BMC Pulmonary Medicine (Oct 2022)

Frequency, proportion of PF-ILD, and prognostic factors in patients with acute exacerbation of ILD related to systemic autoimmune diseases

  • Noriyuki Enomoto,
  • Hyogo Naoi,
  • Yasutaka Mochizuka,
  • Takuya Isayama,
  • Yuko Tanaka,
  • Atsuki Fukada,
  • Yuya Aono,
  • Mineo Katsumata,
  • Hideki Yasui,
  • Kazutaka Mori,
  • Masato Karayama,
  • Hironao Hozumi,
  • Yuzo Suzuki,
  • Kazuki Furuhashi,
  • Tomoyuki Fujisawa,
  • Naoki Inui,
  • Yutaro Nakamura,
  • Takafumi Suda

DOI
https://doi.org/10.1186/s12890-022-02197-3
Journal volume & issue
Vol. 22, no. 1
pp. 1 – 10

Abstract

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Abstract 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%).

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