Treatment course of steroid-dependent nephrotic syndrome: emphasized on treatment effect

Children with steroid-dependent nephrotic syndrome (SDNS) need long-term steroid usage to maintain sustained remission. Cyclophosphamide is a well-known alternative agent to spare the use of steroids and avoid the side-effects that result from long-term steroid therapy. Most children may continue to have SDNS despite receiving cyclophosphamide. Additional alternative drugs may be needed. In the present study, the effects on SDNS of sequential treatment after cyclophosphamide usage were established.

Forty-six children with SDNS were enrolled in this retrospective uncontrolled study. In addition to prednisolone, patients were treated with cyclophosphamide as a first-line alternative drug. Children who still had SDNS despite cyclophosphamide therapy received chlorambucil, levamisole or another course of cyclophosphamide. The treatment responses were recorded and the mean duration of follow up was 96 months.

Seventeen patients (37%) experienced no relapse after cyclophosphamide therapy. Twenty-five patients (54%) had varied responses. Only four patients showed no effect. Children who still had SDNS despite cyclophosphamide therapy received second or more alternative drugs. Cyclophosphamide with or without chlorambucil resolved steroid-dependency in 33 of 46 (72%) children who either had complete remission or developed steroid-sensitive, rather than steroid-dependent, nephrotic syndrome.

With the exception of four patients who were lost to follow up and four who were refractory and needed other treatment, most children with SDNS could spare the steroid (complete remission or steroid sensitive nephrotic syndrome) after using one or more of these modulating agents.