Sakamoto Susumu, Itoh Takafumi, Muramatsu Yoko, Satoh Keita, Ishida Fumiaki, Sugino Keishi, Isobe Kazutoshi, Homma Sakae
Department of Respiratory Medicine, Toho University Omori Medical Center, Japan.
Intern Med. 2013;52(22):2495-501. doi: 10.2169/internalmedicine.52.8498.
To assess the efficacy of pirfenidone in patients with advanced-stage idiopathic pulmonary fibrosis (IPF), we conducted a retrospective study of patients who received pirfenidone therapy. In addition, the combined effects of inhaled N-acetylcysteine (NAC) and pirfenidone were evaluated.
Eligible patients had a clinical and radiologic diagnosis of advanced-stage IPF (stages of severity III&IV). Patients who exhibited a relative decline in forced vital capacity (FVC) of 10% or more within the preceding six (±2) months were enrolled. The outcome was evaluated from the date of the 6-month follow-up PFT. Relative declines in FVC of more than 10% were defined as progressive disease (ineffective group), while those less than 10% were defined as stable disease (effective group). The clinical features were compared between the two groups. We also compared the efficacy of the combined therapy with inhaled NAC and pirfenidone (n=11) with that of pirfenidone alone (n=7).
Eighteen patients 59-82 years of age with IPF who received pirfenidone therapy were reviewed. Pirfenidone stabilized declines in FVC by 10% at six months in eight of the 18 cases (44%). The median changes in FVC at six months were +120 mL in the effective group and -590 mL in the ineffective group. The number of NAC users was significantly higher in the effective group (7/8=87.5%) than in the ineffective group (3/10=30%) (p=0.02). Furthermore, the use of combined NAC therapy was correlated with a favorable outcome. The median change in FVC at six months was 0 mL in the NAC group and -290 mL in the non-NAC group. The median survival period was 557 ± 66 days in the NAC group and 196 ± 57 days in the non-NAC group (p=0.03).
Among the advanced-stage IPF patients with a more progressive status, pirfenidone decreased the rate of decline in FVC. In addition, patients treated with pirfenidone combined with NAC therapy exhibited favorable outcomes. Additional studies are needed to confirm the efficacy of this combined therapy for IPF.
为评估吡非尼酮对晚期特发性肺纤维化（IPF）患者的疗效，我们对接受吡非尼酮治疗的患者进行了一项回顾性研究。此外，还评估了吸入用N - 乙酰半胱氨酸（NAC）与吡非尼酮的联合疗效。
符合条件的患者临床及影像学诊断为晚期IPF（严重程度为III和IV期）。纳入在之前六（±2）个月内用力肺活量（FVC）相对下降10%或更多的患者。从6个月随访肺功能测试日期开始评估结果。FVC相对下降超过10%被定义为疾病进展（无效组），而下降少于10%被定义为疾病稳定（有效组）。比较两组的临床特征。我们还比较了吸入用NAC与吡非尼酮联合治疗（n = 11）和单独使用吡非尼酮治疗（n = 7）的疗效。
回顾了18例年龄在59 - 82岁接受吡非尼酮治疗的IPF患者。18例患者中有8例（44%）在6个月时吡非尼酮使FVC下降稳定在10%以内。有效组6个月时FVC的中位数变化为+120 mL，无效组为 - 590 mL。有效组中使用NAC的患者数量（7/8 = 87.5%）显著高于无效组（3/10 = 30%）（p = 0.02）。此外，联合使用NAC治疗与良好预后相关。NAC组6个月时FVC的中位数变化为0 mL，非NAC组为 - 290 mL。NAC组的中位生存期为557 ± 66天，非NAC组为196 ± 57天（p = 0.03）。
在病情进展较快的晚期IPF患者中，吡非尼酮降低了FVC的下降速率。此外，接受吡非尼酮联合NAC治疗的患者表现出良好的预后。需要进一步研究来证实这种联合治疗对IPF的疗效。
