• Koichi Kamei, Masao Ogura, Mai Sato, Mayumi Sako, Kazumoto Iijima, and Shuichi Ito.
• Division of Nephrology and Rheumatology, National Center for Child Health and Development, Tokyo, Japan.
• Pediatr. Nephrol. 2016 Jan 1; 31 (1): 89-95.

BackgroundRituximab (RTX) is known to be effective for the treatment of refractory steroid-dependent nephrotic syndrome (SDNS). However, there are insufficient data on the risk factors for relapse and long-term outcome after RTX treatment.MethodsWe administered a single dose of RTX to patients with refractory SDNS from November 2007 to December 2013 and continued with immunosuppressants. The risk factors for early relapse and long-term outcome were analyzed.ResultsEighty-one patients were included and the observation period was 13-90 months. Seventy-six patients (94 %) discontinued steroids. Median duration of B-cell depletion was 160 days and 50 % relapse-free survival was 482 days. In multivariate analyses, only a history of steroid-resistant nephrotic syndrome (SRNS) was a statistically significant risk factor (hazard ratio, 2.44; p = 0.048). Fifty percent relapse-free survival in patients without a history of SRNS was 615 days, longer than that of patients with a history of SRNS (393 days) (p=0.005) [corrected]. Fifty-one patients (63 %) received additional RTX treatments for relapses. At last observation, patients using calcineurin inhibitors decreased from 89 % to 23 %, and 12 patients (15 %) discontinued immunosuppressants.ConclusionsRituximab treatment followed by immunosuppressants is an effective option for patients with SDNS, although a history of SRNS is a risk factor for early relapse.

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