Vol.:(0123456789) 1 3 Clinical and Experimental Nephrology https://doi.org/10.1007/s10157-018-1630-y ORIGINAL ARTICLE Therapeutic efcacy of rituximab for the management of adult-onset steroid-dependent nephrotic syndrome: a retrospective study Takayuki Katsuno 1,2  · Tomohiro Masuda 2  · Shoji Saito 2  · Noritoshi Kato 2  · Takuji Ishimoto 2  · Sawako Kato 2  · Tomoki Kosugi 2  · Naotake Tsuboi 2  · Hiroshi Kitamura 3  · Toyonori Tsuzuki 4  · Yasuhiko Ito 1  · Shoichi Maruyama 2 Received: 7 February 2018 / Accepted: 6 August 2018 © Japanese Society of Nephrology 2018 Abstract Background Recent reports have described the efcacy of rituximab in treating steroid-dependent nephrotic syndrome (SDNS) in pediatric patients. However, few reports describe data regarding adult-onset SDNS. We investigated the efcacy of rituximab for the management of adult-onset SDNS. Methods We performed a retrospective cohort study investigating eight patients with adult-onset SDNS who were treated with rituximab. Clinical data were obtained at the initiation of rituximab therapy. The primary outcomes evaluated were successful suppression of relapses and CD19+ cells after rituximab treatment. The corticosteroid- and immunosuppressant- sparing efect and adverse events were additionally evaluated. Results All eight patients were diagnosed with minimal change nephrotic syndrome and received immunosuppressants in addition to corticosteroid. Total number of relapses was 10.5 times as a median value. Rituximab administration was repeated in two patients, whereas six received single-dose rituximab. Three of eight (37.5%) patients showed relapse after rituximab therapy. A rituximab-induced depletion in CD19+ cells noted initially was followed by their reappearance in all patients. There were cases with no relapse after the reappearance of CD19+ cells. The median relapse time pre- and post-rituximab therapy showed a decrease from 1 time/year (interquartile range [IQR] 1–3 times/year) to 0 time/year (IQR 0–1 time/year). Rituximab treatment induced a signifcant reduction in the required doses of corticosteroid and cyclosporine (P < 0.01). No serious adverse events were observed. Conclusion Rituximab treatment was efective not only in childhood-onset but also in adult-onset SDNS. Further studies are needed to establish optimal treatment regimens. Keywords Adult-onset · Steroid-dependent nephrotic syndrome · Frequently relapsing nephrotic syndrome · Minimal change nephrotic syndrome · Rituximab Introduction Minimal change nephrotic syndrome (MCNS) shows a high prevalence in pediatric patients; however, most patients show a good response to corticosteroid therapy and > 90% of patients achieve complete remission with initial treat- ment. MCNS is also a major cause of idiopathic nephrotic syndrome (NS) in adults. Although MCNS patients show a favorable response to steroids, the relapse rate follow- ing a reduction of the steroid dose is reportedly as high as 30–70% [1, 2]. In those with steroid-dependent NS (SDNS) and frequently relapsing NS (FRNS), relapse prevention and reduction of steroids is managed with the addition of immunosuppressants such as cyclosporine (CyA), cyclo- phosphamide (CPA), mizoribine (MZR), tacrolimus (TAC), * Takayuki Katsuno t-katsuno@aichi-med-u.ac.jp 1 Department of Nephrology and Rheumatology, Aichi Medical University, 1-1 Karimata, Yazako, Nagakute 480-1195, Japan 2 Department of Nephrology, Nagoya University Graduate School of Medicine, Nagoya, Japan 3 Department of Pathology, National Hospital Organization Chiba East Hospital, Chiba, Japan 4 Department of Surgical Pathology, Aichi Medical University, Nagakute, Japan