First Department of Pediatrics, Aristotle University of Thessaloniki, Thessaloniki, Greece.
Rheumatol Int. 2013 Mar;33(3):809-13. doi: 10.1007/s00296-011-2239-6. Epub 2011 Nov 19.
Lupus nephritis (LN) is the major determinant of outcome in pediatric systemic lupus erythematosus (pSLE), and its treatment remains a challenge. The aim of this study was to report the experience of our center in treating with rituximab (RTX) SLE patients with severe LN. Four pSLE patients with biopsy-proven LN, who are refractory to conventional immunosuppressive treatment, received four doses of 375-500 mg/m(2) RTX, 2-3 weeks apart. All patients were concurrently receiving corticosteroids (CSs) and mycophenolate mofetil. Patients' clinical and laboratory findings were recorded at RTX initiation, after each infusion and at 3.4 ± 2.1 month intervals thereafter. pSLE activity was assessed using the European Consensus Lupus Activity Measurement (ECLAM), while LN activity using 24-hour urine protein excretion and serum cystatin C. Patients were followed up for 6-21 months (median: 16 months). Full Β-cell depletion was noticed 2-4 weeks after RTX initiation and lasted 4-7 months. All patients achieved complete LN remission 3.5 months (range: 2-4) after RTX initiation, which was retained in 3 patients through the follow-up period. One patient relapsed 15 months after RTX initiation and received one additional RTX dose. ECLAM scores and CSs doses were markedly reduced in all patients, while complement levels were increased. No side effects or infections were observed. In conclusion, RTX is an alternative, safe and efficient treatment for refractory LN.
狼疮肾炎 (LN) 是儿童系统性红斑狼疮 (pSLE) 结局的主要决定因素,其治疗仍然是一个挑战。本研究的目的是报告我们中心使用利妥昔单抗 (RTX) 治疗严重 LN 的经验。4 例经活检证实的 LN 且对常规免疫抑制治疗无效的 pSLE 患者,每 2-3 周接受一次 375-500mg/m² RTX,共 4 次。所有患者均同时接受皮质类固醇 (CSs) 和吗替麦考酚酯治疗。在 RTX 开始时、每次输注后以及此后 3.4±2.1 个月时记录患者的临床和实验室发现。使用欧洲共识狼疮活动测量 (ECLAM) 评估 pSLE 活动,使用 24 小时尿蛋白排泄和血清胱抑素 C 评估 LN 活动。患者随访 6-21 个月(中位数:16 个月)。在 RTX 开始后 2-4 周注意到完全 B 细胞耗竭,并持续 4-7 个月。所有患者在 RTX 开始后 3.5 个月(范围:2-4)达到完全 LN 缓解,在随访期间 3 例患者持续缓解。1 例患者在 RTX 开始后 15 个月复发,接受了额外 1 次 RTX 治疗。所有患者的 ECLAM 评分和 CSs 剂量均显著降低,而补体水平升高。未观察到不良反应或感染。总之,RTX 是治疗难治性 LN 的一种替代、安全且有效的治疗方法。