND Idiopathic pulmonary fibrosis (IPF) differs substantially from other idiopathic interstitial pneumonias regarding disease trajectory and the appropriate management strategies, making meticulous diagnosis essential. However, patient characteristics and clinical practice vary between clinical trials, and real life and registries provide the opportunity to critically analyse current clinical practices in order to ultimately improve patient care. METHODS We aimed to identify characteristics of our baseline IPF cohort at initiation of a web-based registry for patients with idiopathic interstitial pneumonia. Baseline and 6-month follow-up data from all consecutive IPF patients consulting at our centre over 2 years were analysed. RESULTS Forty IPF patients were included for baseline and 23 for longitudinal analysis. Besides many similarities to other IPF populations, our cohort included considerably fewer women. Forced vital capacity impairment in our cohort was more severe and mortality prediction poorer than in clinical trials, which emphasises the importance to confirm the applicability of clinical trial results with data from real life settings. CONCLUSION Registries for rare diseases such as IPF are a valuable resource for studying the course of the disease under current compliance with diagnostic and treatment guidelines and to appreciate local epidemiological particularities.

Item Type:

Journal Article (Original Article)


04 Faculty of Medicine > Department of Gastro-intestinal, Liver and Lung Disorders (DMLL) > Clinic of Pneumology
04 Faculty of Medicine > Pre-clinic Human Medicine > BioMedical Research (DBMR) > Forschungsbereich Mu50 > Forschungsgruppe Pneumologie (Erwachsene)
04 Faculty of Medicine > Department of Radiology, Neuroradiology and Nuclear Medicine (DRNN) > Institute of Diagnostic, Interventional and Paediatric Radiology
04 Faculty of Medicine > Service Sector > Institute of Pathology > Clinical Pathology

UniBE Contributor:

Guler, Sabina; Berezowska, Sabina Anna; Pöllinger, Alexander; Geiser, Thomas and Funke-Chambour, Manuela


600 Technology > 610 Medicine & health




EMH Schweizerischer Ärzteverlag




Sabina Anna Berezowska

Date Deposited:

31 Jan 2018 09:16

Last Modified:

04 Feb 2018 02:20

Publisher DOI:


PubMed ID:





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