TY - JOUR T1 - Nintedanib for non-IPF progressive pulmonary fibrosis: 12-month outcome data from a real-world multicentre observational study JF - ERJ Open Research JO - erjor DO - 10.1183/23120541.00423-2022 VL - 9 IS - 2 SP - 00423-2022 AU - Lavanya Raman AU - Iain Stewart AU - Shaney L. Barratt AU - Felix Chua AU - Nazia Chaudhuri AU - Anjali Crawshaw AU - Michael Gibbons AU - Charlotte Hogben AU - Rachel Hoyles AU - Vasilis Kouranos AU - Jennifer Martinovic AU - Sarah Mulholland AU - Katherine J. Myall AU - Marium Naqvi AU - Elisabetta A. Renzoni AU - Peter Saunders AU - Matthew Steward AU - Dharmic Suresh AU - Muhunthan Thillai AU - Athol U. Wells AU - Alex West AU - Jane A. Mitchell AU - Peter M. George Y1 - 2023/03/01 UR - http://openres.ersjournals.com/content/9/2/00423-2022.abstract N2 - Background Nintedanib slows lung function decline for patients with non-idiopathic pulmonary fibrosis progressive pulmonary fibrosis (PPF) in clinical trials, but the real-world safety and efficacy are not known.Methods In this retrospective cohort study, standardised data were collected from patients in whom nintedanib was initiated for PPF between 2019 and 2020 through an early-access programme across eight centres in the United Kingdom. Rate of lung function change in the 12 months pre- and post-nintedanib initiation was the primary analysis. Symptoms, drug safety, tolerability and stratification by interstitial lung disease subtype and computed tomography pattern were secondary analyses.Results 126 patients were included; 67 (53%) females; mean±sd age 60±13 years. At initiation of nintedanib, mean forced vital capacity (FVC) was 1.87 L (58% predicted) and diffusing capacity of the lung for carbon monoxide (DLCO) was 32.7% predicted. 68% of patients were prescribed prednisolone (median dose 10 mg) and 69% were prescribed a steroid-sparing agent. In the 12 months after nintedanib initiation, lung function decline was significantly lower than in the preceding 12 months: FVC −88.8 mL versus −239.9 mL (p=0.004), and absolute decline in DLCO −2.1% versus −6.1% (p=0.004). Response to nintedanib was consistent in sensitivity and secondary analyses. 89 (71%) out of 126 patients reported side-effects, but 86 (80%) of the surviving 108 patients were still taking nintedanib at 12 months with patients reporting a reduced perception of symptom decline. There were no serious adverse events.Conclusion In PPF, the real-world efficacy of nintedanib replicated that of clinical trials, significantly attenuating lung function decline. Despite the severity of disease, nintedanib was safe and well tolerated in this real-world multicentre study.In a real-world multicentre observational study of patients with non-IPF progressive pulmonary fibrosis, nintedanib was safe, efficacious and well tolerated, reducing annual lung function decline with a similar magnitude of effect as in clinical trials https://bit.ly/3ElA10n ER -