Nintedanib for patients with systemic autoimmune diseases associated pulmonary fibrosis: a real-world multicentre study.

<bold>Background:</bold> The antifibrotic agent nintedanib is currently approved for patients with interstitial lung disease, apart from idiopathic pulmonary fibrosis. It is considered to reduce lung function decline in patients with a progressive fibrotic phenotype, but real-world safety and efficacy data remain limited. <bold>Methods:</bold> In this retrospective study, data was collected from 5 centres in 3 European countries. Pulmonary function tests were analysed before and after nintedanib initiation. Drug safety and tolerability were also evaluated. <bold>Results:</bold> 65 patients were initially included; however, 6 patients discontinued the drug in less than 6 months, 5 patients died during the first year, and 10 patients received the drug less than one year. Drug tolerability was overall well; after 6 months, only 1 patient had to discontinue the drug, while 23 patients had no side effects and continued the drug for at least 1 year. 53% of patients were also prescribed prednisolone at inclusion, while 71% a steroid-sparing agent. Mean age of the patients included was 63±12 years. At nintedanib initiation, mean FVC was 71%, and DLco 46%pred. 1 year later, mean FVC was 75%, while mean DLco remained 46%pred. Lung function decline was lower during the first year after drug initiation, than in the preceding year. <bold>Conclusion:</bold> Our multicentre real-world study supports that nintedanib was safe and well tolerated in patients with systemic autoimmune diseases related pulmonary fibrosis, the majority of whom were also receiving immunomodulation at inclusion. Our results also suggest that nintedanib could be beneficial in slowing disease progression.