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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...

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Autores principales: 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
Formato: Online Artículo Texto
Lenguaje:English
Publicado: MDPI 2020
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.
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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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