What are the second line treatment for nephrotic syndrome?
The choice of therapy in patients with steroid-resistant nephrotic syndrome is a highly contentious topic because of a lack of sufficiently powered clinical trials. Alternative therapies are applied in MCNS in children who are suffering intolerable side effects from steroids.
These patients respond well to mycophenolate mofetil, cyclophosphamide, or calcineurin inhibitors, such as cyclosporine or tacrolimus.
Cyclosporine is the only agent that has been proven to be superior to placebo in patients with FSGS. In those with MN, combined therapy with steroids and an alkylating agent is effective in patients at high risk of progressive deterioration in kidney function.
There are ongoing trials to assess the efficacy of calcineurin inhibitors (CNIs) and rituximab in the treatment of MN. In those with MPGN, a regimen involving steroids, antiplatelet drugs and an alkylating agent has been utilized with uncertain efficacy.
The role of inhibitors of the alternative pathway of complement, such as eculizumab in patients with type II MPGN, remains to be determined.