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

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":"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","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":"{\"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}","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

摘要

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。

查看原文本刊更多论文

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

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.