Department of Nephrology, National Center for Child Health and Development, Tokyo, Japan.
Pediatr Nephrol. 2010 Oct;25(10):2175-7. doi: 10.1007/s00467-010-1571-5. Epub 2010 Jun 9.
Etanercept is a tumor necrosis factor (TNF)-alpha inhibitor that has been applied beneficially for juvenile idiopathic arthritis (JIA). We experienced long-term remission of nephrotic syndrome (NS) in a boy treated with etanercept, which was initially used for concomitant JIA. He developed NS at age 3 years 7 months and had mostly been treated with cyclosporine because of steroid dependency and frequent relapses. Cyclosporine was halted at 10 years 7 months because of nephrotoxicity, and he was subsequently treated with mizoribine. However, he had three relapses in the first year and developed JIA at 11 years 7 months. He was treated with sulfasalazine, methotrexate, and prednisolone, but his arthritis persisted. Etanercept was started at 12 years 3 months. Thereafter, his arthritis went into complete remission. Surprisingly, he has remained relapse-free for both NS and JIA for more than 3 years with etanercept and mizoribine. It is difficult to know whether the NS remission after initiating etanercept was coincidental. However, there are many reports of increased TNF-alpha or soluble TNF-alpha receptor in NS relapse. To date, there are two reports of the efficacy of TNF-alpha inhibitors against NS. It is possible that TNF-alpha inhibitors may have potential as therapeutic agents for NS.
依那西普是一种肿瘤坏死因子（TNF）-α抑制剂，已被成功应用于治疗幼年特发性关节炎（JIA）。我们曾遇到一例依那西普治疗肾病综合征（NS）长期缓解的病例，该患者最初接受依那西普治疗是为了同时治疗 JIA。他在 3 岁 7 个月时出现 NS，由于对激素依赖和频繁复发，主要接受环孢素治疗。10 岁 7 个月时因环孢素的肾毒性而停药，随后接受霉酚酸酯治疗。然而，他在第一年有 3 次复发，并在 11 岁 7 个月时出现 JIA。他接受柳氮磺胺吡啶、甲氨蝶呤和泼尼松龙治疗，但关节炎持续存在。12 岁 3 个月时开始使用依那西普。此后，他的关节炎完全缓解。令人惊讶的是，他在接受依那西普和霉酚酸酯治疗后，NS 和 JIA 均超过 3 年未复发。很难确定依那西普治疗后 NS 缓解是否是巧合。然而，有许多关于 NS 复发时 TNF-α 或可溶性 TNF-α 受体增加的报道。迄今为止，有 2 份关于 TNF-α 抑制剂治疗 NS 的疗效报告。TNF-α 抑制剂可能有作为 NS 治疗药物的潜力。
