Complete remission of nephrotic syndrome in a young woman with anti-LRP2 nephropathy after immunosuppressive therapy

Abstract

Abstract Background Anti-low density lipoprotein receptor-related protein 2 (LRP2) nephropathy/anti-brush border antibody (ABBA) disease is a disorder characterized by acute tubulointerstitial injury associated with circulating antibodies to kidney proximal tubular brush border protein LRP2/megalin. Patients are typically elderly and present with acute kidney injury and subnephrotic proteinuria. They progress to end-stage renal disease with poor response to immunosuppressive therapies. Case presentation We report a case of a 29-year-old Chinese woman, who presented with nephrotic syndrome with normal kidney function. Kidney biopsy showed no obvious tubular injury or interstitial inflammation. Positive immunoglobulin G (IgG) staining was revealed along the brush border of proximal tubular cells. Anti-LRP2 antibody was identified in serum, consistent with a diagnosis of anti-LRP2 nephropathy. The patient achieved complete remission after receiving prednisone and cyclophosphamide. Conclusions Anti-LRP2 nephropathy can also present as nephrotic syndrome in young patients and complete remission from nephrotic syndrome may be achieved after immunosuppressive therapy.

Keywords

• Anti-LRP2 nephropathy
• Anti-brush border antibody
• Kidney biopsy
• LDL receptor-related protein 2
• Renal pathology
• Megalin