Department of Respiratory Medicine, Fukuoka University Chikushi Hospital, Chikushino, Japan.
Department of Respiratory Medicine, Fukuoka University Chikushi Hospital, Chikushino, Japan.
Respir Investig. 2022 Jul;60(4):562-569. doi: 10.1016/j.resinv.2022.03.001. Epub 2022 Apr 6.
The antifibrotic agent nintedanib has been reported to effectively prevent the decline in forced vital capacity (FVC) in a broad range of interstitial lung diseases. However, the efficacy of nintedanib against idiopathic pleuroparenchymal fibroelastosis (iPPFE) remains unclear.
We retrospectively examined patients with idiopathic PPFE or idiopathic pulmonary fibrosis (IPF) who received nintedanib for more than 6 months. We evaluated annual changes in %FVC, radiological PPFE lesions, and body weight before and during nintedanib treatment. To investigate radiological PPFE lesions, we examined the fibrosis score, which was defined as the mean percentage of the high attenuation area in the whole lung parenchyma using three axial computed tomography images.
Overall, 15 patients with iPPFE and 27 patients with IPF were included in the present study. In patients with IPF, the annual rate of decline in %FVC was significantly lower during nintedanib treatment than that before treatment (-2.01%/year [-7.64 to 3.21] versus -7.64%/year [-10.8 to -4.44], p = 0.031). Meanwhile, in patients with iPPFE, the annual rate of decline in %FVC during nintedanib treatment was higher than that before treatment (-18.0%/year [-21.6 to -12.7] versus -9.40%/year [-12.3 to -8.23], p = 0.109). In addition, nintedanib treatment failed to inhibit the annual rate of increase in fibrosis score in patients with iPPFE (6.53/year [1.18-15.3] during treatment versus 2.70/year [0.27-12.2] before treatment, p = 0.175).
Nintedanib efficacy may be limited in patients with iPPFE.
抗纤维化药物尼达尼布已被报道能有效预防广泛的间质性肺疾病用力肺活量（FVC）的下降。然而，尼达尼布治疗特发性胸膜肺弹力纤维增生症（iPPFE）的疗效尚不清楚。
我们回顾性地检查了接受尼达尼布治疗超过 6 个月的特发性 PPFE 或特发性肺纤维化（IPF）患者。我们评估了尼达尼布治疗前后 FVC%的年变化、肺实质的影像学 PPFE 病变和体重。为了研究影像学 PPFE 病变，我们检查了纤维化评分，该评分是使用三个轴向 CT 图像定义的整个肺实质高衰减区域的平均百分比。
总体而言，本研究纳入了 15 例 iPPFE 患者和 27 例 IPF 患者。在 IPF 患者中，尼达尼布治疗期间 FVC%的年下降率明显低于治疗前（-2.01%/年[-7.64 至 3.21]与-7.64%/年[-10.8 至-4.44]，p=0.031）。同时，在 iPPFE 患者中，尼达尼布治疗期间 FVC%的年下降率高于治疗前（-18.0%/年[-21.6 至-12.7]与-9.40%/年[-12.3 至-8.23]，p=0.109）。此外，尼达尼布治疗未能抑制 iPPFE 患者纤维化评分的年增长率（治疗期间为 6.53/年[1.18-15.3]，治疗前为 2.70/年[0.27-12.2]，p=0.175）。
尼达尼布的疗效可能在 iPPFE 患者中受到限制。
