ESPN 50th Annual Meeting

ESPN 2017


 
RITUXIMAB TREATMENT FOR REFRACTORY STEROID-DEPENDENT NEPHROTIC SYNDROME (SDNS) IN CHILDREN: A SINGLE-CENTER STUDY.
TATIANA VASHURINA 1 OLGA ZROBOK 1 PETR ANANIN 1 OLGA KOMAROVA 1 ELENA SEMIKINA 1 SVETLANA DMITRIENKO 1 TATIANA VOZNESENSKAYA 1 TAMARA SERGEEVA 1 ALEXEY TSYGIN 1

1- NATIONAL SCIENTIFIC AND PRACTICAL CENTRE FOR CHILDRENS HEALTH
 
Introduction:

 We evaluated the efficacy and safety of RTX in children with refractory SDNS treated in our center    

Material and methods:

Sixteen patients who were included in prospective single-center study remained dependent on high doses of prednisolone - the median 0,42 mg/kg/day, revealed a high incidence of relapses NS - the median 2,6 times/years, despite the use of different  immunosuppressants, and had clinical signs of severe steroid toxicity. At the beginning of RTX therapy, the median age of the patients was 11,5 years. During the first course of RTX, one to four infusions were administered, the majority of patients (69%) received two infusions.

Results:

Within 6 and 12 months after an initial treatment of RTX, 15 (94%) of 16 patients showed no relapses of NS. Steroids has been discontinued in 50% of patients after 6 months and in 64% at 12 months, in other children the dose of prednisolone was significantly reduced to 0,09 mg/kg/day (range 0,04-0,25) (p=0,043) and 0,04 mg/kg/day (range 0,02-0,16) (p=0,011), respectively. Calcineurin inhibitors have been discontinued in half of the patients, six months after the initial course. Eight patients had a long-term follow-up period of 2 to 7,5 years (median 2,8 years), long-term remission of NS from one to three years without prednisolone is maintained in three (37%), the others were on therapy of low-dose prednisone (the median 0,05 mg/kg/day, range 0,02-0,08) with significant reduction in the frequency of relapses – the median 0,1 times/year, p=0,012. Cyclosporine have been discontinued in all patients. Two (22.2%) of the sixteen patients developed agranulocytosis 4 months after the initial and repeated courses of RTX. In half, hypogammaglobulinemia was detected. One had self limited pulmonary tuberculosis. 

 

Conclusions:

Rituximab shows a significant reduction in the frequency of relapses, as well as a significant steroid-sparing and cyclosporine-sparing effect at refractory SDNS. Carefull follow-up is mandatory.