Department of Pulmonology, Semmelweis University, Budapest, Hungary.
Department of Respiratory Diseases of the First Faculty of Medicine, Charles University, Thomayer Hospital, Prague, Czech Republic.
Adv Ther. 2019 May;36(5):1221-1232. doi: 10.1007/s12325-019-00906-9. Epub 2019 Mar 14.
Idiopathic pulmonary fibrosis (IPF) is one of the most common interstitial lung diseases with limited survival. The effect of IPF therapy in patients with severely impaired lung function is not well established. The aim of this study was to characterize IPF patients with a forced vital capacity (FVC) < 50% (group 1) and FVC 50-60% predicted (group 2) and analyze the effect and adverse events of nintedanib in Hungarian patients diagnosed between April 2015 and July 2017.
The impact of nintedanib therapy on lung function, survival, and adverse events was analyzed longitudinally.
Twenty-two out of 103 patients were included in the analysis (group 1: N = 10; male/female = 6:4, age 62.6 ± 10.8 years and group 2: N = 12; male/female = 3:9, age 65.7 ± 11.6 years). Eighteen patients were treated with nintedanib (8 in group 1, 10 in group 2); treatment stabilized lung function in 42% and 50%, respectively, in the two groups. Median survival was 444 days for group 1 and 476 days for group 2. Adverse events were less common than in clinical trials; dose reduction was necessary in three cases, drug discontinuation in two cases. No differences between groups were identified regarding clinical parameters and radiological pattern; however, hypertension as comorbidity was more common in group 1 patients.
Nintedanib therapy was effective and well tolerated even among patients with severely impaired lung function. Longitudinal follow-up confirmed high mortality in patients with very severe and severe IPF; however, median survival was meaningful as it exceeded 1 year in both groups.
特发性肺纤维化（IPF）是最常见的间质性肺疾病之一，患者的生存时间有限。IPF 患者肺功能严重受损时的治疗效果尚未得到充分证实。本研究的目的是描述用力肺活量（FVC）<50%（第 1 组）和 FVC 50-60%预计值（第 2 组）的 IPF 患者的特征，并分析尼达尼布治疗匈牙利患者的效果和不良事件，这些患者的诊断时间为 2015 年 4 月至 2017 年 7 月。
对尼达尼布治疗的肺功能、生存和不良事件进行了纵向分析。
在 103 例患者中，有 22 例纳入分析（第 1 组：N=10；男/女=6:4，年龄 62.6±10.8 岁；第 2 组：N=12；男/女=3:9，年龄 65.7±11.6 岁）。18 例患者接受了尼达尼布治疗（第 1 组 8 例，第 2 组 10 例）；两组患者的肺功能分别稳定治疗 42%和 50%。第 1 组和第 2 组的中位生存时间分别为 444 天和 476 天。与临床试验相比，不良事件发生率较低；3 例患者需要减少剂量，2 例患者需要停药。两组患者的临床参数和影像学模式无差异；然而，第 1 组患者更常见合并高血压。
尼达尼布治疗即使在肺功能严重受损的患者中也具有疗效，且耐受性良好。纵向随访证实，非常严重和严重的 IPF 患者死亡率很高；然而，两组患者的中位生存时间均有意义，均超过 1 年。
