Idiopathic pulmonary fibrosis in a Swiss interstitial lung disease reference centre
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 be...
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| Format: | Article |
| Language: | English |
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SMW supporting association (Trägerverein Swiss Medical Weekly SMW)
2018-01-01
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| Series: | Swiss Medical Weekly |
| Subjects: | |
| Online Access: | https://www.smw.ch/index.php/smw/article/view/2427 |
| _version_ | 1798003628341985280 |
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| author | Sabina A. Guler Pascal Zumstein Sabina Berezowska Alexander Poellinger Thomas Geiser Manuela Funke-Chambour |
| author_facet | Sabina A. Guler Pascal Zumstein Sabina Berezowska Alexander Poellinger Thomas Geiser Manuela Funke-Chambour |
| author_sort | Sabina A. Guler |
| collection | DOAJ |
| description |
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.
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| first_indexed | 2024-04-11T12:12:05Z |
| format | Article |
| id | doaj.art-b6b16cad89aa4f72a2685d3c028d6666 |
| institution | Directory Open Access Journal |
| issn | 1424-3997 |
| language | English |
| last_indexed | 2024-04-11T12:12:05Z |
| publishDate | 2018-01-01 |
| publisher | SMW supporting association (Trägerverein Swiss Medical Weekly SMW) |
| record_format | Article |
| series | Swiss Medical Weekly |
| spelling | doaj.art-b6b16cad89aa4f72a2685d3c028d66662022-12-22T04:24:36ZengSMW supporting association (Trägerverein Swiss Medical Weekly SMW)Swiss Medical Weekly1424-39972018-01-01148010210.4414/smw.2018.14577Idiopathic pulmonary fibrosis in a Swiss interstitial lung disease reference centreSabina A. Guler0Pascal Zumstein1Sabina Berezowska2Alexander Poellinger3Thomas Geiser4Manuela Funke-Chambour5Department of Pulmonary Medicine, University Hospital and University of Bern, SwitzerlandDepartment of Pulmonary Medicine, University Hospital and University of Bern, SwitzerlandInstitute of Pathology, University of Bern, SwitzerlandDepartment of Diagnostic, Interventional and Paediatric Radiology, University Hospital and University of Bern, SwitzerlandDepartment of Pulmonary Medicine, University Hospital and University of Bern, SwitzerlandDepartment of Pulmonary Medicine, University Hospital and University of Bern, Switzerland 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. https://www.smw.ch/index.php/smw/article/view/2427idiopathic interstitial pneumoniaidiopathic pulmonary fibrosisregistryreal life practiceCohort study |
| spellingShingle | Sabina A. Guler Pascal Zumstein Sabina Berezowska Alexander Poellinger Thomas Geiser Manuela Funke-Chambour Idiopathic pulmonary fibrosis in a Swiss interstitial lung disease reference centre Swiss Medical Weekly idiopathic interstitial pneumonia idiopathic pulmonary fibrosis registry real life practice Cohort study |
| title | Idiopathic pulmonary fibrosis in a Swiss interstitial lung disease reference centre |
| title_full | Idiopathic pulmonary fibrosis in a Swiss interstitial lung disease reference centre |
| title_fullStr | Idiopathic pulmonary fibrosis in a Swiss interstitial lung disease reference centre |
| title_full_unstemmed | Idiopathic pulmonary fibrosis in a Swiss interstitial lung disease reference centre |
| title_short | Idiopathic pulmonary fibrosis in a Swiss interstitial lung disease reference centre |
| title_sort | idiopathic pulmonary fibrosis in a swiss interstitial lung disease reference centre |
| topic | idiopathic interstitial pneumonia idiopathic pulmonary fibrosis registry real life practice Cohort study |
| url | https://www.smw.ch/index.php/smw/article/view/2427 |
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