Shimojima Yasuhiro, Ishii Wataru, Matsuda Masayuki, Kishida Dai, Ikeda Shu-Ichi
From the Department of Medicine (Neurology & Rheumatology), Shinshu University School of Medicine, Matsumoto, Japan.
J Clin Rheumatol. 2017 Mar;23(2):87-93. doi: 10.1097/RHU.0000000000000487.
The current study demonstrated the potential use of calcineurin inhibitor (CNI) in combination therapy for interstitial lung disease (ILD) complicated with dermatomyositis (DM) and polymyositis (PM).
Thirty DM/PM patients with ILD were enrolled in this study. Continuous intravenous administration of cyclosporine A (IV-CsA) was simultaneously started with corticosteroid in patients presenting more than two respiratory distress factors as follows: <70 mmHg of PaO2, percentage of vital capacity <70%, and/or exertional dyspnea. Other patients took CNI orally with corticosteroid. When a patient presented exacerbation of ILD, intravenous cyclophosphamide pulse therapy (IVCY) was additionally administrated. Clinical outcomes were compared with those of 21 patients who had been previously treated in our hospital before starting this study, as the historical comparison group.
Seven patients underwent IV-CsA, maintaining the drug concentration in blood between 300 and 350 ng/mL, and six of them had favorable outcomes without any adverse events. Eight out of the 30 patients additionally required IVCY. Meanwhile, the frequency of death ascribable to respiratory failure was 6.7%, which was significantly lower than that of the historical comparison group (P = 0.043). Furthermore, longer survival free from exacerbation and severe adverse event was significantly shown (P = 0.029).
For repressing the activity of ILD in DM/PM patients, stabilizing the blood concentration of CNI immediately is a reasonable treatment as well as initiating immunosuppressive therapy in the early phase of the illness. IV-CsA may be a useful option for achieving this purpose in patients with severe ILD.
本研究证明了钙调神经磷酸酶抑制剂（CNI）在间质性肺病（ILD）合并皮肌炎（DM）和多发性肌炎（PM）联合治疗中的潜在用途。
30例患有ILD的DM/PM患者纳入本研究。对于出现以下两种以上呼吸窘迫因素的患者：动脉血氧分压（PaO2）<70 mmHg、肺活量百分比<70%和/或劳力性呼吸困难，在给予皮质类固醇的同时开始持续静脉输注环孢素A（IV-CsA）。其他患者口服CNI并联合皮质类固醇。当患者出现ILD加重时，额外给予静脉环磷酰胺脉冲治疗（IVCY）。将临床结果与本研究开始前在我院接受过治疗的21例患者作为历史对照组进行比较。
7例患者接受IV-CsA治疗，血药浓度维持在300至350 ng/mL之间，其中6例预后良好，无任何不良事件。30例患者中有8例额外需要IVCY。同时，因呼吸衰竭导致的死亡率为6.7%，显著低于历史对照组（P = 0.043）。此外，无病情加重和严重不良事件的生存期显著延长（P = 0.029）。
为抑制DM/PM患者ILD的活动，立即稳定CNI血药浓度是一种合理的治疗方法，同时在疾病早期启动免疫抑制治疗。IV-CsA可能是重度ILD患者实现这一目的的有用选择。
