Kronbichler Andreas, Bruchfeld Annette
Department of Internal Medicine IV (Nephrology and Hypertension), Medical University Innsbruck, Innsbruck, Austria.
Nephron Clin Pract. 2014;128(3-4):277-82. doi: 10.1159/000368590. Epub 2014 Nov 8.
Treatment of nephrotic syndrome due to minimal change disease and focal segmental glomerulosclerosis remains a challenge since steroid dependence, steroid resistance and a relapsing disease course exhibits a high cumulative steroid dosage. The necessity of using alternative steroid-sparing immunosuppressive agents with potential toxic side effects also restricts their long-term use. Rituximab, a monoclonal antibody targeting CD20, has been increasingly used in the therapy of difficult-to-treat nephrotic syndrome. A clinical response has been shown for patients with steroid-dependent or frequently relapsing nephrotic syndrome, whereas the benefit seems to be limited in steroid-resistant patients, especially those with underlying focal segmental glomerulosclerosis. No potentially life-threatening adverse events have been observed in the treatment of adult minimal change disease and focal segmental glomerulosclerosis following rituximab administration. Since most reports are retrospective and evidence of efficacy is derived from small case series, more prospective trials in a controlled, randomized manner are highly desirable to delineate the use of rituximab or other B cell-depleting agents in steroid-dependent, frequently relapsing or steroid-resistant patients.
微小病变病和局灶节段性肾小球硬化所致肾病综合征的治疗仍然是一项挑战,因为激素依赖、激素抵抗和疾病复发过程显示出高累积激素剂量。使用具有潜在毒性副作用的替代激素的免疫抑制剂的必要性也限制了它们的长期使用。利妥昔单抗是一种靶向CD20的单克隆抗体,已越来越多地用于治疗难治性肾病综合征。对于激素依赖或频繁复发的肾病综合征患者已显示出临床反应,而在激素抵抗患者中,尤其是那些伴有潜在局灶节段性肾小球硬化的患者,其益处似乎有限。在利妥昔单抗治疗成人微小病变病和局灶节段性肾小球硬化后,未观察到潜在的危及生命的不良事件。由于大多数报告是回顾性的,且疗效证据来自小病例系列,因此非常需要进行更多以对照、随机方式进行的前瞻性试验,以明确利妥昔单抗或其他B细胞耗竭剂在激素依赖、频繁复发或激素抵抗患者中的应用。