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

• 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
• Format: Article
• Language: English
• Subjects: Antifibrotic therapy; Connective tissue disease (CTD); Interstitial lung disease (ILD); Nintedanib; Progressive fibrosing interstitial lung disease (PF-ILD)
• ISSN: 2520-1026; 2520-1026
• DOI: 10.1186/s41927-024-00400-y

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