Skip to main page content
U.S. flag

An official website of the United States government

Dot gov

The .gov means it’s official.
Federal government websites often end in .gov or .mil. Before sharing sensitive information, make sure you’re on a federal government site.

Https

The site is secure.
The https:// ensures that you are connecting to the official website and that any information you provide is encrypted and transmitted securely.

Access keys NCBI Homepage MyNCBI Homepage Main Content Main Navigation
. 2023 Nov;10(1):e001864.
doi: 10.1136/bmjresp-2023-001864.

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

Kazuya Tsubouchi  1 , Naoki Hamada  2 , Shoji Tokunaga  3 , Katsuyuki Ichiki  4 , Shohei Takata  5 , Hiroshi Ishii  6 , Yasuhiko Kitasato  7 , Masaki Okamoto  8 , Satoru Kawakami  9 , Kazuhiro Yatera  10 , Masayuki Kawasaki  11 , Masaki Fujita  2 , Makoto Yoshida  12 , Takashige Maeyama  13 , Taishi Harada  14 , Hiroshi Wataya  15 , Ryo Torii  16 , Masashi Komori  17 , Yuichi Mizuta  18 , Kazunori Tobino  19 , Eiji Harada  20 , Hidetake Yabuuchi  21 , Yoichi Nakanishi  22 , Isamu Okamoto  23
Affiliations

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

Kazuya Tsubouchi et al. BMJ Open Respir Res. 2023 Nov.

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.

Keywords: Clinical Epidemiology; Interstitial Fibrosis.

PubMed Disclaimer

Conflict of interest statement

Competing interests: Yes.

Figures

Figure 1
Figure 1
Flow diagram for the study. COPD, chronic obstructive lung disease; MDD, multidisciplinary discussion.
Figure 2
Figure 2
Diagnosis for the study patients after multidisciplinary discussion. COP, cryptogenic organising pneumonia; DIP, desquamative interstitial pneumonia; IIPs, idiopathic interstitial pneumonias; IPF, idiopathic pulmonary fibrosis; NSIP, non-specific interstitial pneumonia; PPFE, pleuroparenchymal fibroelastosis.
Figure 3
Figure 3
Kaplan-Meier plots of survival probability from the time of registration for patients with idiopathic pulmonary fibrosis (IPF) or non-IPF idiopathic interstitial pneumonias (IIPs). The number of deaths was 132 for IPF and 54 for non-IPF IIPs, and the number of person-years was 1071.4 and 870.1, respectively.
Figure 4
Figure 4
Causes of death in patients with idiopathic pulmonary fibrosis (IPF) or non-IPF idiopathic interstitial pneumonias (IIPs).
Figure 5
Figure 5
Kaplan-Meier plots of survival probability according to risk factors for mortality in patients with idiopathic pulmonary fibrosis (IPF) or non-IPF idiopathic interstitial pneumonias (IIPs). (A) Forced vital capacity (FVC, % predicted) at registration. (B) Modified Medical Research Council (mMRC) score at registration. (C) Age at registration. (D) Relative decline in FVC (% predicted) per year.
Figure 6
Figure 6
Kaplan-Meier plots for the cumulative incidence of acute exacerbation in patients with idiopathic pulmonary fibrosis (IPF) or non-IPF idiopathic interstitial pneumonias (IIPs).
See this image and copyright information in PMC

Similar articles

See all similar articles

Cited by

See all "Cited by" articles

References

    1. Travis WD, Costabel U, Hansell DM, et al. An official American thoracic society/European respiratory society statement: update of the international multidisciplinary classification of the idiopathic interstitial pneumonias. Am J Respir Crit Care Med 2013;188:733–48. 10.1164/rccm.201308-1483ST - DOI - PMC - PubMed
    1. Raghu G, Remy-Jardin M, Richeldi L, et al. Idiopathic pulmonary fibrosis (an update) and progressive pulmonary fibrosis in adults: an official ATS/ERS/JRS/ALAT clinical practice guideline. Am J Respir Crit Care Med 2022;205:e18–47. 10.1164/rccm.202202-0399ST - DOI - PMC - PubMed
    1. Guenther A, Krauss E, Tello S, et al. The European IPF Registry (eurIPFreg): baseline characteristics and survival of patients with idiopathic pulmonary fibrosis. Respir Res 2018;19:141. 10.1186/s12931-018-0845-5 - DOI - PMC - PubMed
    1. Gao J, Kalafatis D, Carlson L, et al. Baseline characteristics and survival of patients of idiopathic pulmonary fibrosis: a longitudinal analysis of the swedish IPF registry. Respir Res 2021;22:40. 10.1186/s12931-021-01634-x - DOI - PMC - PubMed
    1. Behr J, Prasse A, Wirtz H, et al. Survival and course of lung function in the presence or absence of antifibrotic treatment in patients with idiopathic pulmonary fibrosis: long-term results of the INSIGHTS-IPF Registry. Eur Respir J 2020;56:1902279. 10.1183/13993003.02279-2019 - DOI - PubMed

Publication types

MeSH terms