Eculizumab and Refractory Membranoproliferative Glomerulonephritis

• Published in: The New England journal of medicine Vol. 366; no. 12; pp. 1165 - 1166
• Main Authors: Thorner, Paul, Lunn, Andrew, Kirschfink, Michael, Radhakrishnan, Seetha, Hebert, Diane, Langlois, Valerie, Pluthero, Fred, Licht, Christoph
• Format: Journal Article
• Language: English
• Published: United States Massachusetts Medical Society 22.03.2012
• Subjects: Adolescent; Anemia; Antibodies, Monoclonal, Humanized - administration & dosage; Antibodies, Monoclonal, Humanized - therapeutic use; Antibodies, Monoclonal, Murine-Derived - therapeutic use; Complement C3 - analysis; Complement C5 - antagonists & inhibitors; Complement C5 - immunology; Complement Membrane Attack Complex - analysis; Creatinine - blood; Female; Glomerulonephritis; Glomerulonephritis, Membranoproliferative - blood; Glomerulonephritis, Membranoproliferative - drug therapy; Humans; Kidney - immunology; Kidney - pathology; Rituximab
• ISSN: 0028-4793; 1533-4406; 1533-4406
• DOI: 10.1056/NEJMc1106619

This letter to the editor discusses the use of eculizumab in refractory membranoproliferative glomerulonephritis. The response appears to be dramatic. To the Editor: Membranoproliferative glomerulonephritis (MPGN) mainly affects children, often progresses to end-stage kidney disease, and can recur after transplantation. 1 Primary MPGN is one of several glomerulopathies that have recently been linked to dysregulation of the complement alternative pathway, 2 , 3 which triggers the cytotoxic terminal complement response. Current therapeutic strategies for reestablishing complement regulation and preventing the effects of an overactive terminal complement cascade include plasma infusion and exchange and terminal complement blockade through the anti-C5 monoclonal antibody eculizumab. 4 , 5 We describe a patient with refractory MPGN who had a dramatic response to eculizumab treatment. A 16-year-old girl presented with . . .