Prognostic significance of antifibrotic agents in idiopathic pulmonary fibrosis after initiation of long-term oxygen therapy

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Mayuko Ishiwari
Yuta Kono
Yuki Togashi
Kenichi Kobayashi
Ryota Kikuchi
Mariko Kogami
Shinji Abe


idiopathic pulmonary fibrosis (IPF), long term oxygen therapy (LTOT), antifibrotic agent


Background and aim: Idiopathic pulmonary fibrosis (IPF) is a fatal and progressive interstitial lung disease with varying degrees of hypoxemia. Long-term oxygen therapy (LTOT) is frequently used to treat hypoxemia, however the prognostic factors for better survival in IPF patients after initiation of LTOT remain unknown. Methods: We retrospectively investigated favorable factors of survival in consecutive 55 IPF patients with chronic respiratory failure who were introduced LTOT. Results: The 6-, 12-, 18-, and 24-month survival rates in IPF patients after introduction of LTOT were 70.9%, 49.0%, 45.2%, and 32.3%, respectively. Univariate analysis demonstrated that low Glasgow Prognostic Score (GPS) (hazard ratio [HR] 0.482, p=0.043) and treatment with antifibrotic agents (HR 0.401, p=0.013) were associated with favorable survival, while multivariate analysis revealed that treatment with antifibrotic agents was the independent predictor (HR 0.449, p=0.032). Moreover, IPF patients treated with antifibrotic agents with LTOT had significantly longer survival than those without antifibrotic agents (p = 0.0106). Conclusion: In IPF patients who were introduced LTOT, treatment with antifibrotic agents was the independent factor for favorable survival. Treatment with antifibrotic agents may improve prognosis of IPF even after initiation of LTOT.

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1. Richeldi L, Collard HR, and Jone MG. Idiopathic pulmonary fibrosis. The Lancet 389;1859-1952:2017. doi: 10.1016/S0140-6736(17)30866-8.
2. Lederer DJ, and Martinez FJ. Idiopathic pulmonary fibrosis. N Engl J Med 378;1811-1823:2018. doi: 10.1056/NEJMra1705751.
3. Bell EC, Cox NS, Goh N, et al. Oxygen therapy for interstitial lung disease: a systematic review. Eur Respir Rev. 2017 Feb 21;26(143):160080. doi: 10.1183/16000617.0080-2016.
4. Kreuter M, Bendstrup E, Russell AM, et al. Palliative care in interstitial lung disease: living well. Lancet Respir Med. 2017 Dec;5(12):968-980. doi: 10.1016/S2213-2600(17)30383-1.
5. Raghu G, Collard HR, Egan JJ, et al. An official ATS/ERS/JRS/ALAT statement: idiopathic pulmonary fibrosis: evidence-based guidelines for diagnosis and management. Am J Respir Crit Care Med. 2011 Mar 15;183(6):788-824. doi: 10.1164/rccm.2009-040GL.
6. Rantala HA, Korpela SL, Lehtimaki, et al. Predictors of impaired survival in subjects with long-term oxygen therapy. Respir Care. 2019 Nov;64(11):1401-1409. doi: 10.4187/respcare.06615.
7. Kikuchi R, Takoi H, Tsuji T, et al. Glasgow Prognostic Score predicts chemotherapy-triggered acute exacerbation-interstitial lung disease in patients with non-small cell lung cancer. Thorac Cancer 12; 667-675: 2021. doi: 10.1111/1759-7714.13792.
8. Kikuchi R, Takoi H, Tsuji T, et al. Glasgow prognostic score for prediction of chemotherapy-triggered acute exacerbation interstitial lung disease in patients with small cell lung cancer. Thorac Cancer 11;1681-1689:2021. doi: 10.1111/1759-7714.13900.
9. Nishikiori H, Chiba H, Lee SH, A modified GAP model for East-Asian populations with idiopathic pulmonary fibrosis. Respir Investig. 58;395-402:2020. doi: 10.1016/j.resinv.2020.04.001.
10. Raghu G, Jardin MR, Myers JL, et al. Diagnosis of idiopathic pulmonary fibrosis. An official ATS/ERS/JRS/ALAT clinical practice guideline. Am J Respir Crit Care Med. 2018 Sep 1;198(5):e44-e68. doi: 10.1164/rccm.201807-1255ST.
11. Ogawa K, Uruga H, Fujii T, et al. Characteristics of non-small-cell lung cancer with interstitial pneumonia: variation in cancer location, histopathology, and frequency of postoperative acute exacerbations in interstitial pneumonia. BMC Pulm Med 20;307: 2020. doi: 10.1186/s12890-020-01347-9.
12. Kanda Y. Investigation of the freely available easy-to-use software 'EZR' for medical statistics. Bone Marrow Transplant 48;452-458:2013. doi: 10.1038/bmt.2012.244.
13. Ley B, Collard HR, and King TE. Clinical courese and prediction of survival in idiopathic pulmonary fibrosis. Am J Crit Care Med 183;431-440:2011. doi: 10.1164/rccm.201006-0894CI.
14. Jouneau S, Crestani B, Thibault R, et al. Analysis of body mass index, weight loss and progression of idiopathic pulmonary fibrosis. Respir Res 21;312:2020. doi: 10.1186/s12931-020-01528-4.
15. Ha YJ, Hur J, Go DJ, et al. Baseline peripheral blood neutrophil-to-lymphocyte ratio could predict survival in patients with adilt polymyositis and dermatpmyositis: a retrospective observational study. PLoS One. 2018 Jan 2;13(1):e0190411. doi: 10.1371/journal.pone.0190411.
16. Jafri SH, Shi R, and Mills G. Advance lung cancer inflammation index (ALI) at diagnosis is a prtognostic marker in patients with metastatic non-small cell lung cancer (NSCLC): a retrospective review. BMC Cancer 13;158:2013. doi: 10.1186/1471-2407-13-158.
17. Li B, Zhang X, Xu G, et al. Serumprealbumin is a prognostic indicator in idiopathic pulmonary fibrosis.Clin Respir J 13;493–498:2019. doi: 10.1111/crj.13050.
18. Sharp C, Adamali HI, and Millar AB. A comparison of published multidimensional indices to predict outcome in idiopathic pulmonary fibrosis ERJ Open Res. 2017 Mar 14;3(1):00096-2016. doi: 10.1183/23120541.00096-2016.
19. Homma S, Bando M, Azuma A, et al. Japanese guideline for the tratment of idiopathic pulmonary fibrosis. Respir Investig. 2018 Jul;56(4):268-291. doi: 10.1016/j.resinv.2018.03.003.
20. Higashiguchi M, Kijima T, Sumikawa H, et al. A retrospective study of prognostic factors in patients with interstitial pneumonia receiving long-term oxygen therapy. Lung 192;729-37:2014. doi: 10.1007/s00408-014-9623-4.
21. Ahmadi Z, Wysham NG, Lundstrom S, et al. End-of-life care in oxygen-dependent ILD compared with lung cancer: a national population-based study. Thorax. 2016 Jun;71(6):510-6. doi: 10.1136/thoraxjnl-2015-207439.
22. Rantala HA, Korpela SL, Lehtimaki et al. Predictors of impaired survival in subjects with long-term oxygen therapy. Respir Care. 2019 Nov;64(11):1401-1409. doi: 10.4187/respcare.06615.
23. Jo HE, Glaspole I, Grainge C, et al. Baseline characteristics of idiopathic pulmonary fibrosis: analysis from the Australian Idiopathic Pulmonary Fibrosis Registry. Eur Respir J. 49; 1601592: 2017. doi: 10.1183/13993003.01592-2016.
24. 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 Jul 28;19(1):141. doi: 10.1186/s12931-018-0845-5.
25. Caminati A, Cassandro R, Torre O, et al. Severe idiopathic pulmonary fibrosis: what can be done? Respir Rev. 2017 Sep 27;26(145):170047. doi: 10.1183/16000617.0047-2017
26. Lee EG, Lee TH, Hong Y, et al. Effects of low-dose pirfenidone on survival and lung function decline in patients with idiopathic pulmonary fibrosis (IPF): results from a real-world study. PLoS One. 2021 Dec 23;16(12):e0261684. doi: 10.1371/journal.pone.0261684.

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