Division of Neurology, Department of Internal Medicine, Nippon Medical School, 1-1-5 Sendagi, Bunkyo-ku, Tokyo 113-8603, Japan.
Clin Exp Nephrol. 2011 Feb;15(1):73-8. doi: 10.1007/s10157-010-0356-2. Epub 2010 Oct 23.
The long-term prognosis of immunoglobulin A nephropathy (IgAN) is reportedly poor. In Japan, tonsillectomy-steroid pulse therapy has frequently been used for treatment of early IgAN, with favorable outcomes. However, steroid usage is sometimes limited due to adverse reactions. To reduce the total dose of steroids, we have been using mizoribine (MZR) in combination with tonsillectomy-steroid pulse therapy since 2004. Here we report a retrospective evaluation of our protocol outcome.
Forty-two patients aged <70 years with histopathologically confirmed IgAN and an estimated glomerular filtration rate (eGFR) of 30 ml/min/1.73 m(2) or higher were enrolled. After giving informed consent, all the patients underwent bilateral tonsillectomy. One week later, intravenous methylprednisolone pulse therapy (500 mg/day) was administered for 3 days, followed by oral prednisolone (30 mg/day and tapered to 0 over 7 months) and MZR (150 mg/day for 11 months). The complete remission (CR) rate and renoprotective effect were assessed.
The CR rate at 6, 12, and 24 months was 33.3, 69.1, and 76.2%, respectively. Despite a relatively low total steroid dose, renal function was satisfactorily maintained for 24 months or longer with no relapse. The eGFR in patients with stage 3 chronic kidney disease was significantly improved at 6 months after start of treatment. Three patients (7.1%) had mild and transient adverse events.
This protocol appears to be highly effective and safe for IgAN patients with renal dysfunction.
据报道，免疫球蛋白 A 肾病（IgAN）的长期预后较差。在日本，扁桃体切除术-类固醇脉冲疗法经常被用于治疗早期 IgAN，疗效良好。然而，由于不良反应，类固醇的使用有时会受到限制。为了减少类固醇的总剂量，自 2004 年以来，我们一直在使用咪酯（MZR）联合扁桃体切除术-类固醇脉冲疗法。在这里，我们报告了对我们方案结果的回顾性评估。
42 例年龄<70 岁、组织病理学证实为 IgAN 且估计肾小球滤过率（eGFR）为 30 ml/min/1.73 m²或更高的患者入组。在获得知情同意后，所有患者均接受双侧扁桃体切除术。1 周后，给予静脉甲基强的松龙脉冲疗法（500 mg/天）3 天，随后口服泼尼松（30 mg/天，7 个月内逐渐减量至 0）和 MZR（150 mg/天，持续 11 个月）。评估完全缓解（CR）率和肾脏保护作用。
6、12 和 24 个月的 CR 率分别为 33.3%、69.1%和 76.2%。尽管总类固醇剂量相对较低，但肾功能在 24 个月或更长时间内得到了令人满意的维持，没有复发。治疗开始后 6 个月，慢性肾脏病 3 期患者的 eGFR 显著改善。3 名患者（7.1%）出现轻度且短暂的不良反应。
对于肾功能障碍的 IgAN 患者，该方案似乎非常有效且安全。
