학술논문
Survival and acute exacerbation for patients with idiopathic pulmonary fibrosis (IPF) or non-IPF idiopathic interstitial pneumonias: 5-year follow-up analysis of a prospective multi-institutional patient registry
Document Type
article
Author
Hiroshi Ishii; Yoichi Nakanishi; Ryo Torii; Makoto Yoshida; Isamu Okamoto; Masaki Fujita; Kazunori Tobino; Masayuki Kawasaki; Eiji Harada; Takashige Maeyama; Naoki Hamada; Masaki Okamoto; Kazuhiro Yatera; Shohei Takata; Kazuya Tsubouchi; Shoji Tokunaga; Katsuyuki Ichiki; Yasuhiko Kitasato; Satoru Kawakami; Taishi Harada; Hiroshi Wataya; Masashi Komori; Yuichi Mizuta; Hidetake Yabuuchi
Source
BMJ Open Respiratory Research, Vol 10, Iss 1 (2023)
Subject
Language
English
ISSN
2052-4439
Abstract
Objective Few prospective cohort studies with relatively large numbers of patients with non-idiopathic pulmonary fibrosis (non-IPF) of idiopathic interstitial pneumonia (IIP) have been described. We aimed to assess disease progression and cause of death for patients with non-IPF IIPs or IPF under real-life conditions.Methods Data were analysed for a prospective multi-institutional cohort of 528 IIP patients enrolled in Japan between September 2013 and April 2016. Diagnosis of IPF versus non-IPF IIPs was based on central multidisciplinary discussion, and follow-up surveillance was performed for up to 5 years after patient registration. Survival and acute exacerbation (AE) were assessed.Results IPF was the most common diagnosis (58.0%), followed by unclassifiable IIPs (35.8%) and others (6.2%). The 5-year survival rate for non-IPF IIP and IPF groups was 72.8% and 53.7%, respectively, with chronic respiratory failure being the primary cause of death in both groups. AE was the second most common cause of death for both non-IPF IIP (24.1%) and IPF (23.5%) patients. The cumulative incidence of AE did not differ significantly between the two groups (p=0.36), with a 1-year incidence rate of 7.4% and 9.0% in non-IPF IIP and IPF patients, respectively. We found that 30.2% and 39.4% of non-IPF IIP and IPF patients, respectively, who experienced AE died within 3 months after an AE event, whereas 55.8% and 66.7% of such patients, respectively, died within 5 years after registration.Conclusion Closer monitoring of disease progression and palliative care interventions after AE are important for non-IPF IIP patients as well as for IPF patients.