Treatment for Frequent Relapse Nephrotic Syndrome

Initial Management

The Kidney International recommends treating the current relapse with oral prednisone at 60 mg/m² per day until remission, then decreasing to 40 mg/m² per day on alternate days for 1 week, and tapering by 10 mg/m² per week to complete a total of 4 weeks 1, 2, 3, 4
Consider albumin infusion only if there are clinical indicators of hypovolemia, rather than based solely on the low albumin level, and administer furosemide at the end of the infusion to enhance diuresis 5

Administer rituximab as a single intravenous infusion of 375 mg/m², with premedication with acetaminophen and an antihistamine, and monitoring for infusion reactions during administration, and consider a second dose after 1-3 months if B-cell recovery occurs with early signs of relapse 1
The KDIGO 2021 guidelines recommend rituximab as an appropriate steroid-sparing agent for children with steroid-dependent nephrotic syndrome, and it has shown effectiveness in both frequently relapsing and steroid-dependent forms of nephrotic syndrome 1, 2

A kidney biopsy should be considered given the frequent relapses and severe hypoalbuminemia, to help guide further therapy 1, 3
If rituximab is not available or contraindicated, consider alternative therapies such as calcineurin inhibitors, mycophenolate mofetil, cyclophosphamide, or levamisole, in order of preference 1, 3

After rituximab administration, check CD19/CD20 B-cell counts every 1-3 months to monitor for B-cell recovery and potential need for a second dose 1
The absence of a previous biopsy is concerning after multiple relapses, and histological confirmation should be considered to rule out other glomerular pathologies 1, 3

Severe hypoalbuminemia, such as an albumin level of 1.5, may contribute to frequent relapses in nephrotic syndrome, according to the Nature Reviews Nephrology 6
The presence of severe hypoalbuminemia, such as an albumin level of 1.5, may require consideration of albumin infusion, but only if there are clinical indicators of hypovolemia, as suggested by a study published in Nature Reviews Nephrology 6

The Nature Reviews Nephrology suggests that ambulatory management should be considered when the child is stable to increase quality of life and decrease the risk of nosocomial infections 6