Efficacy and Safety of Nintedanib in Patients With Progressive Fibrosing Interstitial Lung Disease (PF-ILD)

Summary

The aim of the current study is to investigate the efficacy and safety of nintedanib over 52 weeks in patients with Progressive Fibrosing Interstitial Lung Disease (PF-ILD) defined as patients who present with features of diffuse fibrosing lung disease of \>10% extent on high-resolution computed tomography (HRCT) and whose lung function and respiratory symptoms or chest imaging have worsened despite treatment with unapproved medications used in clinical practice to treat ILD. There is currently no efficacious treatment available for PF-ILD. Based on its efficacy and safety in Idiopathic Pulmonary Fibrosis (IPF), it is anticipated that Nintedanib will be a new treatment option for patients with PF-ILD.

Timeline

Start

2017-01-17

Primary completion

2019-04-23

Completion

2019-08-12

Outcome

Met primary endpoint

registry analysis (superiority test); 150 mg Nintedanib vs Placebo; p <.0001; Adjusted mean difference 106.96 (95% CI 65.42 to 148.50) NCT02999178 ↗

registry analysis (superiority test); 150 mg Nintedanib vs Placebo; p <.0001; Adjusted mean difference 128.20 (95% CI 70.81 to 185.59) NCT02999178 ↗

release “In the Phase III INBUILD® trial Ofev® (nintedanib) slowed lung function decline by 57% across the overall study population” prnewswire.com ↗

Publications

• Walters SH, Soulard S, Ye W, Elsen A, Kongnakorn T, Raj SS, Pimple P, Ferreira PG, Froidure A. Cost-Effectiveness of Earlier Diagnosis of Fibrotic Interstitial Lung Diseases (Fibrotic ILDs) and Earlier Treatment of Its Progressive Form, Progressive Pulmonary Fibrosis (PPF). Adv Ther. 2026 Jul;43(7):3134-3147. doi: 10.1007/s12325-026-03601-8. Epub 2026 May 5.
• Kolb M, Flaherty KR, Silva RS, Prasse A, Vancheri C, Mueller H, Sroka-Saidi K, Wells AU; INBUILD trial investigators. Effect of Nintedanib in Patients with Progressive Pulmonary Fibrosis in Subgroups with Differing Baseline Characteristics. Adv Ther. 2023 Dec;40(12):5536-5546. doi: 10.1007/s12325-023-02668-x. Epub 2023 Sep 26.
• Kreuter M, Bendstrup E, Jouneau S, Maher TM, Inoue Y, Miede C, Lievens D, Crestani B. Weight loss and outcomes in subjects with progressive pulmonary fibrosis: data from the INBUILD trial. Respir Res. 2023 Mar 9;24(1):71. doi: 10.1186/s12931-023-02371-z.
• Inoue Y, Wells AU, Song JW, Xu Z, Kitamura H, Suda T, Okamoto M, Muller H, Coeck C, Rohr KB, Kolb M, Brown KK. Nintedanib in Asian patients with progressive fibrosing interstitial lung diseases: Results from the INBUILD trial. Respirology. 2023 May;28(5):465-474. doi: 10.1111/resp.14452. Epub 2023 Jan 15.
• Ogura T, Suda T, Inase N, Nishioka Y, Azuma A, Okamoto M, Takizawa A, Ito T, Rohr KB, Inoue Y. Effects of nintedanib on disease progression and safety in Japanese patients with progressive fibrosing interstitial lung diseases: Further subset analysis from the whole INBUILD trial. Respir Investig. 2022 Nov;60(6):787-797. doi: 10.1016/j.resinv.2022.06.009. Epub 2022 Aug 1.
• Cottin V, Martinez FJ, Jenkins RG, Belperio JA, Kitamura H, Molina-Molina M, Tschoepe I, Coeck C, Lievens D, Costabel U. Safety and tolerability of nintedanib in patients with progressive fibrosing interstitial lung diseases: data from the randomized controlled INBUILD trial. Respir Res. 2022 Apr 7;23(1):85. doi: 10.1186/s12931-022-01974-2.
• Swigris JJ, Bushnell DM, Rohr K, Mueller H, Baldwin M, Inoue Y. Responsiveness and meaningful change thresholds of the Living with Pulmonary Fibrosis (L-PF) questionnaire Dyspnoea and Cough scores in patients with progressive fibrosing interstitial lung diseases. BMJ Open Respir Res. 2022 Mar;9(1):e001167. doi: 10.1136/bmjresp-2021-001167.
• Inoue Y, Suda T, Kitamura H, Okamoto M, Azuma A, Inase N, Kuwana M, Makino S, Nishioka Y, Ogura T, Takizawa A, Ugai H, Stowasser S, Schlenker-Herceg R, Takeuchi T. Efficacy and safety of nintedanib in Japanese patients with progressive fibrosing interstitial lung diseases: Subgroup analysis of the randomised, double-blind, placebo-controlled, phase 3 INBUILD trial. Respir Med. 2021 Oct;187:106574. doi: 10.1016/j.rmed.2021.106574. Epub 2021 Aug 12.
• Flaherty KR, Wells AU, Cottin V, Devaraj A, Inoue Y, Richeldi L, Walsh SLF, Kolb M, Koschel D, Moua T, Stowasser S, Goeldner RG, Schlenker-Herceg R, Brown KK; INBUILD Trial Investigators. Nintedanib in progressive interstitial lung diseases: data from the whole INBUILD trial. Eur Respir J. 2022 Mar 17;59(3):2004538. doi: 10.1183/13993003.04538-2020. Print 2022 Mar.
• Maher TM, Brown KK, Kreuter M, Devaraj A, Walsh SLF, Lancaster LH, Belloli EA, Padilla M, Behr J, Goeldner RG, Tetzlaff K, Schlenker-Herceg R, Flaherty KR; INBUILD trial investigators. Effects of nintedanib by inclusion criteria for progression of interstitial lung disease. Eur Respir J. 2022 Feb 3;59(2):2004587. doi: 10.1183/13993003.04587-2020. Print 2022 Feb.
• Cottin V, Richeldi L, Rosas I, Otaola M, Song JW, Tomassetti S, Wijsenbeek M, Schmitz M, Coeck C, Stowasser S, Schlenker-Herceg R, Kolb M; INBUILD Trial Investigators. Nintedanib and immunomodulatory therapies in progressive fibrosing interstitial lung diseases. Respir Res. 2021 Mar 16;22(1):84. doi: 10.1186/s12931-021-01668-1.
• Brown KK, Martinez FJ, Walsh SLF, Thannickal VJ, Prasse A, Schlenker-Herceg R, Goeldner RG, Clerisme-Beaty E, Tetzlaff K, Cottin V, Wells AU. The natural history of progressive fibrosing interstitial lung diseases. Eur Respir J. 2020 Jun 25;55(6):2000085. doi: 10.1183/13993003.00085-2020. Print 2020 Jun.
• Wells AU, Flaherty KR, Brown KK, Inoue Y, Devaraj A, Richeldi L, Moua T, Crestani B, Wuyts WA, Stowasser S, Quaresma M, Goeldner RG, Schlenker-Herceg R, Kolb M; INBUILD trial investigators. Nintedanib in patients with progressive fibrosing interstitial lung diseases-subgroup analyses by interstitial lung disease diagnosis in the INBUILD trial: a randomised, double-blind, placebo-controlled, parallel-group trial. Lancet Respir Med. 2020 May;8(5):453-460. doi: 10.1016/S2213-2600(20)30036-9. Epub 2020 Mar 5.
• Flaherty KR, Wells AU, Cottin V, Devaraj A, Walsh SLF, Inoue Y, Richeldi L, Kolb M, Tetzlaff K, Stowasser S, Coeck C, Clerisme-Beaty E, Rosenstock B, Quaresma M, Haeufel T, Goeldner RG, Schlenker-Herceg R, Brown KK; INBUILD Trial Investigators. Nintedanib in Progressive Fibrosing Interstitial Lung Diseases. N Engl J Med. 2019 Oct 31;381(18):1718-1727. doi: 10.1056/NEJMoa1908681. Epub 2019 Sep 29.
• Flaherty KR, Brown KK, Wells AU, Clerisme-Beaty E, Collard HR, Cottin V, Devaraj A, Inoue Y, Le Maulf F, Richeldi L, Schmidt H, Walsh S, Mezzanotte W, Schlenker-Herceg R. Design of the PF-ILD trial: a double-blind, randomised, placebo-controlled phase III trial of nintedanib in patients with progressive fibrosing interstitial lung disease. BMJ Open Respir Res. 2017 Sep 17;4(1):e000212. doi: 10.1136/bmjresp-2017-000212. eCollection 2017.

Drugs

Evaluation	Drug	Modality	Dose	Route
Subject	Nintedanib	Small molecule	150 mg	—

Indications