Sellier-Leclerc A, Baudouin V, Macher M, et al.
Rituximab in Steroid Sensitive Nephrotic Syndrome.
ASN Annual Meeting -- San Diego
J Am Soc Nephrol
(Nov) 20:416A 2009

Most children with nephrotic syndrome respond to steroids, but a significant
number develop steroid dependency. Although many immunosuppressive agents
were proved effective, some severe cases remain difficult to treat and need
multidrug therapy.
Twenty nine children suffering from steroid
sensitive nephrotic syndrome were treated with rituximab (RTX) for steroid
dependency. The duration of the disease before RTX ranged from 0.7 to 17.5
years (mean 10.3). All patients had been treated with cyclosporine (N = 21)
or tacrolimus (n = 8) for 0.5 to 15.8 years (mean 8.3).
Following the
first cure of RTX, all patients had undetectable B cell count. The duration
of complete B depletion following this first cure ranged from 3.0 to 15.3
months with a mean of 7.8 months.
During the first 15th
months of depletion, a first group of 10 patients experienced a relapse of
nephrotic syndrome: 8 simultaneously to the recurrence of circulating B cells
and 1 while the B cell count was below 5/mm3. B-cells depletion
was easly obtained with additional reinjections of RTX in 7 patients. A
second group of 19 patients had sustained remission: 18 with complete B cell
depletion, either after the first cure of RTX (n=4) or after additional re-
injections (n=14) and 1 had sustained remission 13.7 months after the
recurrence of circulating B cells.
At last follow-up, 15 patients were
depleted for more than 15 months. One experienced a relapse simultaneously to
the recurrence of circulating B cells and 14 had sustained remission either
with complete B cell depletion (n = 7) or after the recurrence of circulating
B cells (n=7; mean duration of 5.8 months; range 0.4-10.2). Prednisone
therapy have been stopped in 23/29 patients, mycophenolate in 20/25,
cyclosporine or tacrolimus in 21/24.
In conclusion, RTX and B cell
depletion allow to sustain remission in steroid sensitive nephrotic syndrome
despite the decrease and the withdrawal of immunosuppressive and steroid
therapy. Long lasting B cell depletion leads to protracted remission in
patients with steroid and multidrug dependency, even after the withdrawal of
RTX and the subsequent normalization of B cell count in the blood.

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