Pirfenidone and nintedanib have been approved for idiopathic pulmonary fibrosis (IPF) due to their ability to statistically slow, over a year, the rate of decline in lung forced vital capacity (FVC), neither drug has been reported to have o positive effects on high-resolution computed tomography (HRCT) of the chest, symptoms, or quality of life. Moreover, pirfenidone and nintedanib have substantial gastrointestinal tolerability issues. Overall, these data highly suggest that novel therapeutic approached are needed. CCN2 has been shown to be a mediator of fibrosis in many preclinical models. Anti-CCN2 strategies are in clinical development for IPF, A recent study by Richeldi and colleagues described the recent Phase II clinical trial for FG-3019 in IPF, and the results were highly encouraging. This commentary contextualizes and summarizes these exciting findings.

中文翻译：

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呼吸，呼吸空气：抗 CCN2 抗体 pamrevlumab (FG-3019) 成功完成了特发性肺纤维化的 II 期临床试验

吡非尼酮和尼达尼布已被批准用于特发性肺纤维化 (IPF)，因为它们能够在一年多的时间内在统计学上减缓肺用力肺活量 (FVC) 的下降速度，这两种药物均未报告对高肺纤维化有积极影响。胸部、症状或生活质量的分辨率计算机断层扫描 (HRCT)。此外，吡非尼酮和尼达尼布存在严重的胃肠道耐受性问题。总体而言，这些数据高度表明需要新的治疗方法。在许多临床前模型中，CCN2 已被证明是纤维化的介质。抗 CCN2 策略正在 IPF 的临床开发中，Richeldi 及其同事最近的一项研究描述了最近在 IPF 中针对 FG-3019 的 II 期临床试验，结果非常令人鼓舞。