Association between the time of initial relapse and subsequent relapses in patients with childhood-onset idiopathic nephrotic syndrome.

Abstract

Nephrotic syndrome relapse within 6months is a known risk factor for steroid-dependent nephrotic syndrome/frequently relapsing nephrotic syndrome (SDNS/FRNS), but the risk of early development of SDNS/FRNS and initiation of immunosuppression therapy remains unknown. Patients with childhood-onset idiopathic nephrotic syndrome who had the first relapse within 6months were enrolled. We analyzed the relationship between the time of the first relapse or the time of initial remission and incidence of SDNS/FRNS or initiation of immunosuppression therapy. Forty-five patients were enrolled. Twenty out of 23 patients (87%) with the first relapse within 30days after discontinuing initial steroid therapy experienced a second relapse within 30days after discontinuing steroid therapy. Additionally, most patients in this group (96%) experienced a second relapse within 6months after the onset and were diagnosed as SDNS/FRNS at this time. In this group, the incidence of SDNS/FRNS development within 6months was 96%. In contrast, the incidence of SDNS/FRNS development within 6months was 18% in patients with the first relapse more than 30days after steroid discontinuation. The incidence of initiation of immunosuppressive agents within 6months was 83% in the former group and 14% in the latter group. Most patients with the first relapse within 30days after discontinuing steroid therapy developed SDNS/FRNS and were administered immunosuppressive agents within 6months. Thus, it might be reasonable to start immunosuppression therapy in this group without waiting for the second relapse.