Severity-based treatment for Japanese patients with MPO-ANCA-associated vasculitis: the JMAAV study.
Severity-based treatment for Japanese patients with MPO-ANCA-associated vasculitis: the JMAAV study.
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DOI：
10.1007/s10165-011-0525-5
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发表时间：
2012-06
影响因子：
2.2
通讯作者：
Kato H
中科院分区：
文献类型：
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作者：
Ozaki S;Atsumi T;Hayashi T;Ishizu A;Kobayashi S;Kumagai S;Kurihara Y;Kurokawa MS;Makino H;Nagafuchi H;Nakabayashi K;Nishimoto N;Suka M;Tomino Y;Yamada H;Yamagata K;Yoshida M;Yumura W;Amano K;Arimura Y;Hatta K;Ito S;Kikuchi H;Muso E;Nakashima H;Ohsone Y;Suzuki Y;Hashimoto H;Koyama A;Matsuo S;Kato H
我们（JMAAV [日本髓过氧化物酶-抗中性粒细胞胞浆抗体（MPO-ANCA）相关血管炎患者]研究组）进行了一项前瞻性、开放标签、多中心试验，以评价基于严重程度的治疗对髓过氧化物酶-抗中性粒细胞胞浆抗体（MPO-ANCA）相关血管炎日本患者的有效性。MPO-ANCA相关性血管炎患者根据适当的方案接受基于严重程度的治疗方案：轻度患者使用低剂量皮质类固醇，必要时使用环磷酰胺或硫唑嘌呤;重度患者使用高剂量皮质类固醇和环磷酰胺;最重度患者使用重度方案加血浆置换。我们对患者进行了18个月的随访。主要终点为诱导缓解、死亡和终末期肾病（ESRD）。登记了52名患者，本研究入组了48名患者（轻度，n = 23;重度，n = 23;最严重的形式，n = 2）。在接受预定治疗的47例患者中，42例在6个月内获得缓解，5例死亡，1例发展为ESRD。在18个月的随访期间，42例缓解患者中有8例疾病突然发作。JMAAV试验是在日本进行的第一项针对MPO-ANCA相关血管炎的前瞻性试验。缓解率和死亡率与之前在西方国家进行的几项临床试验相当。本试验中采用的方案是根据患者的疾病严重程度和疾病类型量身定制的，并且似乎标准化可以与根据严重程度做出的治疗选择一致。
We (JMAAV [Japanese patients with MPO-ANCA-associated vasculitis] Study Group) performed a prospective, open-label, multi-center trial to evaluate the usefulness of severity-based treatment in Japanese patients with myeloperoxidase-anti-neutrophil cytoplasmic antibodies (MPO-ANCA)-associated vasculitis. Patients with MPO-ANCA-associated vasculitis received a severity-based regimen according to the appropriate protocol: low-dose corticosteroid and, if necessary, cyclophosphamide or azathioprine in patients with mild form; high-dose corticosteroid and cyclophosphamide in those with severe form; and the severe-form regimen plus plasmapheresis in those with the most severe form. We followed up the patients for 18 months. The primary end points were the induction of remission, death, and end-stage renal disease (ESRD). Fifty-two patients were registered, and 48 patients were enrolled in this study (mild form, n = 23; severe form, n = 23; most severe form, n = 2). Among the 47 patients who received the predefined therapies, 42 achieved remission within 6 months, 5 died, and 1 developed ESRD. Disease flared up in 8 of the 42 patients with remission during the 18-month follow-up period. The JMAAV trial is the first prospective trial for MPO-ANCA-associated vasculitis to be performed in Japan. The remission and death rates were comparable to those in several previous clinical trials performed in western counties. The regimen employed in this trial was tailor-made based on patients’ disease severity and disease type, and it seems that standardization can be consistent with treatment choices made according to severity.