Cyclophosphamide in frequent-relapsing or steroid-depent nephrotic syndrome: Review of 38 patients
ABSTRACT: Steroid-sensitive
nephrotic syndrome (SSNS) in children is characterized by relapsing courses in
a substantial proportion of affected individuals. Children with
frequent-relapsing nephrotic syndrome (FRNS) or steroid-dependent nephrotic
syndrome (SDNS) are at risk of severe steroid toxicity and need individualized
treatment. Previous studies have elucidated that cyclophosphamide (CPA) reduced
the risk of relapses and increased the length of subsequent remissions in
children with relapsing SSNS.
Methods This retrospective study evaluated 38 patients (26 FRNS and 12
SDNS) after cyclophosphamide therapy to elucidate the efficacy of CPA in FRNS
or SDNS in the Department of Child Health, Cipto Mangunkusumo Hospital. All
patients were treated with CPA (2 mg/kg per day) for 8 weeks, in combination
with prednisone.
Results The median (range) duration of follow up was 45 months (24-140
months) for FRNS and 29 months (24-63 months) for SDNS. The mean relapse rate
one year prior to CPA therapy in FRNS and SDNS were 3.8 relapses/year (95%CI
3.4; 4.2) and 4.0 relapses/year (95%CI 3.3; 4.7), which were reduced to 1.6
relapses/ year (95% CI 1.1; 2.1) and 2.3 relapses/year (95%CI 1.5;3.2),
respectively. The overall rate of cumulative sustained good response (complete
remission or infrequent relapses) was 65% after 36 months. Frequent relapsing
versus steroid-dependent status was significantly correlated with rate of
sustained good response after 36 months (85% versus 15%) with OR=23 (95%CI
3.1;225.2). Conclusion The efficacy of cyclophosphamide therapy in the
management of FRNS is better than in SDNS.
Author: Yulia Iriani, MD;
Taralan Tambunan, MD; Sudigdo Sastroasmoro, MD, PhD
Journal Code: jpkedokterangg050056
