ESPN 51th Annual Meeting

ESPN 2018


 
THE RESULTS OF RITUXIMAB THERAPY IN THE PATIENTS WITH STEROID DEPENDENT NEPHROTIC SYNDROME
DEMET TEKCAN 1 OZLEM AYDOG 1 GURKAN GENC 2 NERGIS KENDIRCI 3 HULYA NALCACIOGLU 4 OZAN OZKAYA 5

1- ONDOKUZ MAYIS UNIVERSITY FACULTY OF MEDICINE, PEDIATRIC NEPHROLOGY DEPARTMENT
2- SPECIAL CLINIC, MD, PEDIATRIC NEPHROLOGY
3- ONDOKUZ MAYIS UNIVERSITY FACULTY OF MEDICINE, PEDIATRIC RHEUMATOLOGY DEPARTMENT
4- KAYSERI EDUCATION AND RESEARCH HOSPITAL, PEDIATRIC NEPHROLOGY DEPARTMENT
5- ISTINYE UNIVERSITY FACULTY OF MEDICINE, PEDIATRIC NEPHROLOGY DEPARTMENT
 
Introduction:

The patients with steroid dependent nephritic syndrome (SDNS) require prolonged steroid therapy. To reduce the side effects of prolonged steroid therapy, alternative treatments which have steroid-sparing effect are required. Alternative treatments include alkylating agents such as cyclophosphamide or chlorambucil, mycophenolate mofetil (MMF), cyclosporine, and more recently rituximab (RTX).

 

Material and methods:

Retrospectively, we evaluated the data of 6 SDNS patients who had only minimal changes on renal biopsy and received RTX in addition to steroid and other alternative treatments.

 

Results:

6 patients (2 girls, 4 boys) were included. The median age at the diagnosis of SDNS was 2.5 years (0.6–5.1 years). All patients were given alternative treatments; 2 patients oral cyclophosphamide, all patients cyclosporine and MMF. None of the patients responded to these, and they experienced frequent relapses. These 6 patients were given RTX.

Median duration between the diagnosis of SDNS and the onset of RTX was 10.2 years (5.5–14 years). The mean age of the patients at the onset of RTX was 12.5±3 years. The patients received RTX in a dose of 375 mg/m2 iv weekly for 2-4 weeks and repeated every 6 months as maintenance therapy for maximum 2 years. Rituximab was well tolerated in all patients, only one patient had adverse effects like skin rash and sore throat. All patients received low dose steroid and cyclosporine or MMF in addition RTX infusions.

The median duration of follow-up after the onset of RTX was 2 years (1-5.7 years). At last visit, 5 patients were in complete remission with preserved renal function without proteinuria. Unfortunately one patient who was noncompliant to RTX treatment, underwent to dialysis.

 

Conclusions:

Rituximab may be an effective and safe alternative treatment option for SDNS patients. Especially the rate of remission is higher in SDNS patients with minimal changes on renal biopsy.