Rituximab and low-dose cyclosporine combination therapy for steroid-resistant focal segmental glomerulosclerosis.
BACKGROUND
We investigated the efficacy of rituximab and low-dose cyclosporine combination therapy for focal segmental glomerulosclerosis (FSGS) in children with steroid-resistant nephrotic syndrome (SRNS).


METHODS
Five FSGS children with SRNS were treated twice with rituximab and low-dose cyclosporine (CyA) combination therapy (RTX-CyAT). The clinical features and laboratory data were investigated before and after RTX-CyAT, and the outcomes were assessed.


RESULTS
Prednisolone (PSL) was discontinued 3 months after RTX-CyAT in all patients. The number of CD19-positive cells decreased to <1% of all white blood cells in all patients at 1 month after RTX-CyAT, and was maintained at this level for 259.6 ± 68.2 days. All patients remained in remission for the duration of the decrease in CD19-positive cells to <1%. Two patients also remained in remission throughout the observation period, with three patients having a single relapse at 333 ± 89 days (range, 231-376 days) after RTX-CyAT. In all patients, the mean steroid and CyA doses after RTX-CyAT were lower than those before RTX-CyAT.


CONCLUSIONS
RTX-CyAT is effective in FSGS patients with SRNS and may ameliorate the side-effects of PSL and immunosuppressive drugs.
Department of Pediatrics, Fukushima Medical University School of Medicine, Fukushima City, Fukushima, Japan.
Kazuhide
K
Suyama
Department of Pediatrics, Fukushima Medical University School of Medicine, Fukushima City, Fukushima, Japan.
Yukihiko
Y
Kawasaki
Department of Pediatrics, Fukushima Medical University School of Medicine, Fukushima City, Fukushima, Japan.
Kyohei
K
Miyazaki
Department of Pediatrics, Fukushima Medical University School of Medicine, Fukushima City, Fukushima, Japan.
Syuto
S
Kanno
Department of Pediatrics, Fukushima Medical University School of Medicine, Fukushima City, Fukushima, Japan.
Atsushi
A
Ono
Department of Pediatrics, Fukushima Medical University School of Medicine, Fukushima City, Fukushima, Japan.
Yuichi
Y
Suzuki
Department of Pediatrics, Fukushima Medical University School of Medicine, Fukushima City, Fukushima, Japan.
Shinichiro
S
Ohara
Department of Pediatrics, Fukushima Medical University School of Medicine, Fukushima City, Fukushima, Japan.
Mitsuaki
M
Hosoya
D000293:Adolescent; D002648:Child; D002675:Child, Preschool; D016572:Cyclosporine / Q000008:administration & dosage*; D004305:Dose-Response Relationship, Drug; D004351:Drug Resistance; D004359:Drug Therapy, Combination; D005260:Female; D005500:Follow-Up Studies; D005923:Glomerulosclerosis, Focal Segmental / Q000175:diagnosis / Q000188:drug therapy*; D005938:Glucocorticoids / Q000494:pharmacology*; D006801:Humans; D007155:Immunologic Factors / Q000008:administration & dosage; D007166:Immunosuppressive Agents / Q000008:administration & dosage; D007223:Infant; D008297:Male; D012074:Remission Induction; D012189:Retrospective Studies; D000069283:Rituximab / Q000008:administration & dosage*; D016896:Treatment Outcome
D005938:Glucocorticoids; D007155:Immunologic Factors; D007166:Immunosuppressive Agents; D000069283:Rituximab; D016572:Cyclosporine
children; cyclosporine; focal segmental glomerulosclerosis; nephrotic syndrome; rituximab