Progressive fibrosing interstitial lung disease: prevalence and clinical outcome

Abstract Background The progressive fibrosing (PF) phenotype of interstitial lung disease (ILD) is characterised by worsening respiratory symptoms, lung function, and extent of fibrosis on high-resolution computed tomography. We aimed to investigate the prevalence and clinical outcomes of PF-ILD in...

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Autores principales:	Byoung Soo Kwon, Jooae Choe, Eun Jin Chae, Hee Sang Hwang, Yong-Gil Kim, Jin Woo Song
Formato:	article
Lenguaje:	EN
Publicado:	BMC 2021
Materias:	Phenotype Interstitial lung disease Outcome Prevalence Progressive Diseases of the respiratory system RC705-779
Acceso en línea:	https://doaj.org/article/d201f08ad0a2424cb3e5efb94afeffdd
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spelling	oai:doaj.org-article:d201f08ad0a2424cb3e5efb94afeffdd2021-11-08T11:03:48ZProgressive fibrosing interstitial lung disease: prevalence and clinical outcome10.1186/s12931-021-01879-61465-993Xhttps://doaj.org/article/d201f08ad0a2424cb3e5efb94afeffdd2021-10-01T00:00:00Zhttps://doi.org/10.1186/s12931-021-01879-6https://doaj.org/toc/1465-993XAbstract Background The progressive fibrosing (PF) phenotype of interstitial lung disease (ILD) is characterised by worsening respiratory symptoms, lung function, and extent of fibrosis on high-resolution computed tomography. We aimed to investigate the prevalence and clinical outcomes of PF-ILD in a real-world cohort and assess the prognostic significance of the PF-ILD diagnostic criteria. Methods Clinical data of patients with fibrosing ILD other than idiopathic pulmonary fibrosis (IPF) consecutively diagnosed at a single centre were retrospectively reviewed. A PF phenotype was defined based on the criteria used in the INBUILD trial. Results The median follow-up duration was 62.7 months. Of the total of 396 patients, the mean age was 58.1 years, 39.9% were men, and rheumatoid arthritis-ILD was the most common (42.4%). A PF phenotype was identified in 135 patients (34.1%). The PF-ILD group showed lower forced vital capacity and total lung capacity (TLC) than the non-PF-ILD group. The PF-ILD group also showed poorer survival (median survival, 91.2 months vs. not reached; P < 0.001) than the non-PF-ILD group. In multivariable Cox analysis adjusted for age, DLCO, HRCT pattern, and specific diagnosis, PF phenotype was independent prognostic factor (hazard ratio, 3.053; P < 0.001) in patients with fibrosing ILD. Each criterion of PF-ILD showed similar survival outcomes. Conclusions Our results showed that approximately 34% of patients with non-IPF fibrosing ILD showed a progressive phenotype and a poor outcome similar to that of IPF, regardless of the diagnostic criteria used.Byoung Soo KwonJooae ChoeEun Jin ChaeHee Sang HwangYong-Gil KimJin Woo SongBMCarticlePhenotypeInterstitial lung diseaseOutcomePrevalenceProgressiveDiseases of the respiratory systemRC705-779ENRespiratory Research, Vol 22, Iss 1, Pp 1-9 (2021)
institution	DOAJ
collection	DOAJ
language	EN
topic	Phenotype Interstitial lung disease Outcome Prevalence Progressive Diseases of the respiratory system RC705-779
spellingShingle	Phenotype Interstitial lung disease Outcome Prevalence Progressive Diseases of the respiratory system RC705-779 Byoung Soo Kwon Jooae Choe Eun Jin Chae Hee Sang Hwang Yong-Gil Kim Jin Woo Song Progressive fibrosing interstitial lung disease: prevalence and clinical outcome
description	Abstract Background The progressive fibrosing (PF) phenotype of interstitial lung disease (ILD) is characterised by worsening respiratory symptoms, lung function, and extent of fibrosis on high-resolution computed tomography. We aimed to investigate the prevalence and clinical outcomes of PF-ILD in a real-world cohort and assess the prognostic significance of the PF-ILD diagnostic criteria. Methods Clinical data of patients with fibrosing ILD other than idiopathic pulmonary fibrosis (IPF) consecutively diagnosed at a single centre were retrospectively reviewed. A PF phenotype was defined based on the criteria used in the INBUILD trial. Results The median follow-up duration was 62.7 months. Of the total of 396 patients, the mean age was 58.1 years, 39.9% were men, and rheumatoid arthritis-ILD was the most common (42.4%). A PF phenotype was identified in 135 patients (34.1%). The PF-ILD group showed lower forced vital capacity and total lung capacity (TLC) than the non-PF-ILD group. The PF-ILD group also showed poorer survival (median survival, 91.2 months vs. not reached; P < 0.001) than the non-PF-ILD group. In multivariable Cox analysis adjusted for age, DLCO, HRCT pattern, and specific diagnosis, PF phenotype was independent prognostic factor (hazard ratio, 3.053; P < 0.001) in patients with fibrosing ILD. Each criterion of PF-ILD showed similar survival outcomes. Conclusions Our results showed that approximately 34% of patients with non-IPF fibrosing ILD showed a progressive phenotype and a poor outcome similar to that of IPF, regardless of the diagnostic criteria used.
format	article
author	Byoung Soo Kwon Jooae Choe Eun Jin Chae Hee Sang Hwang Yong-Gil Kim Jin Woo Song
author_facet	Byoung Soo Kwon Jooae Choe Eun Jin Chae Hee Sang Hwang Yong-Gil Kim Jin Woo Song
author_sort	Byoung Soo Kwon
title	Progressive fibrosing interstitial lung disease: prevalence and clinical outcome
title_short	Progressive fibrosing interstitial lung disease: prevalence and clinical outcome
title_full	Progressive fibrosing interstitial lung disease: prevalence and clinical outcome
title_fullStr	Progressive fibrosing interstitial lung disease: prevalence and clinical outcome
title_full_unstemmed	Progressive fibrosing interstitial lung disease: prevalence and clinical outcome
title_sort	progressive fibrosing interstitial lung disease: prevalence and clinical outcome
publisher	BMC
publishDate	2021
url	https://doaj.org/article/d201f08ad0a2424cb3e5efb94afeffdd
work_keys_str_mv	AT byoungsookwon progressivefibrosinginterstitiallungdiseaseprevalenceandclinicaloutcome AT jooaechoe progressivefibrosinginterstitiallungdiseaseprevalenceandclinicaloutcome AT eunjinchae progressivefibrosinginterstitiallungdiseaseprevalenceandclinicaloutcome AT heesanghwang progressivefibrosinginterstitiallungdiseaseprevalenceandclinicaloutcome AT yonggilkim progressivefibrosinginterstitiallungdiseaseprevalenceandclinicaloutcome AT jinwoosong progressivefibrosinginterstitiallungdiseaseprevalenceandclinicaloutcome
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Progressive fibrosing interstitial lung disease: prevalence and clinical outcome

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