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. 2023 Mar 23;13(7):1221.
doi: 10.3390/diagnostics13071221.

Efficacy and Safety of Nintedanib in Patients with Connective Tissue Disease-Interstitial Lung Disease (CTD-ILD): A Real-World Single Center Experience

Maria Boutel  1 , Afroditi Boutou  2 , Georgia Pitsiou  3 , Alexandros Garyfallos  1 , Theodoros Dimitroulas  1
Affiliations

Efficacy and Safety of Nintedanib in Patients with Connective Tissue Disease-Interstitial Lung Disease (CTD-ILD): A Real-World Single Center Experience

Maria Boutel et al. Diagnostics (Basel). .

Abstract

Connective Tissue Disease-Interstitial Lung Disease (CTD-ILD) is a severe and fatal manifestation of systemic autoimmune disorders. Therapies rely on immunomodulators but their efficacy in ILD progression remains uncertain. Nintedanib, an antifibrotic agent that slows pulmonary function decline, has been approved for CTD-ILD treatment. The aim of this study was to assess the effectiveness and safety of nintedanib in CTD-ILD patients in a real-world data setting. A single-center, retrospective, and descriptive analysis of CTD-ILD patients treated with nintedanib from June 2019 to November 2022 was performed. The assessment of nintedanib treatment's efficacy was judged solely on the evolution of pulmonary function tests (PFTs), which were evaluated before and after treatment. Twenty-one patients (67% females, median age 64 years (IQR = 9) with CTD-ILD (systemic sclerosis n = 9, rheumatoid arthritis n = 5, dermatomyositis n = 4, juvenile rheumatoid arthritis n = 1, undifferentiated CTD n = 1, interstitial pneumonia with autoimmune features n = 1), 18 of whom were on concomitant immunosuppressives, had a median follow-up period of 10 months (IQR = 5). PFTs before and after treatment did not significantly differ. The mean FVC% difference was +0.9 (sd = 7.6) and the mean DLco% difference was +3.4 (sd = 12.6), suggesting numerical improvement of PFTs. The average percentage change was -0.3% and +7.6% for FVC% and DLco%, respectively, indicating stabilization of lung function. Our real-world data across a broad spectrum of CTD-ILD suggest that nintedanib could be beneficial in combination with immunosuppressives in slowing the rate of lung function decline.

Keywords: CTD-ILD; ILD; connective tissue interstitial lung disease; nintedanib; real-world data.

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Conflict of interest statement

The authors declare no conflict of interest.

Figures

Figure 1
Figure 1
FVC (% pred) before and after nintedanib treatment.
Figure 2
Figure 2
DLCO (% pred) before and after nintedanib treatment. Outliers are marked with a circle and extreme outliers are marked with a star.
Figure 3
Figure 3
Patients’ FVC (% pred) before and after nintedanib treatment.
Figure 4
Figure 4
Patients’ DLco (% pred) before and after nintedanib treatment.
Figure 5
Figure 5
Relationship of CTD-ILD duration and other systemic manifestations.
See this image and copyright information in PMC

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