Eculizumab as a therapeutic approach for severe crescentic recurrence of immunoglobulin A nephropathy after kidney transplantation

Am J Transplant. 2023 Oct;23(10):1626-1630. doi: 10.1016/j.ajt.2023.05.031. Epub 2023 May 31.

Abstract

Crescentic forms of immunoglobulin A nephropathy (IgAN) are rare but can be associated with rapid kidney failure and a high rate of end-stage renal disease despite immunosuppression therapy. Complement activation has emerged as a key driver of glomerular injury in IgAN. Therefore, complement inhibitors may be a rational treatment option in patients unresponsive to first-line immunosuppressive therapy. Here, we describe the case of a 24-year-old woman presenting with crescentic IgAN recurrence a few months after living kidney transplantation. Considering the dramatic graft failure accompanied by malignant hypertension and thrombotic microangiopathy features worsening after a first-line of high-dose steroids and 3 sessions of plasma exchanges, eculizumab was started as a rescue therapy. For the first time, the clinical response to eculizumab was highly successful, with a complete graft recovery without any relapse after 1 year of treatment. Further clinical studies are strongly needed to specify which patients might benefit from terminal complement blockade.

Keywords: IgA nephropathy; complement; glomerulonephritis; recurrence; transplantation.

Publication types

  • Case Reports
  • Research Support, Non-U.S. Gov't

MeSH terms

  • Adult
  • Antibodies, Monoclonal, Humanized / therapeutic use
  • Female
  • Glomerulonephritis, IGA* / complications
  • Glomerulonephritis, IGA* / drug therapy
  • Humans
  • Immunosuppressive Agents / therapeutic use
  • Kidney Transplantation* / adverse effects
  • Recurrence
  • Young Adult

Substances

  • eculizumab
  • Antibodies, Monoclonal, Humanized
  • Immunosuppressive Agents