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

Autor: 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
Jazyk: angličtina
Rok vydání: 2022
Předmět:
Zdroj: BMC Pulmonary Medicine, Vol 22, Iss 1, Pp 1-10 (2022)
Druh dokumentu: article
ISSN: 1471-2466
DOI: 10.1186/s12890-022-02197-3
Popis: 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
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