| Title: |
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 |
| Authors: |
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) |
| Publisher Information: |
BMJ Publishing Group, 2023. |
| Publication Year: |
2023 |
| Collection: |
LCC:Medicine
LCC:Diseases of the respiratory system |
| Subject Terms: |
Medicine, Diseases of the respiratory system, RC705-779 |
| More Details: |
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. |
| Document Type: |
article |
| File Description: |
electronic resource |
| Language: |
English |
| ISSN: |
2052-4439 |
| Relation: |
https://bmjopenrespres.bmj.com/content/10/1/e001864.full; https://doaj.org/toc/2052-4439 |
| DOI: |
10.1136/bmjresp-2023-001864 |
| Access URL: |
https://doaj.org/article/da0f29146c624bcfb66096242d58ed0d |
| Accession Number: |
edsdoj.0f29146c624bcfb66096242d58ed0d |
| Database: |
Directory of Open Access Journals |
