دورية أكاديمية
Clinical and Functional Characteristics of Patients with Unclassifiable Interstitial Lung Disease (uILD): Long-Term Follow-Up Data from European IPF Registry (eurIPFreg)
العنوان: | Clinical and Functional Characteristics of Patients with Unclassifiable Interstitial Lung Disease (uILD): Long-Term Follow-Up Data from European IPF Registry (eurIPFreg) |
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المؤلفون: | Ekaterina Krauss, Mustapha El-Guelai, Joern Pons-Kuehnemann, Ruth C. Dartsch, Silke Tello, Martina Korfei, Poornima Mahavadi, Andreas Breithecker, Ludger Fink, Mark Stoehr, Raphael W. Majeed, Werner Seeger, Bruno Crestani, Andreas Guenther |
المصدر: | Journal of Clinical Medicine, Vol 9, Iss 8, p 2499 (2020) |
بيانات النشر: | MDPI AG, 2020. |
سنة النشر: | 2020 |
المجموعة: | LCC:Medicine |
مصطلحات موضوعية: | unclassifiable interstitial lung disease (uILD), idiopathic pulmonary fibrosis (IPF), European Registry for idiopathic pulmonary fibrosis (eurIPFreg), interstitial lung diseases (ILD), Health-related quality of life (HRQoL), Medicine |
الوصف: | (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. |
نوع الوثيقة: | article |
وصف الملف: | electronic resource |
اللغة: | English |
تدمد: | 2077-0383 39857883 |
Relation: | https://www.mdpi.com/2077-0383/9/8/2499; https://doaj.org/toc/2077-0383 |
DOI: | 10.3390/jcm9082499 |
URL الوصول: | https://doaj.org/article/44d24489b1974b75a34686fe39857883 |
رقم الأكسشن: | edsdoj.44d24489b1974b75a34686fe39857883 |
قاعدة البيانات: | Directory of Open Access Journals |
تدمد: | 20770383 39857883 |
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DOI: | 10.3390/jcm9082499 |