Clinical profile of unclassifiable interstitial lung disease: Comparison with chronic fibrosing idiopathic interstitial pneumonias

Objective Unclassifiable interstitial lung disease (ILD) is a common problem in clinical practice. These patients pose a distinct challenge with regard to appropriate evaluation and management. We investigated the clinical features and prognosis of unclassifiable ILD and compared its clinical profil...

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Main Authors: Daniel Traila, Cristian Oancea, Emanuela Tudorache, Ovidiu Fira Mladinescu, Bogdan Timar, Voicu Tudorache
Format: Article
Language:English
Published: SAGE Publishing 2018-01-01
Series:Journal of International Medical Research
Online Access:https://doi.org/10.1177/0300060517719767
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author Daniel Traila
Cristian Oancea
Emanuela Tudorache
Ovidiu Fira Mladinescu
Bogdan Timar
Voicu Tudorache
author_facet Daniel Traila
Cristian Oancea
Emanuela Tudorache
Ovidiu Fira Mladinescu
Bogdan Timar
Voicu Tudorache
author_sort Daniel Traila
collection DOAJ
description Objective Unclassifiable interstitial lung disease (ILD) is a common problem in clinical practice. These patients pose a distinct challenge with regard to appropriate evaluation and management. We investigated the clinical features and prognosis of unclassifiable ILD and compared its clinical profile with that of idiopathic pulmonary fibrosis (IPF) and idiopathic nonspecific interstitial pneumonia (NSIP). Methods Patients with IPF (n = 40), NSIP (n = 14), and unclassifiable ILD (n = 27) were selected from an ongoing database. Baseline clinical features, pulmonary function, and the extent of fibrosis on high-resolution computed tomography (HRCT) were evaluated. Mortality was estimated based on the ILD–Gender, Age, Physiology (ILD-GAP) index and composite physiologic index (CPI). Results IPF was associated with the worst survival (hazard ratio [HR] = 4.361 compared with NSIP), followed by unclassifiable cases (HR = 1.251 compared with NSIP). Increasing mortality was significantly impacted by age (HR = 1.04 per 1-year increase), lower carbon monoxide diffusing capacity of the lung (HR = 0.97), HRCT interstitial score (HR = 1.119 per 1-point increase), ILD-GAP score (HR = 1.570 per 1-point increase), and CPI (HR = 1.039 per 1-point increase). Conclusions Patients with unclassifiable ILD had an intermediate prognosis between that of IPF and NSIP. Patients at high risk of mortality can be identified using baseline clinical, physiological, and radiological features.
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spelling doaj.art-c6be1a80b74d4fb6a1a6d46ecaddc5aa2022-12-21T19:08:46ZengSAGE PublishingJournal of International Medical Research0300-06051473-23002018-01-014610.1177/0300060517719767Clinical profile of unclassifiable interstitial lung disease: Comparison with chronic fibrosing idiopathic interstitial pneumoniasDaniel Traila0Cristian Oancea1Emanuela Tudorache2Ovidiu Fira Mladinescu3Bogdan Timar4Voicu Tudorache5Department of Pulmonary Medicine, University of Medicine and Pharmacy “Victor Babes,” Timisoara, RomâniaDepartment of Pulmonary Medicine, University of Medicine and Pharmacy “Victor Babes,” Timisoara, RomâniaDepartment of Pulmonary Medicine, University of Medicine and Pharmacy “Victor Babes,” Timisoara, RomâniaDepartment of Pulmonary Medicine, University of Medicine and Pharmacy “Victor Babes,” Timisoara, RomâniaDepartment of Biostatistics and Medical Informatics, University of Medicine and Pharmacy “Victor Babes,” Timisoara, RomâniaDepartment of Pulmonary Medicine, University of Medicine and Pharmacy “Victor Babes,” Timisoara, RomâniaObjective Unclassifiable interstitial lung disease (ILD) is a common problem in clinical practice. These patients pose a distinct challenge with regard to appropriate evaluation and management. We investigated the clinical features and prognosis of unclassifiable ILD and compared its clinical profile with that of idiopathic pulmonary fibrosis (IPF) and idiopathic nonspecific interstitial pneumonia (NSIP). Methods Patients with IPF (n = 40), NSIP (n = 14), and unclassifiable ILD (n = 27) were selected from an ongoing database. Baseline clinical features, pulmonary function, and the extent of fibrosis on high-resolution computed tomography (HRCT) were evaluated. Mortality was estimated based on the ILD–Gender, Age, Physiology (ILD-GAP) index and composite physiologic index (CPI). Results IPF was associated with the worst survival (hazard ratio [HR] = 4.361 compared with NSIP), followed by unclassifiable cases (HR = 1.251 compared with NSIP). Increasing mortality was significantly impacted by age (HR = 1.04 per 1-year increase), lower carbon monoxide diffusing capacity of the lung (HR = 0.97), HRCT interstitial score (HR = 1.119 per 1-point increase), ILD-GAP score (HR = 1.570 per 1-point increase), and CPI (HR = 1.039 per 1-point increase). Conclusions Patients with unclassifiable ILD had an intermediate prognosis between that of IPF and NSIP. Patients at high risk of mortality can be identified using baseline clinical, physiological, and radiological features.https://doi.org/10.1177/0300060517719767
spellingShingle Daniel Traila
Cristian Oancea
Emanuela Tudorache
Ovidiu Fira Mladinescu
Bogdan Timar
Voicu Tudorache
Clinical profile of unclassifiable interstitial lung disease: Comparison with chronic fibrosing idiopathic interstitial pneumonias
Journal of International Medical Research
title Clinical profile of unclassifiable interstitial lung disease: Comparison with chronic fibrosing idiopathic interstitial pneumonias
title_full Clinical profile of unclassifiable interstitial lung disease: Comparison with chronic fibrosing idiopathic interstitial pneumonias
title_fullStr Clinical profile of unclassifiable interstitial lung disease: Comparison with chronic fibrosing idiopathic interstitial pneumonias
title_full_unstemmed Clinical profile of unclassifiable interstitial lung disease: Comparison with chronic fibrosing idiopathic interstitial pneumonias
title_short Clinical profile of unclassifiable interstitial lung disease: Comparison with chronic fibrosing idiopathic interstitial pneumonias
title_sort clinical profile of unclassifiable interstitial lung disease comparison with chronic fibrosing idiopathic interstitial pneumonias
url https://doi.org/10.1177/0300060517719767
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