A Case of C3 Glomerulonephritis Successfully Treated with Eculizumab

Overview

Journal Pediatr Nephrol

Specialties Nephrology
Pediatrics

Date 2015 Mar 23

PMID 25796589

Citations 16

Authors

Alexis Payette

Natalie Patey

Marie-Agnes Dragon-Durey

Veronique Fremeaux-Bacchi

Francoise Le Deist

Anne-Laure Lapeyraque

Affiliations

Soon will be listed here.

Abstract

Background: C3 glomerulonephritis (C3GN) is a rare form of glomerulopathy that is characterized by predominant C3 deposits. Eculizumab, a humanized monoclonal C5 antibody, has recently emerged as a treatment option for C3GN. We report a C3GN patient successfully treated with eculizumab.

Case Diagnosis/treatment: A 5-year-old boy who presented with proteinuria, hematuria, high ASO titers, and low C3 levels was initially diagnosed with post-streptococcal GN. His first kidney biopsy confirmed this diagnosis, but complement investigations identified three alternative pathway dysregulation factors: C3 nephritic factor, complement factor I heterozygous mutation (I398L), and anti-factor H autoantibodies (4,500 AU/ml). A second biopsy performed 11 months after initial presentation (nephrotic range proteinuria) showed a C3GN suggestive of isolated C3 deposits. Despite the use of intensive immunosuppressive therapy (rituximab, corticosteroids, mycophenolate), nephrotic-range proteinuria persisted and a third kidney biopsy showed the same C3GN pattern with more endocapillary proliferation. The serum C5b-9 level was elevated. Eculizumab was initiated and resulted in a significant decline of proteinuria (5.3 to 1.3 g/day) and an improvement in pathologic features. A transient interruption of eculizumab resulted in a rapid rise in proteinuria to 9.3 g/day, which decreased to 0.8 g/day after resumption of treatment.

Conclusions: The administration of anti-C5 antibodies may represent a valuable therapeutic option in patients with C3GN.

Citing Articles

Complement Terminal Pathway Activation and Intrarenal Immune Response in C3 Glomerulopathy.

Meuleman M, Petitprez F, Pickering M, le Quintrec M, Artero M, DuVal A J Am Soc Nephrol. 2024; 35(8):1034-1044.

PMID: 38709564 PMC: 11377803. DOI: 10.1681/ASN.0000000000000373.

Contemporary Monoclonal Antibody Utilization in Glomerular Diseases.

Mansour I, Murugapandian S, Tanriover B, Thajudeen B Mayo Clin Proc Innov Qual Outcomes. 2023; 7(4):276-290.

PMID: 37448529 PMC: 10338194. DOI: 10.1016/j.mayocpiqo.2023.04.009.

Renal diseases and the role of complement: Linking complement to immune effector pathways and therapeutics.

Freiwald T, Afzali B Adv Immunol. 2021; 152:1-81.

PMID: 34844708 PMC: 8905641. DOI: 10.1016/bs.ai.2021.09.001.

Deposition of the Membrane Attack Complex in Healthy and Diseased Human Kidneys.

Koopman J, van Essen M, Rennke H, de Vries A, van Kooten C Front Immunol. 2021; 11:599974.

PMID: 33643288 PMC: 7906018. DOI: 10.3389/fimmu.2020.599974.

Eculizumab for pediatric dense deposit disease: A case report and literature review.

Kasahara K, Gotoh Y, Majima H, Takeda A, Mizuno M Clin Nephrol Case Stud. 2020; 8:96-102.

PMID: 33329990 PMC: 7737524. DOI: 10.5414/CNCS110309.

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