Dello Strologo Luca, Guzzo Isabella, Laurenzi Chiara, Vivarelli Marina, Parodi Angelica, Barbano Giancarlo, Camilla Roberta, Scozzola Floriana, Amore Alessandro, Ginevri Fabrizio, Murer Luisa
Nephrology and Urology Department, Bambino Gesù Children's Hospital, Institute for Scientific Research, Piazza S. Onofrio 4, Rome, Italy.
Transplantation. 2009 Aug 15;88(3):417-20. doi: 10.1097/TP.0b013e3181aed9d7.
Focal and segmental glomerulosclerosis (FSGS) accounts for more than 10% of all cases of renal diseases leading to renal failure in children. After renal transplantation, 20% to 40% of FSGS relapse, frequently leading to renal loss.Plasmapheresis is considered the first option to treat relapses by several authors but is often ineffective. The anti-CD20 monoclonal antibody rituximab has been proposed as a possible treatment.
We reviewed the effect of rituximab in seven children or young adults with pretransplant FSGS and relapse of proteinuria after transplantation who did not respond to intensive plasmapheresis.
After treatment, urine protein disappeared in three patients, was reduced by 70% in one patient and by 50% in one patient. No response was observed in one patient who had a quick deterioration of renal function and reached end-stage renal failure after 3 months. One additional patient developed a severe reaction a few minutes after the start of the first rituximab infusion.
Rituximab is a possible option to treat some resistant cases of FSGS with relapsing proteinuria after transplantation. It is important that therapy is started early after evidence of failure of plasmapheresis, before sclerosis develops in the glomeruli. The response to treatment can occur after several months. During the follow-up period, CD19 cells should be monitored carefully, and additional rituximab infusions considered to maintain B-cell depletion.
局灶节段性肾小球硬化(FSGS)在导致儿童肾衰竭的所有肾脏疾病病例中占比超过10%。肾移植后,20%至40%的FSGS会复发,常导致移植肾丧失。几位作者认为血浆置换是治疗复发的首选方法,但往往无效。抗CD20单克隆抗体利妥昔单抗已被提议作为一种可能的治疗方法。
我们回顾了利妥昔单抗对7例患有移植前FSGS且移植后蛋白尿复发、对强化血浆置换无反应的儿童或年轻成人的疗效。
治疗后,3例患者尿蛋白消失,1例患者尿蛋白减少70%,1例患者尿蛋白减少50%。1例患者肾功能迅速恶化,3个月后达到终末期肾衰竭,未观察到反应。另1例患者在首次输注利妥昔单抗开始几分钟后出现严重反应。
利妥昔单抗是治疗移植后复发性蛋白尿的一些耐药FSGS病例的一种可能选择。重要的是在血浆置换失败证据出现后尽早开始治疗,在肾小球硬化发展之前。治疗反应可能在数月后出现。在随访期间,应仔细监测CD19细胞,并考虑额外输注利妥昔单抗以维持B细胞耗竭。