Nintedanib for non-IPF progressive pulmonary fibrosis: 12-month outcome data from a real-world multicentre observational study

Lavanya Raman, Iain Stewart, Shaney L Barratt, Felix Chua, Nazia Chaudhuri, Anjali Crawshaw, Michael Gibbons, Charlotte Hogben, Rachel Hoyles, Vasilis Kouranos, Jennifer Martinovic, Sarah Mulholland, Katherine J Myall, Marium Naqvi, Elisabetta A Renzoni, Peter Saunders, Matthew Steward, Dharmic Suresh, Muhunthan Thillai, Athol U WellsAlex West, Jane A Mitchell, Peter M George

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Background Nintedanib slows lung function decline for patients with non-IPF 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 was collected across 8 UK centres from patients in whom nintedanib was initiated for PPF between 2019 and 2020 through an early access programme. 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 (ILD) subtype and CT pattern were secondary analyses.

Results 126 patients were included; 67(53%) females, mean age 60(±13) years. At initiation of nintedanib, mean FVC was 1.87 L (58%) and DLco 32.7% predicted. 68% of patients were prescribed prednisolone (median dose 10 mg) and 69% 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 respectively, (p=0.004) and absolute decline in DLco −2.1% versus −6.1% respectively; (p=0.004). Response to nintedanib was consistent in sensitivity and secondary analyses. 89/126 (71%) of patients reported side effects but 86 of the surviving 108 patients (80%) 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.
Original languageEnglish
Article numberERJOR-00423-2022.R1
JournalERJ Open Research
Early online date3 Jan 2023
Publication statusPublished online - 3 Jan 2023

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