ESPN 50th Annual Meeting

ESPN 2017


 
LONG TERM RESULTS OF RITUXIMAB IN A TERTIARY REFERRAL CENTER FOR DIFFICULT-TO-TREAT NEPHROTIC SYNDROME
Kübra Çeleğen 1 Bora Gülhan 1 Mihriban İnözü 1 Nesrin Taş 1 Fatih Özaltın 1 Ali Düzova 1 Rezan Topaloğlu 1

1- HACETTEPE UNIVERSITY FACULTY OF MEDICINE DEPARTMENT OF PEDIATRIC NEPHROLOGY
 
Introduction:

Rituximab (RTX) has been proposed as an alternative treatment for steroid dependent nephrotic syndrome (SDNS), frequently relapsing nephrotic syndrome (FRNS) and steroid resistant nephrotic syndrome (SRNS).

Material and methods:

We evaluated the data of SDNS, FRNS and SRNS patients under RTX treatment. Initial RTX course consisted of 2-4 weekly infusions at the dose of 375 mg/m2.

Results:

A total amount of 38 patients (20 girls, 18 boys) were included in the study. The median age of NS diagnosis was 4.3 years (IQR, 1.8-12.1 years). Renal biopsy performed to all patients before RTX and revealed focal segmental glomerulosclerosis in 20, minimal change disease in 13 and other diagnoses in 5 patients. A total of 21 patients were categorized as SRNS, three patients as FRNS and 14 patients as SDNS according to steroid response. All SRNS patients were also resistant to calcineurin inhibitors (CNI). Median duration between nephrotic syndrome and initial RTX dose was 3.7 years (IQR; 1.7-8.4). The mean age of RTX treatment was 11.7±4.9 years. Transitory side effects were observed in two patients (throat soarness, erythematous rash; respectively). Mean duration of follow-up after RTX treatment was 2.7±1.6 years. Nineteen patients received regular maintenance treatment every 6-9 months. At last visit, six (28.5%) out of 21 SRNS patients were in remission. In three out of these six SRNS patients with remission, steroids and CNIs could be also stopped. Two (66.6%) out of three FRNS were relapse free after RTX treatment at last visit. In SDNS group, steroids or CNIs could be stopped in 11 out of 14 patients (78.6%). In SDNS group, five patients received regular RTX treatment every 6-9 months. In SDNS group, mean steroid dose at last visit was lower than before RTX treatment (p=0.008).

Conclusions:

Rituximab seems to be effective and safe treatment option for difficult-to treat nephrotic syndrome especially for SDNS and FRNS patient groups.