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Kidney Week

Abstract: PO1909

Recurrent Nephrotic Syndrome in Podocyte Infolding Glomerulopathy: Remission with Rituximab

Session Information

Category: Trainee Case Report

  • 1203 Glomerular Diseases: Clinical, Outcomes, and Trials

Authors

  • Hodgins, Spencer, Baystate Medical Center, Springfield, Massachusetts, United States
  • Greco, Barbara A., Baystate Medical Center, Springfield, Massachusetts, United States

Group or Team Name

  • Baystate Medical Center Renal Fellowship
Introduction

Podocyte infolding glomerulopathy (PIG) is a recently recognized entity. The sentinel paper showed that it is associated with autoimmune disorders and is often responsive to corticosteroids (CS). We present a case of PIG with recurrent nephrotic syndrome (NS) who is maintaining remission on Rituximab off of CS.

Case Description

A 61-year old woman with type II diabetes mellitus, inflammatory bowel disease (IBD), and uveitis presented in 2009 with proteinuria. Kidney biopsy was read as focal segmental glomerulosclerosis (FSGS). She was treated with angiotensin converting enzyme inhibitor (ACE I) and prednisone, and proteinuria remitted. In 2012, an increase in proteinuria promped repeat biopsy resulting in a diagnosis of membranous glomerulonephritis. Anti-PLA2R was negative. ACE I and prednisone were continued and proteinuria remitted. In 2014,she developed nephrotic syndrome (NS). Repeat biopsy was read as showing FSGS. Prednisone 1 mg/kg/day induced remission. When prednisone was tapered, NS flared. She did not remit with high dose prednisone and at 8 weeks developed acute kidney injury (AKI). Tacrolimus was added and proteinuria and creatinine (cr) improved. Steroids were tapered over several months. On Humira for IBD and tacrolimus (level 11 mg/dl), NS and AKI flared 1 year later with urine protein:creatinine (UP/C) of 2 and cr 2.1 mg/dl. Prednisone was restarted. Intravenous cyclosphosphamide was started, steroids were tapered, and Humira was discontinued. Within 5 weeks, proteinuria worsened (UP/C 29) and cr rose to 4 mg/dl. Renal biopsy demonstrated foot process invagination yielding a diagnosis of PIG. At trial of cyclosporine was stopped at 1 week due to drug intolerance. Mycophenolate mofetil (MMF) in combination with prednisone and tacrolimus was started. MMF caused severe diarrhea. In September of 2019, 4 weekly doses of Rituximab 375 mg/m2 followed by 1 gram doses at 4 month intervals were administered. Proteinuria remitted and creatinine improved to 1 mg/dl. She remains in remission off of steroids and on lower dose tacrolimus now for 11 months.

Discussion

This case is the first to describe the effectiveness of Rituxan as rescue and maintenance therapy following failure of other IS regimens in a patient with recurrent NS due to PIG. Rituxan should be considered in refractory cases of PIG to induce and maintain remission and allow for steroid sparing.