The treatment of relapse in adults with minimal change nephrotic syndrome : myths and facts

Abstract EN

Few controlled trials have studied the treatment of relapse in adults with minimal change disease.

Repeated courses of steroids, cyclophosphamide, cyclosporine and even mycophenolate mofetil (MMF), all seem to play a role.

The aim of this study was to review and critically analyze the literature regarding the use of immunosuppressive therapy for the treatment of relapse in adults with minimal change nephrotic syndrome (MCNS).

An intensive search was done for published trials in the general medical database.

Retrieved studies were further sorted according to specific inclusion and exclusion criteria.

Selected trials were critically analyzed and evaluated using the Oxford Centre for Evidence-based Medicine Levels of Evidence, 2009 rating.

Six studies were selected and systematically reviewed.

One randomized controlled trial compared the use of cyclophosphamide versus cyclosporine (11 adults) and showed that both drugs are effective in the treatment of frequent relapses [level 1b evidence (grade B)].

Three trials (total of 20 patients) tested the use of cyclosporine therapy and showed that cyclosporine, though effective in the treatment of relapse, is associated with an extremely high incidence of subsequent relapses following drug withdrawal.

A long-term follow-up cross-sectional study of 95 patients, with 69 relapsers, supported the use of steroids in the treatment of occasional relapses [level 2c evidence (grade C)].

One case series described the benefits of MMF [level 4 evidence (grade C)].

Most of the the clinical trials studied were heterogeneous, underpowered by small adult populations, open-labelled, non-randomized, with poor statistical analysis, validity and utility.

We conclude that there is poor evidence that successful treatment of the first relapse of adult MCNS can be achieved with a second course of steroids or cyclosporine.

Also, there is weak evidence that frequent relapses can be treated using cyclophosphamide, cyclosporine or MMF.

Powered, multi-centered, randomized, blinded, controlled trials, with long-term follow-up are required to know the optimal treatment for relapsing adult MCNS.