Abstract
Various connective tissue diseases tend to affect specific organs, lungs being the organ with the most serious repercussions and consequences. The diagnosis of interstitial lung disease makes the treatment more difficult and worsens long-term prognosis and overall survival. Positive results from the registration studies of nintedanib led to approval of the drug for the treatment of idiopathic pulmonary fibrosis and chronic fibrosing interstitial lung diseases in connective tissue diseases. After registration, real-world data on the use of nintedanib are being collected in everyday clinical practise. The objective of the study was to collect and analyse real world experience gathered after the registration of nintedanib for the treatment of CTD-ILD and to show if the positive results collected from a homogeneous and “representative” study population can be applied to everyday clinical practice. We are presenting a retrospective observational case-series study of patients treated with nintedanib from the three largest Croatian centers specialised in the treatment of connective tissue diseases with interstitial lung diseases. Stabilisation or improved of lung function tests was reported in 68% of patients when changes in predicted FVC were observed and in 72% of patients when changes in DLco were analysed. Almost all of the reported patients (98%) were treated with nintedanib as an add-on drug to immunosuppressants. The most common side-effects were gastrointestinal symptoms and abnormal liver function tests in less extent. Our real-world data confirm the tolerability, efficacy and similar side-effects of nintedanib as reported in pivotal trials.
Key Points • Interstitial lung disease is a common manifestation of several connective tissue diseases and its progressive fibrosing phenotype contributes to high mortality rate and many unmet needs regarding the treatment remain. • Registration studies of nintedanib obtained sufficient data and positive results to support approval of the drug. • Real-world evidence from our CTD-ILD centres confirm the clinical trial data regarding efficacy, tolerability and safety of nintedanib. |
Data availability
The data from this study are available from the corresponding author upon reasonable request.
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The authors express their gratitude to the members of Multidisciplinary teams for interstitial lung diseases.
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We declare that this manuscript is original, has not been published before, and is not currently being considered for publication elsewhere. We have no conflicts of interest associated with this publication and there has been no financial support for this work that could have influenced its outcome. As the corresponding author, I confirm that the manuscript has been read and approved for submission by all named authors with subsequent modifications. All authors approved the final manuscript as submitted and agree to be accountable for all aspects of the work.
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Barešić, M., Novak, S., Perković, D. et al. Real world experience with nintedanib in connective tissue disease-related interstitial lung disease: a retrospective cohort study. Clin Rheumatol 42, 2897–2903 (2023). https://doi.org/10.1007/s10067-023-06689-3
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DOI: https://doi.org/10.1007/s10067-023-06689-3