Treatment Response and Safety Profile of Nintedanib in Connective Tissue Disease-Associated Interstitial Lung Disease: A Retrospective Observational Study.

Objective: Interstitial lung disease (ILD) is a significant clinical complication that can occur in various connective tissue diseases (CTDs). Treatment includes immunosuppressants to reduce inflammation, while the antifibrotic nintedanib has been approved for slowing lung function decline in certain CTD-ILD patients. We aimed to examine the use of nintedanib in a Greek population, offering insight in real-world clinical setting. Methods: Retrospective collection of data from patients with various CTD-ILDs receiving nintedanib, including demographic, clinical and laboratory features. The analysis comprised records of pulmonary function tests (PFTs) conducted at all available time points prior to and following treatment with nintedanib, in addition to assessments of the drug's tolerability and the incidence of significant adverse events. Results: A total of 60 patients with CTD-ILD (43 females, mean age 60.1 ± 11.8, 50% systemic sclerosis, 21.7% rheumatoid arthritis, 13.3% idiopathic inflammatory myopathies) were included. 58 patients (96.6%) received nintedanib in combination with immunosuppressants. The mean baseline FVC% was 66.5 ± 16.4 and the mean FVC% after treatment was 64.1 ± 19.0, showing a downward trend (p=0.090). The presence of other systemic manifestations such as PAH, cardiac involvement, digital ulcers or GI manifestations emerged as a significant predictor in both %change and absolute DLCO difference. Dose reduction occurred in 16 (26.7%) patients while permanent discontinuation only in four patients (6.7%). The most common adverse event was diarrhoea (21.6%). Conclusion: Our real-world data across a broad spectrum of CTD-ILD suggests that nintedanib is associated with disease stabilisation, and is generally well tolerated. It may be beneficial in combination with immunosuppressives in slowing the rate of lung function decline.