Michele Mondoni, Francesco Varone, Fabrizio Luppi, Paolo Cameli, Stefania Cerri, Mariangela Valentina Puci, Jacopo Cefalo, Simone Contino, Alessia Martini, Bruno Iovene, Giacomo Sgalla, Giovanni Franco, Umberto Zanini, Tommaso Pianigiani, Akter Dilroba, Daniele Puggioni, Athina Patsoura, Benedetta Mosole, Olga Torre, Elena Bargagli, Luca Richeldi, Giovanni Sotgiu
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引用次数: 0
Abstract
Purpose: Pulmonary fibrosis is defined as progressive based on the combination of radiological, clinical, and functional criteria. Nintedanib can reduce the rate of lung function decline, but no data are available on its effectiveness to hamper disease progression evaluated by these criteria. The primary aim of the study was to assess the number of patients with progressive pulmonary fibrosis (PPF) who no longer meet progression criteria after one year of treatment with nintedanib.
Methods: A retrospective, observational, multicenter study was carried out in Italy.
Results: 172 patients (91 (52.9%) males) with PPF were recruited, and 135 (78.5%) completed one year of treatment. After one year, 87 (64.4%) patients no longer met INBUILD progression criteria, while 107 (79.3%) did not meet 2022 ATS/ERS/JRS/ALAT guidelines criteria. Nintedanib hampered progression regardless of inclusion criteria, radiological pattern, and etiological diagnosis. Forced vital capacity (FVC) decline was significantly higher in the 12 months before than in those of treatment (mean, SD): 2512.3 (863.2) ml vs. 2313.6 (821.9) ml; p < 0.0001; 2313.6 (821.9) vs. 2335.7 (865.1); p = 0.82). FVC decline was significantly higher in the year before than in the year of treatment regardless of radiological pattern, etiological subtypes, and respiratory function. Diarrhea (mostly mild) was the most frequent adverse event (51.7%). A permanent discontinuation of the drug was recorded in 15 (9%) patients.
Conclusion: Nintedanib is effective and safe in patients with PPF. Besides slowing lung function decline, it hampers progression regardless of etiological diagnosis, radiological pattern, respiratory function, and baseline inclusion criteria.
目的：基于影像学、临床和功能标准，肺纤维化被定义为进行性。尼达尼布可以降低肺功能下降的速度，但没有数据表明其有效地阻碍了这些标准评估的疾病进展。该研究的主要目的是评估在尼达尼布治疗一年后不再符合进展标准的进行性肺纤维化（PPF）患者的数量。方法：在意大利进行回顾性、观察性、多中心研究。结果：172例PPF患者（91例（52.9%）男性）被招募，135例（78.5%）完成了1年的治疗。一年后，87例（64.4%）患者不再符合INBUILD进展标准，107例（79.3%）患者不符合2022年ATS/ERS/JRS/ALAT指南标准。无论纳入标准、放射学模式和病因诊断如何，尼达尼布都阻碍了进展。用力肺活量（FVC）下降在治疗前12个月明显高于治疗组（平均，SD）： 2512.3 (863.2) ml vs. 2313.6 (821.9) ml；结论：尼达尼布对PPF患者安全有效。除了减缓肺功能下降外，无论病因诊断、放射学模式、呼吸功能和基线纳入标准如何，它都会阻碍进展。
期刊介绍：
Lung publishes original articles, reviews and editorials on all aspects of the healthy and diseased lungs, of the airways, and of breathing. Epidemiological, clinical, pathophysiological, biochemical, and pharmacological studies fall within the scope of the journal. Case reports, short communications and technical notes can be accepted if they are of particular interest.
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