This study explored the effectiveness and safety of nintedanib (NTB), a drug approved for progressive fibrosing interstitial lung disease (PF-ILD), in combination with immunosuppressive agents (IS) for treating connective tissue disease-associated PF-ILD (CTD-PF-ILD). Researchers conducted a retrospective analysis of 25 patients treated with NTB at Kagawa University in Japan between 2020 and 2022. They assessed changes in lung function, measured by forced vital capacity (FVC), and levels of a blood marker (KL-6) associated with lung disease activity.

The results showed significant improvement in lung function, with average FVC increasing from 59.1% to 65.3% over 6-12 months, and mean FVC volume rising from 1666.2 mL to 1821.4 mL. Monthly FVC decline slowed notably after starting NTB, indicating better lung health. KL-6 levels dropped significantly, correlating with the observed improvement in lung function. When comparing patients who received both NTB and new IS (Group A) to those who received only NTB (Group B), Group A demonstrated greater improvement in lung function.

In terms of safety, the common side effects were diarrhea, nausea, elevated liver enzymes, and headaches. Some patients had their NTB dose reduced due to side effects, but none discontinued the medication. Overall, the study concluded that NTB, especially when combined with immunosuppressive agents like rituximab, is an effective and safe treatment for CTD-PF-ILD. The reduction in KL-6 levels may also serve as a useful marker for monitoring treatment response.