BMJ open · 2025
Characterisation of patients with antifibrotic-treated non-idiopathic pulmonary fibrosis progressive pulmonary fibrosis: a retrospective real-life study.
СтатьяНазвание и аннотация: Перевод в очереди
Обработка статьи · этапы, ошибки и управление
Загрузка состояния…
Ошибки и ограничения
История · было → стало
Состояние обновляется каждые 5 секунд, пока блок открыт. Процент показывается только там, где известен объём работы. Пауза останавливает продолжение этапа; текущий запрос к источнику может завершиться.
Renner Andreas, Vertanen Elisa, Sutinen Eva, Ainola Mari, Myllärniemi Marjukka, Hollmén Maria
Аннотация
OBJECTIVE: Nintedanib 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 SETTING: This retrospective, real-life study characterised all patients with non-IPF PPF diagnosed and treated at the Hospital District of Helsinki and Uusimaa.
PARTICIPANTS: Thirty-one patients were identified as having non-IPF fibrotic ILD and meeting the PPF criteria in 2022 and 2023.
RESULTS: The 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.
CONCLUSIONS: In 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.
DESIGN AND SETTING: This retrospective, real-life study characterised all patients with non-IPF PPF diagnosed and treated at the Hospital District of Helsinki and Uusimaa.
PARTICIPANTS: Thirty-one patients were identified as having non-IPF fibrotic ILD and meeting the PPF criteria in 2022 and 2023.
RESULTS: The 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.
CONCLUSIONS: In 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.