ArticleBMJ open2025
Characterisation of patients with antifibrotic-treated non-idiopathic pulmonary fibrosis progressive pulmonary fibrosis: a retrospective real-life study.
Article in BMJ open, 2025. The graph could read no effect estimate from its abstract, so it casts no vote on the map. Cited by 2 papers.
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Who cites it
2 citing papers in PubMed.
- Combination antifibrotic and immunosuppressive therapy in progressive fibrosing ILD.Sarcoidosis, vasculitis, and diffuse lung diseases : official journal of WASOG · 2026Article
- A seven-gene panel with diagnostic and prognostic value for idiopathic pulmonary fibrosis and candidate herbal drugs identified based on these genes.International journal of clinical and experimental pathology · 2026Article
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Authors and funding
6 authors.
Funding
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Abstract
objectiveNintedanib is approved for the treatment of interstitial lung disease (ILD) with progressive pulmonary fibrosis (PPF). Real-life data on nintedanib treatment for this indication and on the combined use of antifibrotic and immunomodulatory drugs are limited. This study was aimed to collect detailed real-life data from all patients with non-idiopathic pulmonary fibrosis (IPF) PPF at the Hospital District of Helsinki and Uusimaa to identify the gap between patients included in randomised controlled trials and real-life patients. DESIGN AND
settingThis retrospective, real-life study characterised all patients with non-IPF PPF diagnosed and treated at the Hospital District of Helsinki and Uusimaa.
participantsThirty-one patients were identified as having non-IPF fibrotic ILD and meeting the PPF criteria in 2022 and 2023.
resultsThe most common diagnosis was connective tissue disease-ILDs, with 13 patients (42%), followed by fibrotic idiopathic non-specific interstitial pneumonia (iNSIP), with 10 (32%) patients. The most common radiological pattern was NSIP in 18 (58%) patients.Among the 30 patients treated with nintedanib, six (20%) had to permanently discontinue treatment owing to side effects. Of the 19 patients who continued to receive nintedanib at the end of the follow-up period and for whom the dose was known, 9 (47%) continued to receive nintedanib at the full dose (150 mg two times per day) and 10 (53%) at the reduced dose (100 mg two times per day). Twenty-seven (87%) patients received immunomodulatory treatment at the time of PPF.
conclusionsIn our cohort, the underlying diagnoses and immunomodulatory treatments received significantly differed from those in the phase III licensing trial. More patients received nintedanib at a reduced dose than at a full dose, despite limited evidence of its efficacy and effectiveness. This highlights the large gap in evidence and the need for additional real-world data.
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