Observational studyBMC pulmonary medicine2026
Nintedanib for progressive pulmonary fibrosis in real-world setting: an observational study comparing outcomes with an IPF cohort.
Observational study in BMC pulmonary medicine, 2026. The graph could read no effect estimate from its abstract, so it casts no vote on the map. Cited by 1 paper.
What it found
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The abstract states no effect estimate the extractor could read, or names no intervention and outcome on the map, so this paper lights no cell and moves no belief. It is still indexed, cited and linked below.
The trial behind it
Trials whose registry record cites this paper, or whose number appears in the abstract. A trial that started after this paper was published is citing it as background, not reporting it.
Neither the registry nor the abstract names a trial number. If this is a trial report, that itself is worth knowing.
Who cites it
1 citing paper in PubMed.
- Disease Mechanisms and Therapeutic Advances in Idiopathic and Progressive Pulmonary Fibrosis: From Approved Drugs to Emerging Strategies.Journal of clinical medicine · 2026Review
Corrections and comments
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Authors and funding
10 authors.
Funding
No grant is acknowledged in the PubMed record.
Abstract
introductionProgressive Pulmonary Fibrosis (PPF) includes various fibrosing ILDs evolving similarly to Idiopathic Pulmonary Fibrosis (IPF). Nintedanib, previously approved for IPF, showed antifibrotic efficacy in PPF, but real-world data remain limited. This retrospective study investigated the real-world effectiveness of nintedanib in PPF compared to IPF, focusing on PFTs trends, tolerability, and impact of concomitant therapies.
methodsData were retrospectively collected from an Italian referral centre (March 2021–August 2024). Study cohort met PPF classification criteria, while consecutive IPF patients were enrolled as control group. Lung function data at baseline, 12-months pre- and 12-month post-treatment were analysed using Linear Mixed Models. Adverse events and dose adjustments were recorded to assess safety.
resultsEighty-two PPF patients and 85 with IPF were included. At treatment initiation, the PPF group had significantly lower FVC (2.04 L vs 2.63 L; p < 0.001) and DLCO (46.41% vs 55.98%; p = 0.002), indicating more advanced disease. Nintedanib significantly reduced PPF cohort’s FVC decline (–141.40 mL before treatment then –20.36 mL; p < 0.001). A sub-analysis in PPF patient with radiological UIP/UIPp pattern revealed an increased efficacy of antifibrotic treatment. Although IPF group had better baseline condition, they showed higher rate of lung function decline. Most PPF patients (81.7%) received concurrently immunomodulatory drugs. Adverse events were primarily gastrointestinal in both groups, with similar incidence of dose reductions and treatment discontinuation.
conclusionIn a real-world setting, nintedanib significantly slowed disease progression in PPF patients, mirroring outcomes and tolerability profile demonstrated in IPF. Further large-scale, prospective studies are needed to understand how to optimize antifibrotic therapy for PPF.
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