ESPN 51th Annual Meeting

ESPN 2018


 
EFFICACY OF TREATMENT WITH RITUXIMAB FOR DIFFICULT STEROID-RESISTANT AND -DEPENDENT NEPHROTIC SYNDROME; SINGLE CENTER EXPERIENCE
─░LKNUR GIRISGEN 1 SELCUK YUKSEL 1 YUCEL PEKAL 2

1- PAMUKKALE UNIVERSITY FACULTY OF MEDICINE DEPARTMENT OF PEDIATRIC NEPHROLOGY
2- PAMUKKALE UNIVERSITY FACULTY OF MEDICINE DEPARTMENT OF PEDIATRICS
 
Introduction:

Studies relating to rituximab treatment and outcomes in children with various nephrotic syndrome are scarce and unclear. The aim of this case-series study is to evaluate and share the outcomes of the efficacy of rituximab treatment in children with various nephrotic syndromes in our clinic.

Material and methods:

We reviewed the data from 13 pediatric patients with clinically nephrotic syndrome who were treated with rituximab. All the patients were either steroid-dependent (3 patients) or steroid-resistant (10 patients) nephrotic syndrome with various histological backgrounds, who failed to respond to other immunosuppressant drugs. All of them received rituximab (375mg/m2) once weekly for 4 weeks. The clinical features and laboratory data (leukocyte count including CD19 and CD20, liver function and renal function tests and proteinuria for 24 hours) were evaluated before and after rituximab. Partial remission was defined when proteinuria continued between 4-40 mg/m2/hour and complete remission when proteinuria levels were below 4 mg/m2/hour. At the end of the study, the outcomes and adverse events were recorded.

Results:

Median age was 15.6±4.0 (6-22) years, five girls and eight boys. CD19-CD20 cell depletion (<1%) was observed in 9 of 13 children. Kidney biopsy was performed in 12 patients; FSGS were in 5, MPGN in 2, IgA nephropathy in 1, and normal findings in 4. Complete remission was observed in 4 (normal biopsy in 3, FSGS in 1), partial remission in 2 patients (one of them FSGS, the other was not performed kidney biopsy). The patients with MPGN and IgA nephropathy did not respond. All of steroid dependent patients (three children) achieved complete remission, whereas only one patient achieved in steroid resistant children.  Severe leukopenia was observed in one patient with IgA nephropathy, but no serious infection.

Conclusions:

 Our study strongly suggested that rituximab could be an effective treatment for steroid-dependent nephrotic syndrome. We also observed that some (two of five) of patients with FSGS showed a positive influence of rituximab. More studies are necessary about relationship rituximab and FSGS.