Management of Steroid-Sensitive Nephrotic Syndrome in Transitioning Adults

Introduction to Treatment

The American Journal of Kidney Diseases recommends continuing to treat relapses with standard corticosteroid protocols and implementing steroid-sparing agents for frequently relapsing or steroid-dependent disease, as approximately one-third of patients will have active disease persisting into adulthood 1, 2

For infrequent relapses, initiate prednisone 60 mg/m² or 2 mg/kg daily until achieving complete remission for at least 3 consecutive days, then switch to alternate-day prednisone 40 mg/m² or 1.5 mg/kg for at least 4 weeks 1, 2
For frequently relapsing or steroid-dependent disease, treat relapses with daily prednisone until remission for 3 days, then alternate-day prednisone for at least 3 months 1, 2, 3

The Kidney International guidelines suggest using cyclosporine 4-5 mg/kg/day or tacrolimus 0.1 mg/kg/day as corticosteroid-sparing agents, with regular monitoring to limit nephrotoxicity 1, 2
Cyclophosphamide 2 mg/kg/day for 8-12 weeks is recommended for frequently relapsing SSNS, but should not be started until remission is achieved with corticosteroids 1, 2, 4
Mycophenolate mofetil 1200 mg/m²/day for at least 12 months is suggested as a steroid-sparing agent, with monitoring for gastrointestinal side effects 1, 2, 4
Rituximab 375 mg/m² for 1-4 doses may be considered for steroid-dependent patients with continuing frequent relapses despite optimal combinations of prednisone and other steroid-sparing agents 1, 2, 4, 5

Regular monitoring of serum creatinine and kidney function is necessary for patients on calcineurin inhibitors to distinguish nephrotoxicity from disease progression 5, 6
Weekly complete blood counts and monitoring for hemorrhagic cystitis are necessary during cyclophosphamide treatment 4, 5

Patients should be educated on home urine dipstick monitoring for early relapse detection and counseled regarding infection prevention, as upper respiratory infections commonly trigger relapses 2, 3, 5