Eculizumab in a Patient with Dense-Deposit Disease

• Published in: The New England journal of medicine Vol. 366; no. 12; pp. 1161 - 1163
• Main Authors: Daina, Erica, Remuzzi, Giuseppe, Noris, Marina
• Format: Journal Article
• Language: English
• Published: United States Massachusetts Medical Society 22.03.2012
• Subjects: Adolescent; Antibodies, Monoclonal, Humanized - administration & dosage; Antibodies, Monoclonal, Humanized - therapeutic use; Antibodies, Monoclonal, Murine-Derived - therapeutic use; Biopsy; Complement C5 - antagonists & inhibitors; Complement C5 - genetics; Complement C5 - immunology; Complement Membrane Attack Complex - analysis; Complement Membrane Attack Complex - antagonists & inhibitors; Creatinine - blood; Female; Glomerulonephritis; Glomerulonephritis, Membranoproliferative - blood; Glomerulonephritis, Membranoproliferative - drug therapy; Glomerulonephritis, Membranoproliferative - genetics; Humans; Kidneys; Nephrotic syndrome; Proteins; Rituximab; Rodents
• ISSN: 0028-4793; 1533-4406; 1533-4406
• DOI: 10.1056/NEJMc1112273

This letter indicates that in a patient with long-standing dense-deposit disease, a variant of membranoproliferative glomerulonephritis, eculizumab can be useful in the resolution of the nephrotic syndrome. To the Editor: In 1999, an 11-year-old girl with the nephrotic syndrome received a biopsy-confirmed diagnosis of membranoproliferative glomerulonephritis. The level of plasma complement C3 was 7 mg per deciliter (normal range, 90 to 180). Glucocorticoids, administered for 5 years, were ineffective. We first saw the patient at that point. She had nephrotic-range proteinuria (urinary protein excretion, 4.8 g per 24 hours), a low level of C3 (12 mg per deciliter), and normal renal function. We withdrew glucocorticoid therapy and initiated treatment with ramipril plus losartan. She continued to have nephrotic-range proteinuria. Electron microscopy of a second biopsy specimen showed . . .