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Clinical and Functional Characteristics of Patients with Unclassifiable Interstitial Lung Disease (uILD): Long-Term Follow-Up Data from European IPF Registry (eurIPFreg)
(1) Aim of the study: In spite of extensive research, up to 20% of interstitial lung diseases (ILD) patients cannot be safely classified. We analyzed clinical features, progression factors, and outcomes of unclassifiable ILD (uILD). (2) Methods: A total of 140 uILD subjects from the University of Gi...
Autores principales: | , , , , , , , , , , , , , |
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Formato: | Online Artículo Texto |
Lenguaje: | English |
Publicado: |
MDPI
2020
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Materias: | |
Acceso en línea: | https://www.ncbi.nlm.nih.gov/pmc/articles/PMC7464480/ https://www.ncbi.nlm.nih.gov/pubmed/32756496 http://dx.doi.org/10.3390/jcm9082499 |
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author | Krauss, Ekaterina El-Guelai, Mustapha Pons-Kuehnemann, Joern Dartsch, Ruth C. Tello, Silke Korfei, Martina Mahavadi, Poornima Breithecker, Andreas Fink, Ludger Stoehr, Mark Majeed, Raphael W. Seeger, Werner Crestani, Bruno Guenther, Andreas |
author_facet | Krauss, Ekaterina El-Guelai, Mustapha Pons-Kuehnemann, Joern Dartsch, Ruth C. Tello, Silke Korfei, Martina Mahavadi, Poornima Breithecker, Andreas Fink, Ludger Stoehr, Mark Majeed, Raphael W. Seeger, Werner Crestani, Bruno Guenther, Andreas |
author_sort | Krauss, Ekaterina |
collection | PubMed |
description | (1) Aim of the study: In spite of extensive research, up to 20% of interstitial lung diseases (ILD) patients cannot be safely classified. We analyzed clinical features, progression factors, and outcomes of unclassifiable ILD (uILD). (2) Methods: A total of 140 uILD subjects from the University of Giessen and Marburg Lung Center (UGMLC) were recruited between 11/2009 and 01/2019 into the European Registry for idiopathic pulmonary fibrosis (eurIPFreg) and followed until 01/2020. The diagnosis of uILD was applied only when a conclusive diagnosis could not be reached with certainty. (3) Results: In 46.4% of the patients, the uILD diagnosis was due to conflicting clinical, radiological, and pathological data. By applying the diagnostic criteria of usual interstitial pneumonia (UIP) based on computed tomography (CT), published by the Fleischner Society, 22.2% of the patients displayed a typical UIP pattern. We also showed that forced vital capacity (FVC) at baseline (p = 0.008), annual FVC decline ≥10% (p < 0.0001), smoking (p = 0.033), and a diffusing capacity of the lung for carbon monoxide (DLco) ≤55% of predicted value at baseline (p < 0.0001) were significantly associated with progressive disease. (4) Conclusions: The most important prognostic factors in uILD are baseline level and decline in lung function and smoking. The use of Fleischner diagnostic criteria allows further differentiation and accurate diagnosis. |
format | Online Article Text |
id | pubmed-7464480 |
institution | National Center for Biotechnology Information |
language | English |
publishDate | 2020 |
publisher | MDPI |
record_format | MEDLINE/PubMed |
spelling | pubmed-74644802020-09-04 Clinical and Functional Characteristics of Patients with Unclassifiable Interstitial Lung Disease (uILD): Long-Term Follow-Up Data from European IPF Registry (eurIPFreg) Krauss, Ekaterina El-Guelai, Mustapha Pons-Kuehnemann, Joern Dartsch, Ruth C. Tello, Silke Korfei, Martina Mahavadi, Poornima Breithecker, Andreas Fink, Ludger Stoehr, Mark Majeed, Raphael W. Seeger, Werner Crestani, Bruno Guenther, Andreas J Clin Med Article (1) Aim of the study: In spite of extensive research, up to 20% of interstitial lung diseases (ILD) patients cannot be safely classified. We analyzed clinical features, progression factors, and outcomes of unclassifiable ILD (uILD). (2) Methods: A total of 140 uILD subjects from the University of Giessen and Marburg Lung Center (UGMLC) were recruited between 11/2009 and 01/2019 into the European Registry for idiopathic pulmonary fibrosis (eurIPFreg) and followed until 01/2020. The diagnosis of uILD was applied only when a conclusive diagnosis could not be reached with certainty. (3) Results: In 46.4% of the patients, the uILD diagnosis was due to conflicting clinical, radiological, and pathological data. By applying the diagnostic criteria of usual interstitial pneumonia (UIP) based on computed tomography (CT), published by the Fleischner Society, 22.2% of the patients displayed a typical UIP pattern. We also showed that forced vital capacity (FVC) at baseline (p = 0.008), annual FVC decline ≥10% (p < 0.0001), smoking (p = 0.033), and a diffusing capacity of the lung for carbon monoxide (DLco) ≤55% of predicted value at baseline (p < 0.0001) were significantly associated with progressive disease. (4) Conclusions: The most important prognostic factors in uILD are baseline level and decline in lung function and smoking. The use of Fleischner diagnostic criteria allows further differentiation and accurate diagnosis. MDPI 2020-08-03 /pmc/articles/PMC7464480/ /pubmed/32756496 http://dx.doi.org/10.3390/jcm9082499 Text en © 2020 by the authors. Licensee MDPI, Basel, Switzerland. This article is an open access article distributed under the terms and conditions of the Creative Commons Attribution (CC BY) license (http://creativecommons.org/licenses/by/4.0/). |
spellingShingle | Article Krauss, Ekaterina El-Guelai, Mustapha Pons-Kuehnemann, Joern Dartsch, Ruth C. Tello, Silke Korfei, Martina Mahavadi, Poornima Breithecker, Andreas Fink, Ludger Stoehr, Mark Majeed, Raphael W. Seeger, Werner Crestani, Bruno Guenther, Andreas Clinical and Functional Characteristics of Patients with Unclassifiable Interstitial Lung Disease (uILD): Long-Term Follow-Up Data from European IPF Registry (eurIPFreg) |
title | Clinical and Functional Characteristics of Patients with Unclassifiable Interstitial Lung Disease (uILD): Long-Term Follow-Up Data from European IPF Registry (eurIPFreg) |
title_full | Clinical and Functional Characteristics of Patients with Unclassifiable Interstitial Lung Disease (uILD): Long-Term Follow-Up Data from European IPF Registry (eurIPFreg) |
title_fullStr | Clinical and Functional Characteristics of Patients with Unclassifiable Interstitial Lung Disease (uILD): Long-Term Follow-Up Data from European IPF Registry (eurIPFreg) |
title_full_unstemmed | Clinical and Functional Characteristics of Patients with Unclassifiable Interstitial Lung Disease (uILD): Long-Term Follow-Up Data from European IPF Registry (eurIPFreg) |
title_short | Clinical and Functional Characteristics of Patients with Unclassifiable Interstitial Lung Disease (uILD): Long-Term Follow-Up Data from European IPF Registry (eurIPFreg) |
title_sort | clinical and functional characteristics of patients with unclassifiable interstitial lung disease (uild): long-term follow-up data from european ipf registry (euripfreg) |
topic | Article |
url | https://www.ncbi.nlm.nih.gov/pmc/articles/PMC7464480/ https://www.ncbi.nlm.nih.gov/pubmed/32756496 http://dx.doi.org/10.3390/jcm9082499 |
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