Nintedanib combined with immunosuppressive agents improves forced vital capacity in connective tissue disease-associated PF-ILD: a single-center study

In 2020, Nintedanib (NTB), a tyrosine kinase inhibitor, was the first drug approved worldwide for treating progressive fibrosing interstitial lung disease (PF-ILD). This study evaluated the efficacy and safety of NTB in Japanese patients with CTD-associated PF-ILD in a real-world setting, as there a...

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Published in:BMC rheumatology 2024-06, Vol.8 (1), p.27-9
Main Authors: Ushio, Yusuke, Wakiya, Risa, Kameda, Tomohiro, Nakashima, Shusaku, Shimada, Hiromi, Miyagi, Taichi, Sugihara, Koichi, Mino, Rina, Mizusaki, Mao, Chujo, Kanako, Kagawa, Ryoko, Yamaguchi, Hayamasa, Kadowaki, Norimitsu, Dobashi, Hiroaki
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Language:English
Subjects:
Antifibrotic therapy
Connective tissue disease (CTD)
Interstitial lung disease (ILD)
Nintedanib
Progressive fibrosing interstitial lung disease (PF-ILD)
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container_title BMC rheumatology
container_volume 8
creator Ushio, Yusuke
Wakiya, Risa
Kameda, Tomohiro
Nakashima, Shusaku
Shimada, Hiromi
Miyagi, Taichi
Sugihara, Koichi
Mino, Rina
Mizusaki, Mao
Chujo, Kanako
Kagawa, Ryoko
Yamaguchi, Hayamasa
Kadowaki, Norimitsu
Dobashi, Hiroaki
description In 2020, Nintedanib (NTB), a tyrosine kinase inhibitor, was the first drug approved worldwide for treating progressive fibrosing interstitial lung disease (PF-ILD). This study evaluated the efficacy and safety of NTB in Japanese patients with CTD-associated PF-ILD in a real-world setting, as there are few reports on this topic. We also evaluated the efficacy and safety of combination therapy with NTB and immunosuppressive agents (IS). CTD-associated PF-ILD patients receiving NTB at our institution were included in this retrospective study. To evaluate the efficacy and safety of NTB, we investigated changes in forced vital capacity (FVC) (%), diffusing capacity for carbon monoxide (DLCO) (%), monthly change in FVC (%/month), serum Krebs von den Lungen-6 (KL-6) levels (U/mL) before and after NTB treatment, and adverse events (AEs) during NTB treatment. Moreover, to evaluate the efficacy of the NTB + IS combination therapy, we divided the patients into two groups: one received only NTB (NTB group), and the other received both NTB and IS (NTB + IS group) following the diagnosis of CTD-associated PF-ILD. We analyzed the differences in the changes of these variables between the two groups. Twenty-six patients with CTD-associated PF-ILD were included. After NTB treatment, there were no significant deteriorations in FVC (%) and DLCO (%), while the monthly change in FVC (%/month) significantly increased (p 
doi_str_mv 10.1186/s41927-024-00400-y
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This study evaluated the efficacy and safety of NTB in Japanese patients with CTD-associated PF-ILD in a real-world setting, as there are few reports on this topic. We also evaluated the efficacy and safety of combination therapy with NTB and immunosuppressive agents (IS). CTD-associated PF-ILD patients receiving NTB at our institution were included in this retrospective study. To evaluate the efficacy and safety of NTB, we investigated changes in forced vital capacity (FVC) (%), diffusing capacity for carbon monoxide (DLCO) (%), monthly change in FVC (%/month), serum Krebs von den Lungen-6 (KL-6) levels (U/mL) before and after NTB treatment, and adverse events (AEs) during NTB treatment. Moreover, to evaluate the efficacy of the NTB + IS combination therapy, we divided the patients into two groups: one received only NTB (NTB group), and the other received both NTB and IS (NTB + IS group) following the diagnosis of CTD-associated PF-ILD. 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subjects Antifibrotic therapy
Connective tissue disease (CTD)
Interstitial lung disease (ILD)
Nintedanib
Progressive fibrosing interstitial lung disease (PF-ILD)
title Nintedanib combined with immunosuppressive agents improves forced vital capacity in connective tissue disease-associated PF-ILD: a single-center study
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