Risk factors for relapse and long-term outcome in steroid-dependent nephrotic syndrome treated with rituximab.
BACKGROUND
Rituximab (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.


METHODS
We 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.


RESULTS
Eighty-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.


CONCLUSIONS
Rituximab treatment followed by immunosuppressants is an effective option for patients with SDNS, although a history of SRNS is a risk factor for early relapse.
Division of Nephrology and Rheumatology, National Center for Child Health and Development, Tokyo, Japan.
Koichi
K
Kamei
Division of Nephrology and Rheumatology, National Center for Child Health and Development, Tokyo, Japan.
Masao
M
Ogura
Division of Nephrology and Rheumatology, National Center for Child Health and Development, Tokyo, Japan.
Mai
M
Sato
Division for Clinical Trials, Department of Development Strategy, Center for Social and Clinical Research, National Research Institute for Child Health and Development, National Center for Child Health and Development, Tokyo, Japan.
Mayumi
M
Sako
Department of Pediatrics, Kobe University Graduate School of Medicine, Hyogo, Japan.
Kazumoto
K
Iijima
Division of Nephrology and Rheumatology, National Center for Child Health and Development, Tokyo, Japan. itoshu@yokohama-cu.ac.jp.|Department of Pediatrics, Yokohama City University, 3-9, Fukuura, Kanazawa-ku, Yokohama-shi, Kanagawa, 236-0004, Japan. itoshu@yokohama-cu.ac.jp.
Shuichi
S
Ito
D000293:Adolescent; D065095:Calcineurin Inhibitors / Q000008:administration & dosage; D002648:Child; D002675:Child, Preschool; D018572:Disease-Free Survival; D004334:Drug Administration Schedule; D004359:Drug Therapy, Combination; D005260:Female; D006801:Humans; D007166:Immunosuppressive Agents / Q000008:administration & dosage* / Q000009:adverse effects; D007223:Infant; D008297:Male; D009404:Nephrotic Syndrome / Q000175:diagnosis / Q000188:drug therapy* / Q000276:immunology; D012008:Recurrence; D012189:Retrospective Studies; D012307:Risk Factors; D000069283:Rituximab / Q000008:administration & dosage* / Q000009:adverse effects; D013256:Steroids / Q000008:administration & dosage* / Q000009:adverse effects; D013997:Time Factors; D016896:Treatment Outcome
D065095:Calcineurin Inhibitors; D007166:Immunosuppressive Agents; D013256:Steroids; D000069283:Rituximab
B-cell; Children; Rituximab; Steroid-dependent nephrotic syndrome; Steroid-resistant nephrotic syndrome