Clinical and Functional Characteristics of Patients with Unclassifiable Interstitial Lung Disease (uILD): Long-Term Follow-Up Data from European IPF Registry (eurIPFreg).

European Registry for idiopathic pulmonary fibrosis (eurIPFreg) Health-related quality of life (HRQoL) idiopathic pulmonary fibrosis (IPF) interstitial lung diseases (ILD) unclassifiable interstitial lung disease (uILD)

Journal

Journal of clinical medicine
ISSN: 2077-0383
Titre abrégé: J Clin Med
Pays: Switzerland
ID NLM: 101606588

Informations de publication

Date de publication:
03 Aug 2020
Historique:
received: 07 07 2020
revised: 26 07 2020
accepted: 31 07 2020
entrez: 7 8 2020
pubmed: 7 8 2020
medline: 7 8 2020
Statut: epublish

Résumé

(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 (

Identifiants

pubmed: 32756496
pii: jcm9082499
doi: 10.3390/jcm9082499
pmc: PMC7464480
pii:
doi:

Types de publication

Journal Article

Langues

eng

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Auteurs

Ekaterina Krauss (E)

European IPF Registry & Biobank (eurIPFreg/bank), 35392 Giessen, Germany.
Universities of Giessen and Marburg Lung Center (UGMLC), Member of the German Center for Lung Research (DZL), 35392 Giessen, Germany.

Mustapha El-Guelai (M)

European IPF Registry & Biobank (eurIPFreg/bank), 35392 Giessen, Germany.
Universities of Giessen and Marburg Lung Center (UGMLC), Member of the German Center for Lung Research (DZL), 35392 Giessen, Germany.

Joern Pons-Kuehnemann (J)

Medical Statistics, Institute of Medical Informatics, Justus-Liebig University of Giessen; 35392 Giessen, Germany.

Ruth C Dartsch (RC)

European IPF Registry & Biobank (eurIPFreg/bank), 35392 Giessen, Germany.
Universities of Giessen and Marburg Lung Center (UGMLC), Member of the German Center for Lung Research (DZL), 35392 Giessen, Germany.

Silke Tello (S)

European IPF Registry & Biobank (eurIPFreg/bank), 35392 Giessen, Germany.
Universities of Giessen and Marburg Lung Center (UGMLC), Member of the German Center for Lung Research (DZL), 35392 Giessen, Germany.

Martina Korfei (M)

European IPF Registry & Biobank (eurIPFreg/bank), 35392 Giessen, Germany.
Universities of Giessen and Marburg Lung Center (UGMLC), Member of the German Center for Lung Research (DZL), 35392 Giessen, Germany.

Poornima Mahavadi (P)

European IPF Registry & Biobank (eurIPFreg/bank), 35392 Giessen, Germany.
Universities of Giessen and Marburg Lung Center (UGMLC), Member of the German Center for Lung Research (DZL), 35392 Giessen, Germany.

Andreas Breithecker (A)

Universities of Giessen and Marburg Lung Center (UGMLC), Member of the German Center for Lung Research (DZL), 35392 Giessen, Germany.
Gesundheitszentrum Wetterau, 61231 Bad Nauheim, Germany.

Ludger Fink (L)

Universities of Giessen and Marburg Lung Center (UGMLC), Member of the German Center for Lung Research (DZL), 35392 Giessen, Germany.
Gesundheitszentrum Wetterau, 61231 Bad Nauheim, Germany.
Institute of Pathology, Cytology, and Molecular Pathology, 35578 Wetzlar, Germany.

Mark Stoehr (M)

European IPF Registry & Biobank (eurIPFreg/bank), 35392 Giessen, Germany.

Raphael W Majeed (RW)

European IPF Registry & Biobank (eurIPFreg/bank), 35392 Giessen, Germany.

Werner Seeger (W)

European IPF Registry & Biobank (eurIPFreg/bank), 35392 Giessen, Germany.
Universities of Giessen and Marburg Lung Center (UGMLC), Member of the German Center for Lung Research (DZL), 35392 Giessen, Germany.
Cardio-Pulmonary Institute (CPI) 35392 Giessen, Germany.

Bruno Crestani (B)

European IPF Registry & Biobank (eurIPFreg/bank), 35392 Giessen, Germany.
Institute National de la Sainté et de la Recherche Médicale, Hopital Bichat, Service de Pneumologie, 75018 Paris, France.

Andreas Guenther (A)

European IPF Registry & Biobank (eurIPFreg/bank), 35392 Giessen, Germany.
Universities of Giessen and Marburg Lung Center (UGMLC), Member of the German Center for Lung Research (DZL), 35392 Giessen, Germany.
Cardio-Pulmonary Institute (CPI) 35392 Giessen, Germany.
Agaplesion Lung Clinic Waldhof-Elgershausen, 35753 Greifenstein, Germany.

Classifications MeSH