Incidence and clinical significance of acute exacerbation of interstitial lung disease in patients with primary Sjögren's syndrome

IF 2.4 Q2 RESPIRATORY SYSTEM

Respiratory investigation Pub Date : 2025-07-04 DOI:10.1016/j.resinv.2025.07.001

Tomoyuki Fujisawa , Yuko Tanaka , Yosuke Kamiya , Masato Kono , Shinpei Kato , Norimichi Akiyama , Keigo Koda , Sayomi Matsushima , Hiromitsu Sumikawa , Takeshi Johkoh , Yusuke Inoue , Hideki Yasui , Hironao Hozumi , Yuzo Suzuki , Masato Karayama , Kazuki Furuhashi , Noriyuki Enomoto , Naoki Inui , Takafumi Suda

{"title":"Incidence and clinical significance of acute exacerbation of interstitial lung disease in patients with primary Sjögren's syndrome","authors":"Tomoyuki Fujisawa , Yuko Tanaka , Yosuke Kamiya , Masato Kono , Shinpei Kato , Norimichi Akiyama , Keigo Koda , Sayomi Matsushima , Hiromitsu Sumikawa , Takeshi Johkoh , Yusuke Inoue , Hideki Yasui , Hironao Hozumi , Yuzo Suzuki , Masato Karayama , Kazuki Furuhashi , Noriyuki Enomoto , Naoki Inui , Takafumi Suda","doi":"10.1016/j.resinv.2025.07.001","DOIUrl":null,"url":null,"abstract":"<div><h3>Background</h3><div>Acute exacerbation (AE) is defined as acute deterioration in the respiratory status that can lead to a fatal outcome. It may occur in various types of interstitial lung disease (ILD). However, its incidence and clinical picture in patients with primary Sjögren's syndrome-associated interstitial lung disease (pSjS-ILD) remain unclear. Therefore, this study aimed to examine the incidence and clinical significance of AE in patients with pSjS-ILD.</div></div><div><h3>Methods</h3><div>This study included 101 consecutive patients with pSjS-ILD. The clinical findings at diagnosis, AE development, and outcome were analyzed. Furthermore, the prognostic significance of AE was evaluated via Cox proportional-hazards analysis with time-dependent covariates. The Fine–Gray subdistribution hazard model was employed to identify factors associated with AE development.</div></div><div><h3>Results</h3><div>Of the 101 patients, 17 (17 %) developed AE during their clinical course. The cumulative incidence rates of AE were 2.0 %, 11.2 %, and 18.1 % at 1, 5, and 10 years, respectively. Multivariate analysis revealed that AE development was significantly associated with poor prognosis (hazard ratio: 3.69; <em>P</em> = 0.01) as well as age, male sex, and %FVC. Moreover, the serum levels of surfactant protein-D at diagnosis were significantly associated with AE development. Of the 17 patients demonstrating AE, 8 died, with a 60-day post-AE mortality rate of 47 %. The age and serum C-reactive protein level at AE onset were significantly associated with 60-day mortality in patients with AE of pSjS-ILD.</div></div><div><h3>Conclusion</h3><div>AE occurs with a certain frequency and is strongly associated with poor outcome in patients with pSjS-ILD.</div></div>","PeriodicalId":20934,"journal":{"name":"Respiratory investigation","volume":"63 5","pages":"Pages 838-843"},"PeriodicalIF":2.4000,"publicationDate":"2025-07-04","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":"0","resultStr":null,"platform":"Semanticscholar","paperid":null,"PeriodicalName":"Respiratory investigation","FirstCategoryId":"1085","ListUrlMain":"https://www.sciencedirect.com/science/article/pii/S2212534525001029","RegionNum":0,"RegionCategory":null,"ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":null,"EPubDate":"","PubModel":"","JCR":"Q2","JCRName":"RESPIRATORY SYSTEM","Score":null,"Total":0}

引用次数: 0

Abstract

Background

Acute exacerbation (AE) is defined as acute deterioration in the respiratory status that can lead to a fatal outcome. It may occur in various types of interstitial lung disease (ILD). However, its incidence and clinical picture in patients with primary Sjögren's syndrome-associated interstitial lung disease (pSjS-ILD) remain unclear. Therefore, this study aimed to examine the incidence and clinical significance of AE in patients with pSjS-ILD.

Methods

This study included 101 consecutive patients with pSjS-ILD. The clinical findings at diagnosis, AE development, and outcome were analyzed. Furthermore, the prognostic significance of AE was evaluated via Cox proportional-hazards analysis with time-dependent covariates. The Fine–Gray subdistribution hazard model was employed to identify factors associated with AE development.

Results

Of the 101 patients, 17 (17 %) developed AE during their clinical course. The cumulative incidence rates of AE were 2.0 %, 11.2 %, and 18.1 % at 1, 5, and 10 years, respectively. Multivariate analysis revealed that AE development was significantly associated with poor prognosis (hazard ratio: 3.69; P = 0.01) as well as age, male sex, and %FVC. Moreover, the serum levels of surfactant protein-D at diagnosis were significantly associated with AE development. Of the 17 patients demonstrating AE, 8 died, with a 60-day post-AE mortality rate of 47 %. The age and serum C-reactive protein level at AE onset were significantly associated with 60-day mortality in patients with AE of pSjS-ILD.

Conclusion

AE occurs with a certain frequency and is strongly associated with poor outcome in patients with pSjS-ILD.

查看原文本刊更多论文

原发性Sjögren综合征患者间质性肺病急性加重的发生率及临床意义

背景：急性加重（AE）被定义为可导致致命结果的呼吸状态急性恶化。它可发生在各种类型的间质性肺疾病（ILD）。然而，其在原发性Sjögren综合征相关间质性肺疾病（pSjS-ILD）患者中的发病率和临床表现尚不清楚。因此，本研究旨在探讨AE在pSjS-ILD患者中的发生率及临床意义。方法本研究纳入101例pSjS-ILD患者。分析诊断时的临床表现、AE的发展和结局。此外，通过含时间相关协变量的Cox比例风险分析评估AE的预后意义。采用Fine-Gray亚分布风险模型识别与AE发展相关的因素。结果101例患者中，17例（17%）在临床过程中发生AE。AE的累积发病率在1年、5年和10年分别为2.0%、11.2%和18.1%。多因素分析显示AE的发生与不良预后显著相关(风险比：3.69；P = 0.01)、年龄、男性性别、FVC %。此外，诊断时血清表面活性蛋白d水平与AE的发展有显著相关性。在17例出现AE的患者中，8例死亡，AE后60天死亡率为47%。AE发病时的年龄和血清c反应蛋白水平与AE合并pSjS-ILD患者60天死亡率显著相关。结论ae在pSjS-ILD患者中有一定的发生率，且与预后不良密切相关。

本文章由计算机程序翻译，如有差异，请以英文原文为准。

求助全文

约1分钟内获得全文求助全文

来源期刊