Respiratory investigation · 2025
Incidence and clinical significance of acute exacerbation of interstitial lung disease in patients with primary Sjögren's syndrome.
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Fujisawa Tomoyuki, Tanaka Yuko, Kamiya Yosuke, Kono Masato, Kato Shinpei, Akiyama Norimichi, Koda Keigo, Matsushima Sayomi, Sumikawa Hiromitsu, Johkoh Takeshi, Inoue Yusuke, Yasui Hideki, Hozumi Hironao, Suzuki Yuzo, Karayama Masato, Furuhashi Kazuki, Enomoto Noriyuki, Inui Naoki, Suda Takafumi
Аннотация
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.
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.