Remission of steroid-resistant nephrotic syndrome due to focal and segmental glomerulosclerosis using rituximab.
BACKGROUND
Therapeutic options are limited in cases of focal and segmental glomerulosclerosis (FSGS) that fail to respond to steroids and calcineurin inhibitors. We describe a case in which steroid-resistant nephrotic syndrome (SRNS) secondary to FSGS did not respond to conventional treatment, but was successfully treated with rituximab. Unlike previous reports in which rituximab was used in conjunction with a calcineurin inhibitor, we present the first case where rituximab was used as the sole therapeutic agent.


METHODS
An 11-month-old boy presented with severe manifestations of primary nephrotic syndrome with a subsequent non-responsive steroid course. A renal biopsy confirmed FSGS, with normal staining for podocin and nephrin. Genetic studies for podocin were normal. The child developed ciclosporin-induced hemolytic uremic syndrome (HUS), and the response to plasma exchange, following ciclosporin withdrawal, was only temporary. A trial of a combination of mycophenolate and dexamethasone did not have any effect on proteinuria or fluid status. Four weekly rituximab infusions at 375 mg/m2/dose induced a complete remission without any adverse effects.


CONCLUSIONS
This case suggests that rituximab may be used as the sole therapeutic agent in the treatment of SRNS secondary to FSGS, especially in cases where calcineurin inhibitors are contraindicated.
Suri
Megha
M
Department of Pediatrics, Children's Hospital of Western Ontario, University of Western Ontario, 800 Commissioners Road East, London, ON, Canada N6A 5W9.
Tran
Kim
K
Sharma
Ajay P
AP
Filler
Guido
G
Grimmer
Joanne
J
D000911:Antibodies, Monoclonal / Q000627:therapeutic use*; D058846:Antibodies, Monoclonal, Murine-Derived; D005923:Glomerulosclerosis, Focal Segmental / Q000150:complications*; D006801:Humans; D007166:Immunosuppressive Agents / Q000627:therapeutic use*; D007223:Infant; D008297:Male; D009404:Nephrotic Syndrome / Q000188:drug therapy* / Q000209:etiology; D012074:Remission Induction; D000069283:Rituximab; D013256:Steroids / Q000627:therapeutic use
D000911:Antibodies, Monoclonal; D058846:Antibodies, Monoclonal, Murine-Derived; D007166:Immunosuppressive Agents; D013256:Steroids; D000069283:Rituximab