Cargando…

Acute exacerbations of fibrosing interstitial lung disease associated with connective tissue diseases: a population-based study

BACKGROUND: Acute exacerbation (AE) is the major cause of morbidity and mortality in patients with idiopathic pulmonary fibrosis (IPF). AEs also occur in other forms of fibrosing interstitial lung disease (fILD). The clinical features and prognosis of AE patients with connective tissue diseases (CTD...

Descripción completa

Detalles Bibliográficos
Autores principales: Cao, Mengshu, Sheng, Jian, Qiu, Xiaohua, Wang, Dandan, Wang, Dongmei, Wang, Yang, Xiao, Yonglong, Cai, Hourong
Formato: Online Artículo Texto
Lenguaje:English
Publicado: BioMed Central 2019
Materias:
Acceso en línea:https://www.ncbi.nlm.nih.gov/pmc/articles/PMC6857302/
https://www.ncbi.nlm.nih.gov/pubmed/31727051
http://dx.doi.org/10.1186/s12890-019-0960-1
_version_ 1783470740896481280
author Cao, Mengshu
Sheng, Jian
Qiu, Xiaohua
Wang, Dandan
Wang, Dongmei
Wang, Yang
Xiao, Yonglong
Cai, Hourong
author_facet Cao, Mengshu
Sheng, Jian
Qiu, Xiaohua
Wang, Dandan
Wang, Dongmei
Wang, Yang
Xiao, Yonglong
Cai, Hourong
author_sort Cao, Mengshu
collection PubMed
description BACKGROUND: Acute exacerbation (AE) is the major cause of morbidity and mortality in patients with idiopathic pulmonary fibrosis (IPF). AEs also occur in other forms of fibrosing interstitial lung disease (fILD). The clinical features and prognosis of AE patients with connective tissue diseases (CTDs) associated-ILD has not been fully described. METHODS: We retrospectively reviewed 177 patients with either IPF or a characterized CTD-ILD admitted to Nanjing Drum Tower Hospital with an AE from January 2010 to December 2016. RESULTS: The study cohort included 107 subjects with AE-IPF and 70 cases with AE-CTD-ILD. Female gender, prior use of corticosteroid and immunosupressants, lower serum albumin, higher D-dimer level, TLC% pred, survival, and treatment with immunosupressants and caspofungin were more common in the CTD-ILD group (all p<0.05). The incidences of AE-CTD-ILD and AE-IPF were similar in our single center (p = 0.526). TLC% pred was the risk factor for AE after ILD diagnosis for 1 year in CTD patients (p = 0.018). Log-rank tests showed patients with CTD-ILD had a significantly lower mortality rate compared with IPF patients after AEs (p = 0.029). No significant difference in survival was noted among CTD subgroups (p = 0.353). The survival was negatively correlated with WBC count, LDH and CT score, (p = 0.006, p = 0.013 and p = 0.035, respectively), and positively correlated with PaO(2)/FiO(2) ratio (p<0.001) in the CTD-ILD group. WBC count and PO(2)/FiO(2) ratio were the independent predictors for survival in AE-CTD-ILD after adjusting for other clinical variates in Cox regression Models (p = 0.038 and p < 0.001, respectively). CONCLUSIONS: The clinical characteristics of patients with AE-CTD-ILD differed from those with AE-IPF, while AE incidences were similar between the two groups. Subjects with AE-CTD-fILD tended to have a better prognosis, and WBC count and PO(2)/FiO(2) ratio were the independent survival predictors for these patients.
format Online
Article
Text
id pubmed-6857302
institution National Center for Biotechnology Information
language English
publishDate 2019
publisher BioMed Central
record_format MEDLINE/PubMed
spelling pubmed-68573022019-12-05 Acute exacerbations of fibrosing interstitial lung disease associated with connective tissue diseases: a population-based study Cao, Mengshu Sheng, Jian Qiu, Xiaohua Wang, Dandan Wang, Dongmei Wang, Yang Xiao, Yonglong Cai, Hourong BMC Pulm Med Research Article BACKGROUND: Acute exacerbation (AE) is the major cause of morbidity and mortality in patients with idiopathic pulmonary fibrosis (IPF). AEs also occur in other forms of fibrosing interstitial lung disease (fILD). The clinical features and prognosis of AE patients with connective tissue diseases (CTDs) associated-ILD has not been fully described. METHODS: We retrospectively reviewed 177 patients with either IPF or a characterized CTD-ILD admitted to Nanjing Drum Tower Hospital with an AE from January 2010 to December 2016. RESULTS: The study cohort included 107 subjects with AE-IPF and 70 cases with AE-CTD-ILD. Female gender, prior use of corticosteroid and immunosupressants, lower serum albumin, higher D-dimer level, TLC% pred, survival, and treatment with immunosupressants and caspofungin were more common in the CTD-ILD group (all p<0.05). The incidences of AE-CTD-ILD and AE-IPF were similar in our single center (p = 0.526). TLC% pred was the risk factor for AE after ILD diagnosis for 1 year in CTD patients (p = 0.018). Log-rank tests showed patients with CTD-ILD had a significantly lower mortality rate compared with IPF patients after AEs (p = 0.029). No significant difference in survival was noted among CTD subgroups (p = 0.353). The survival was negatively correlated with WBC count, LDH and CT score, (p = 0.006, p = 0.013 and p = 0.035, respectively), and positively correlated with PaO(2)/FiO(2) ratio (p<0.001) in the CTD-ILD group. WBC count and PO(2)/FiO(2) ratio were the independent predictors for survival in AE-CTD-ILD after adjusting for other clinical variates in Cox regression Models (p = 0.038 and p < 0.001, respectively). CONCLUSIONS: The clinical characteristics of patients with AE-CTD-ILD differed from those with AE-IPF, while AE incidences were similar between the two groups. Subjects with AE-CTD-fILD tended to have a better prognosis, and WBC count and PO(2)/FiO(2) ratio were the independent survival predictors for these patients. BioMed Central 2019-11-14 /pmc/articles/PMC6857302/ /pubmed/31727051 http://dx.doi.org/10.1186/s12890-019-0960-1 Text en © The Author(s). 2019 Open AccessThis article is distributed under the terms of the Creative Commons Attribution 4.0 International License (http://creativecommons.org/licenses/by/4.0/), which permits unrestricted use, distribution, and reproduction in any medium, provided you give appropriate credit to the original author(s) and the source, provide a link to the Creative Commons license, and indicate if changes were made. The Creative Commons Public Domain Dedication waiver (http://creativecommons.org/publicdomain/zero/1.0/) applies to the data made available in this article, unless otherwise stated.
spellingShingle Research Article
Cao, Mengshu
Sheng, Jian
Qiu, Xiaohua
Wang, Dandan
Wang, Dongmei
Wang, Yang
Xiao, Yonglong
Cai, Hourong
Acute exacerbations of fibrosing interstitial lung disease associated with connective tissue diseases: a population-based study
title Acute exacerbations of fibrosing interstitial lung disease associated with connective tissue diseases: a population-based study
title_full Acute exacerbations of fibrosing interstitial lung disease associated with connective tissue diseases: a population-based study
title_fullStr Acute exacerbations of fibrosing interstitial lung disease associated with connective tissue diseases: a population-based study
title_full_unstemmed Acute exacerbations of fibrosing interstitial lung disease associated with connective tissue diseases: a population-based study
title_short Acute exacerbations of fibrosing interstitial lung disease associated with connective tissue diseases: a population-based study
title_sort acute exacerbations of fibrosing interstitial lung disease associated with connective tissue diseases: a population-based study
topic Research Article
url https://www.ncbi.nlm.nih.gov/pmc/articles/PMC6857302/
https://www.ncbi.nlm.nih.gov/pubmed/31727051
http://dx.doi.org/10.1186/s12890-019-0960-1
work_keys_str_mv AT caomengshu acuteexacerbationsoffibrosinginterstitiallungdiseaseassociatedwithconnectivetissuediseasesapopulationbasedstudy
AT shengjian acuteexacerbationsoffibrosinginterstitiallungdiseaseassociatedwithconnectivetissuediseasesapopulationbasedstudy
AT qiuxiaohua acuteexacerbationsoffibrosinginterstitiallungdiseaseassociatedwithconnectivetissuediseasesapopulationbasedstudy
AT wangdandan acuteexacerbationsoffibrosinginterstitiallungdiseaseassociatedwithconnectivetissuediseasesapopulationbasedstudy
AT wangdongmei acuteexacerbationsoffibrosinginterstitiallungdiseaseassociatedwithconnectivetissuediseasesapopulationbasedstudy
AT wangyang acuteexacerbationsoffibrosinginterstitiallungdiseaseassociatedwithconnectivetissuediseasesapopulationbasedstudy
AT xiaoyonglong acuteexacerbationsoffibrosinginterstitiallungdiseaseassociatedwithconnectivetissuediseasesapopulationbasedstudy
AT caihourong acuteexacerbationsoffibrosinginterstitiallungdiseaseassociatedwithconnectivetissuediseasesapopulationbasedstudy