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Frequency, proportion of PF-ILD, and prognostic factors in patients with acute exacerbation of ILD related to systemic autoimmune diseases
BACKGROUND: Acute exacerbation (AE) of systemic autoimmune disease-related interstitial lung diseases (SAID-ILD) is less common than AE of idiopathic pulmonary fibrosis (IPF) and the details of AE-SAID-ILD have not been elucidated, but the prognosis is similarly devastating. This study was undertake...
Autores principales: | , , , , , , , , , , , , , , , , , |
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Formato: | Online Artículo Texto |
Lenguaje: | English |
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BioMed Central
2022
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Materias: | |
Acceso en línea: | https://www.ncbi.nlm.nih.gov/pmc/articles/PMC9608932/ https://www.ncbi.nlm.nih.gov/pubmed/36289542 http://dx.doi.org/10.1186/s12890-022-02197-3 |
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author | Enomoto, Noriyuki Naoi, Hyogo Mochizuka, Yasutaka Isayama, Takuya Tanaka, Yuko Fukada, Atsuki Aono, Yuya Katsumata, Mineo Yasui, Hideki Mori, Kazutaka Karayama, Masato Hozumi, Hironao Suzuki, Yuzo Furuhashi, Kazuki Fujisawa, Tomoyuki Inui, Naoki Nakamura, Yutaro Suda, Takafumi |
author_facet | Enomoto, Noriyuki Naoi, Hyogo Mochizuka, Yasutaka Isayama, Takuya Tanaka, Yuko Fukada, Atsuki Aono, Yuya Katsumata, Mineo Yasui, Hideki Mori, Kazutaka Karayama, Masato Hozumi, Hironao Suzuki, Yuzo Furuhashi, Kazuki Fujisawa, Tomoyuki Inui, Naoki Nakamura, Yutaro Suda, Takafumi |
author_sort | Enomoto, Noriyuki |
collection | PubMed |
description | BACKGROUND: Acute exacerbation (AE) of systemic autoimmune disease-related interstitial lung diseases (SAID-ILD) is less common than AE of idiopathic pulmonary fibrosis (IPF) and the details of AE-SAID-ILD have not been elucidated, but the prognosis is similarly devastating. This study was undertaken to determine the incidences of AE-ILD in each SAID and to elucidate the proportion of progressive fibrosing (PF)-ILD in AE-SAID-ILD. METHODS: We retrospectively analysed data for patients with SAID-ILD who were diagnosed and observed at our hospital between 1999 and 2020. RESULTS: Two hundred and thirty-two patients with SAID-ILD were enrolled, with a mean observation period of 100.2 months. AE-SAID-ILD was found in 25 patients (10.78%), mainly in patients with RA (17 patients, 68%) and elderly male patients with a smoking history. The overall incidence of AE-SAID-ILD was 1.29%/person-year, and the incidence for each SAID was as follows: RA 2.193, microscopic polyarteritis (MPA) 3.203, systemic sclerosis (SSc) 2.277, primary Sjögren syndrome 0.426, and polymyositis/dermatomyositis 0.222. The incidence of AE of RA/MPA/SSc-ILD was significantly higher than that of other AE-SAID-ILD (p < 0.001). Five of 25 patients (20%) fulfilled the criteria for PF-ILD. The 90-day survival rate was 48.0%, and a higher neutrophil count at AE (HR 13.27, 95%CI 2.447–246, p = 0.001) and early commencement of long-duration direct haemoperfusion with a polymyxin B-immobilised fibre column (HR 0.105, 95%CI 0.005–0.858, p = 0.035) were significant prognostic factors. CONCLUSIONS: The incidence of AE-SAID-ILD was significantly higher in patients with RA, MPA, or SSc than in patients with other SAID. Furthermore, even in patients with AE-SAID-ILD, the proportion of PF-ILD just before AE was not high (20%). SUPPLEMENTARY INFORMATION: The online version contains supplementary material available at 10.1186/s12890-022-02197-3. |
format | Online Article Text |
id | pubmed-9608932 |
institution | National Center for Biotechnology Information |
language | English |
publishDate | 2022 |
publisher | BioMed Central |
record_format | MEDLINE/PubMed |
spelling | pubmed-96089322022-10-28 Frequency, proportion of PF-ILD, and prognostic factors in patients with acute exacerbation of ILD related to systemic autoimmune diseases Enomoto, Noriyuki Naoi, Hyogo Mochizuka, Yasutaka Isayama, Takuya Tanaka, Yuko Fukada, Atsuki Aono, Yuya Katsumata, Mineo Yasui, Hideki Mori, Kazutaka Karayama, Masato Hozumi, Hironao Suzuki, Yuzo Furuhashi, Kazuki Fujisawa, Tomoyuki Inui, Naoki Nakamura, Yutaro Suda, Takafumi BMC Pulm Med Research BACKGROUND: Acute exacerbation (AE) of systemic autoimmune disease-related interstitial lung diseases (SAID-ILD) is less common than AE of idiopathic pulmonary fibrosis (IPF) and the details of AE-SAID-ILD have not been elucidated, but the prognosis is similarly devastating. This study was undertaken to determine the incidences of AE-ILD in each SAID and to elucidate the proportion of progressive fibrosing (PF)-ILD in AE-SAID-ILD. METHODS: We retrospectively analysed data for patients with SAID-ILD who were diagnosed and observed at our hospital between 1999 and 2020. RESULTS: Two hundred and thirty-two patients with SAID-ILD were enrolled, with a mean observation period of 100.2 months. AE-SAID-ILD was found in 25 patients (10.78%), mainly in patients with RA (17 patients, 68%) and elderly male patients with a smoking history. The overall incidence of AE-SAID-ILD was 1.29%/person-year, and the incidence for each SAID was as follows: RA 2.193, microscopic polyarteritis (MPA) 3.203, systemic sclerosis (SSc) 2.277, primary Sjögren syndrome 0.426, and polymyositis/dermatomyositis 0.222. The incidence of AE of RA/MPA/SSc-ILD was significantly higher than that of other AE-SAID-ILD (p < 0.001). Five of 25 patients (20%) fulfilled the criteria for PF-ILD. The 90-day survival rate was 48.0%, and a higher neutrophil count at AE (HR 13.27, 95%CI 2.447–246, p = 0.001) and early commencement of long-duration direct haemoperfusion with a polymyxin B-immobilised fibre column (HR 0.105, 95%CI 0.005–0.858, p = 0.035) were significant prognostic factors. CONCLUSIONS: The incidence of AE-SAID-ILD was significantly higher in patients with RA, MPA, or SSc than in patients with other SAID. Furthermore, even in patients with AE-SAID-ILD, the proportion of PF-ILD just before AE was not high (20%). SUPPLEMENTARY INFORMATION: The online version contains supplementary material available at 10.1186/s12890-022-02197-3. BioMed Central 2022-10-26 /pmc/articles/PMC9608932/ /pubmed/36289542 http://dx.doi.org/10.1186/s12890-022-02197-3 Text en © The Author(s) 2022 https://creativecommons.org/licenses/by/4.0/Open AccessThis article is licensed under a Creative Commons Attribution 4.0 International License, which permits use, sharing, adaptation, distribution and reproduction in any medium or format, as long as you give appropriate credit to the original author(s) and the source, provide a link to the Creative Commons licence, and indicate if changes were made. The images or other third party material in this article are included in the article's Creative Commons licence, unless indicated otherwise in a credit line to the material. If material is not included in the article's Creative Commons licence and your intended use is not permitted by statutory regulation or exceeds the permitted use, you will need to obtain permission directly from the copyright holder. To view a copy of this licence, visit http://creativecommons.org/licenses/by/4.0/ (https://creativecommons.org/licenses/by/4.0/) . The Creative Commons Public Domain Dedication waiver (http://creativecommons.org/publicdomain/zero/1.0/ (https://creativecommons.org/publicdomain/zero/1.0/) ) applies to the data made available in this article, unless otherwise stated in a credit line to the data. |
spellingShingle | Research Enomoto, Noriyuki Naoi, Hyogo Mochizuka, Yasutaka Isayama, Takuya Tanaka, Yuko Fukada, Atsuki Aono, Yuya Katsumata, Mineo Yasui, Hideki Mori, Kazutaka Karayama, Masato Hozumi, Hironao Suzuki, Yuzo Furuhashi, Kazuki Fujisawa, Tomoyuki Inui, Naoki Nakamura, Yutaro Suda, Takafumi Frequency, proportion of PF-ILD, and prognostic factors in patients with acute exacerbation of ILD related to systemic autoimmune diseases |
title | Frequency, proportion of PF-ILD, and prognostic factors in patients with acute exacerbation of ILD related to systemic autoimmune diseases |
title_full | Frequency, proportion of PF-ILD, and prognostic factors in patients with acute exacerbation of ILD related to systemic autoimmune diseases |
title_fullStr | Frequency, proportion of PF-ILD, and prognostic factors in patients with acute exacerbation of ILD related to systemic autoimmune diseases |
title_full_unstemmed | Frequency, proportion of PF-ILD, and prognostic factors in patients with acute exacerbation of ILD related to systemic autoimmune diseases |
title_short | Frequency, proportion of PF-ILD, and prognostic factors in patients with acute exacerbation of ILD related to systemic autoimmune diseases |
title_sort | frequency, proportion of pf-ild, and prognostic factors in patients with acute exacerbation of ild related to systemic autoimmune diseases |
topic | Research |
url | https://www.ncbi.nlm.nih.gov/pmc/articles/PMC9608932/ https://www.ncbi.nlm.nih.gov/pubmed/36289542 http://dx.doi.org/10.1186/s12890-022-02197-3 |
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