Clinical Research Center, National Hospital Organization, Kinki-Chuo Chest Medical Center, Sakai City, Japan.
Department of Internal Medicine, National Hospital Organization, Kinki-Chuo Chest Medical Center, Sakai City, Japan.
Respirology. 2017 Oct;22(7):1363-1370. doi: 10.1111/resp.13065. Epub 2017 May 15.
Acute exacerbation (AE) of idiopathic pulmonary fibrosis (IPF) (AE-IPF) is a poor prognostic disorder. AE is also reported to occur in other idiopathic interstitial pneumonias (IIPs). There are limited data available regarding the effectiveness of treatment for AE-IIPs. The objective of this study was to clarify the prognostic impact of the initial dose of prednisolone (PSL) for treating AE-IIPs.
Eighty-five patients with AE-IIPs, diagnosed according to the criteria of the Japanese Respiratory Society, were enrolled in this study (IPF/non-IPF: 63/22 patients) from 2004 to 2013. We performed multivariate Cox proportional hazard regression analysis to identify poor prognostic factors. HRCT patterns at the onset of AE-IIPs were classified as diffuse or non-diffuse. We evaluated the prognostic significance of the initial dose of PSL by adjusting for other prognostic factors.
Median survival time (MST) after AE-IIPs diagnosis was 49 days. MST of AE-IPF and AE-non-IPF was 39 and 49 days, respectively. A diffuse HRCT pattern, lower serum IgG and higher serum surfactant protein-D at AE diagnosis, long-term oxygen therapy (LTOT) before AE and positive pressure ventilation (PPV) use for AE were significant poor prognostic factors for all patients, as were LTOT before AE and lower serum IgG for no-PPV patients. High-dose PSL ≥ 0.6 mg/kg was a significant prognostic factor for no-PPV patients after adjusting for other prognostic factors.
We concluded that a dose of PSL ≥ 0.6 mg/kg after i.v. high-dose methylprednisolone therapy should be recommended for the treatment of AE-IIPs.
特发性肺纤维化（IPF）的急性加重（AE）是一种预后不良的疾病。AE 也有报道发生于其他特发性间质性肺炎（IIP）。目前关于治疗 AE-IIP 的有效性的数据有限。本研究的目的是明确初始泼尼松龙（PSL）剂量对治疗 AE-IIP 的预后影响。
2004 年至 2013 年，我们根据日本呼吸学会的标准，共纳入 85 例 AE-IIP 患者（IPF/非 IPF：63/22 例）。我们进行了多变量 Cox 比例风险回归分析，以确定不良预后因素。AE-IIP 发病时的 HRCT 模式分为弥漫性或非弥漫性。我们通过调整其他预后因素来评估初始 PSL 剂量的预后意义。
AE-IIP 诊断后的中位生存时间（MST）为 49 天。AE-IPF 和 AE-非 IPF 的 MST 分别为 39 天和 49 天。AE 时弥漫性 HRCT 模式、较低的血清 IgG 和较高的血清表面活性剂蛋白-D、AE 前长期氧疗（LTOT）和 AE 时正压通气（PPV）的使用是所有患者的显著不良预后因素，AE 前 LTOT 和非 PPV 患者的血清 IgG 较低也是显著不良预后因素。AE 后调整其他预后因素后，PSL 剂量≥0.6mg/kg 是无 PPV 患者的显著预后因素。
我们的结论是，对于 AE-IIP，在静脉注射大剂量甲泼尼龙治疗后，应推荐使用 PSL 剂量≥0.6mg/kg。
