ESPN 51th Annual Meeting

ESPN 2018


 
Long-term use of calcineurin inhibitors in difficult-to-treat nephrotic syndrome
NUR CANPOLAT 1 SEHA KAMIL SAYGILI 1 ISIN KILICASLAN 2 MEHMET TASDEMIR 1 AYSE AGBAS 1 RUMEYSA YASEMIN CICEK 1 ISHAK ARSLAN 1 SALIM CALISKAN 1 LALE SEVER 1

1- ISTANBUL UNIVERSITY CERRAHPASA FACULTY OF MEDICINE, DEPARTMENT OF PEDIATRIC NEPHROLOGY
2- ISTANBUL UNIVERSITY ISTANBUL FACULTY OF MEDICINE, DEPARTMENT OF PATHOLOGY
 
Introduction:

Despite the known nephrotoxic effects, calcineurin inhibitor (CNI)s have been widely used as steroid-sparing agents in patients with difficult-to-treat nephrotic syndrome. The aim of the present study was to evaluate renal function and growth in children with nephrotic syndrome treated with CNI.

Material and methods:

This single center study included 36 patients with nephrotic syndrome [18 steroid sensitive (SSNS), 18 steroid resistant (SRNS)] treated with CNI for ≥12 months. All patients received firstly cyclosporin (CsA) + low dose steroid. In the case of toxicity CsA was switched to tacrolimus (TAC). Clinical features, the course of the disease before and after CNI treatment, adverse effects, biopsy findings and serum creatinine levels (at the initiation, during therapy and at the cessation of CNI) were retrospectively recorded. eGFR and standard deviation score (SDS)s of height and BMI were calculated. 

Results:

Mean age at the onset of NS and at the initiation of CNI were 5.3±4.7 and 8.1±5.0 years, respectively. Initial renal histology showed FSGS (n=16), MCD (n=7) and mesengial proliferation (n=6). Mean duration of CNI treatment was 48±31 (12-126) months. Complete remission was achieved in 11 (31%) and partial remission in 2 patients (6%). Remaining 23 patients had infrequent relapses and frequency of relapses per patient months significantly decreased after CNI treatment (p=0.001). There was no significant difference in eGFR between the initiation and cessation of CNI. CNI toxicity was not observed in any post-treatment biopsies (n=8). Sixteen patients (44%) suffered from adverse effects of CsA and 7 patients were switched to TAC. At the first year of CNI, height-SDS significantly increased in patients with both SSNS (p=0.008) and SRNS (p=0.033), and BMI-SDS significantly decreased in patients with SSNS (p=0.035).

Conclusions:

 CNI usage has no negative effects on renal function and is associated with an improvement on growth in patients with difficult-to-treat nephrotic syndrome.