ArticleBMC nephrology2018
Treating C3 glomerulopathy with eculizumab.
Article in BMC nephrology, 2018. The graph could read no effect estimate from its abstract, so it casts no vote on the map. Cited by 27 papers.
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Who cites it
27 citing papers in PubMed, 58 citations in OpenAlex.
- C3 Glomerulonephritis Associated With Anti-complement Factor B Antibodies Following Anti-cancer Treatment With Pembrolizumab.Kidney medicine · 2026Article
- Mechanistic Insights and Therapeutic Advances of Anti-Inflammatory Biologics in Immune-Mediated Glomerulonephritis: A Narrative Review.Journal of inflammation research · 2026Review
- Eculizumab in C3 Glomerulopathy: A Systematic Review of Therapeutic Efficacy and Clinical Outcomes.Pharmaceutics · 2025Review
- Improved therapeutic efficacy of a bifunctional anti-C5 mAb-FH SCR1-5 fusion protein over anti-C5 mAb in an accelerated mouse model of C3 glomerulopathy.ImmunoHorizons · 2025Article
- Complement Cascade Proteins Correlate with Fibrosis and Inflammation in Early-Stage Type 1 Diabetic Kidney Disease in the Ins2Akita Mouse Model.International journal of molecular sciences · 2024Article
- Clinical advances in immunotherapy for immune-mediated glomerular diseases.Clinical and experimental medicine · 2023Review
- Eculizumab as a treatment for C3 glomerulopathy: a single-center retrospective study.BMC nephrology · 2023Article
- Low-molecular weight inhibitors of the alternative complement pathway.Immunological reviews · 2023Review
- C3 glomerulopathies: dense deposit disease and C3 glomerulonephritis.Frontiers in medicine · 2023Review
- Application of C5 inhibitors in glomerular diseases in 2021.Kidney research and clinical practice · 2022Article
- Repository Corticotropin in Treating de novo C3 Glomerulonephritis after Transplantation.Glomerular diseases · 2022Article
- A clinical approach to children with C3 glomerulopathy.Pediatric nephrology (Berlin, Germany) · 2022Review
- Murine Factor H Co-Produced in Yeast With Protein Disulfide Isomerase Ameliorated C3 Dysregulation in Factor H-Deficient Mice.Frontiers in immunology · 2021Article
- Article
- Homodimeric Minimal Factor H:Frontiers in immunology · 2021Article
- Long-Term Outcomes of C3 Glomerulopathy and Immune-Complex Membranoproliferative Glomerulonephritis in Children.Kidney international reports · 2020Article
- Use of Bortezomib in the Treatment of C3 Glomerulonephritis Refractory to Eculizumab and Rituximab.Kidney international reports · 2020Article
- A Narrative Review on C3 Glomerulopathy: A Rare Renal Disease.International journal of molecular sciences · 2020Review
- Complement, a Therapeutic Target in Diabetic Kidney Disease.Frontiers in medicine · 2020Review
- Diverse Clinical Presentations of C3 Dominant Glomerulonephritis.Frontiers in medicine · 2020Article
Corrections and comments
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Authors and funding
8 authors at 1 institution in 1 country.
Funding
No grant is acknowledged in the PubMed record.
Abstract
backgroundC3 glomerulopathy (C3G) is a rare, but severe glomerular disease with grim prognosis. The complex pathogenesis is just unfolding, and involves acquired as well as inherited dysregulation of the alternative pathway of the complement cascade. Currently, there is no established therapy. Treatment with the C5 complement inhibitor eculizumab may be a therapeutic option. However, due to rarity of the disease, parameters predicting treatment response remain largely unknown.
methodsSeven patients with C3G (five with C3 glomerulonephritis and two with dense deposit disease) were treated with eculizumab. Subjects underwent biopsy before enrollment. The histopathology, clinical data, and response to eculizumab treatment were analyzed. The key parameters to determine outcome were changes of serum creatinine and urinary protein over time.
resultsAfter treatment with eculizumab, four subjects showed significantly improved or stable renal function and urinary protein. A positive response occurred between 2 weeks and 6 months after therapy initiation. One subject (with allograft recurrent C3 glomerulonephritis) initially showed a positive response, but relapsed when eculizumab was discontinued, and did not respond after re-initiation of treatment. Two subjects showed impaired renal function and increasing urinary protein despite therapy with eculizumab.
conclusionsEculizumab may be a therapeutic option for a subset of C3G patients. The response to eculizumab is heterogeneous, and early as well as continuous treatment may be necessary to prevent disease progression. These findings emphasize the need for studies identifying genetic and functional complement abnormalities that may help to guide eculizumab treatment and predict response.
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