Trial reportJournal of the American Society of Nephrology : JASN2012
Pathology after eculizumab in dense deposit disease and C3 GN.
Trial report in Journal of the American Society of Nephrology : JASN, 2012. The graph could read no effect estimate from its abstract, so it casts no vote on the map. Cited by 76 papers.
What it found
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The abstract states no effect estimate the extractor could read, or names no intervention and outcome on the map, so this paper lights no cell and moves no belief. It is still indexed, cited and linked below.
The trial behind it
Trials whose registry record cites this paper, or whose number appears in the abstract. A trial that started after this paper was published is citing it as background, not reporting it.
Neither the registry nor the abstract names a trial number. If this is a trial report, that itself is worth knowing.
Who cites it
76 citing papers in PubMed, 149 citations in OpenAlex.
- Safety and Efficacy of Avacopan in Patients with Complement 3 Glomerulopathy: Randomized, Double-Blind Clinical Trial.Journal of the American Society of Nephrology : JASN · 2025Trial
- Eculizumab for dense deposit disease and C3 glomerulonephritis.Clinical journal of the American Society of Nephrology : CJASN · 2012Trial
- Long-term eculizumab treatment failure in C3 glomerulonephritis: two case reports.Clinical kidney journal · 2026Article
- Complement Dysregulation in Kidney Diseases: Mechanisms, Biomarkers, and Emerging Targeted Therapies.International journal of molecular sciences · 2026Review
- Complement-targeted therapies for C3 glomerulopathy and atypical hemolytic uremic syndrome: a time-limited rapid systematic review with narrative synthesis.Frontiers in medicine · 2026Review
- C3 glomerulopathy post kidney transplantation: A single center experience.World journal of transplantation · 2025Article
- Successfully treated C3 glomerulopathy in which protein and genetic analyses were useful for diagnosis.CEN case reports · 2025Article
- The role of complement in normal pregnancy and preeclampsia.Frontiers in immunology · 2025Review
- Congophilic fibrils in the glomeruli with polyclonal immunoglobulin gamma staining - another cause for diagnostic overlap: A case report.World journal of clinical cases · 2024Article
- C3 glomerulopathy: a kidney disease mediated by alternative pathway deregulation.Frontiers in nephrology · 2024Review
- An update of proliferative glomerulonephritis with monoclonal immunoglobulin deposits.Clinical kidney journal · 2022Review
- Myeloperoxidase immunohistochemical staining can identify glomerular endothelial cell injury in dense deposit disease.Pediatric nephrology (Berlin, Germany) · 2021Article
- Homodimeric Minimal Factor H:Frontiers in immunology · 2021Article
- Renal diseases and the role of complement: Linking complement to immune effector pathways and therapeutics.Advances in immunology · 2021Review
- Treatment of C3 Glomerulopathy in Adult Kidney Transplant Recipients: A Systematic Review.Medical sciences (Basel, Switzerland) · 2020Article
- Deposition of the Membrane Attack Complex in Healthy and Diseased Human Kidneys.Frontiers in immunology · 2020Review
- Eculizumab for pediatric dense deposit disease: A case report and literature review.Clinical nephrology. Case studies · 2020Article
- Characteristics of membranoproliferative glomerulonephritis based on a new classification at a single center.Clinical and experimental nephrology · 2019Article
- Glomerular membrane attack complex is not a reliable marker of ongoing C5 activation in lupus nephritis.Kidney international · 2019Article
- C3 glomerulonephritis secondary to mutations in factors H and I: rapid recurrence in deceased donor kidney transplant effectively treated with eculizumab.Nephrology, dialysis, transplantation : official publication of the European Dialysis and Transplant Association - European Renal Association · 2018Article
16 more citing papers are in PubMed but not listed here.
Corrections and comments
PubMed lists nothing against this paper. Absence here is not a guarantee, only a check that was made.
Authors and funding
8 authors at 3 institutions in 1 country.
Funding
No grant is acknowledged in the PubMed record.
Abstract
Eculizumab might benefit C3 glomerulopathies mediated by dysregulation of the alternative complement pathway. Here, we report renal biopsy findings before and after eculizumab therapy in three patients with dense deposit disease and two with C3 GN. All pretreatment biopsies had glomerular and tubular basement membrane deposits that stained exclusively for C3 without significant Ig. After 1 year of therapy, there was reduction in active glomerular proliferation and neutrophil infiltration in three of five patients, consistent with effective C5 blockade, which prevents production of chemotactin C5a. One individual with mild mesangial disease had no significant change in activity or chronicity. One patient exhibited persistent activity and worsening chronicity despite therapy. Immunofluorescence showed no significant reduction in C3 or C5b-9, and electron microscopy revealed persistent deposits in all cases, suggesting a long t(1/2) of C5b-9 in extracellular matrix. Normal renal biopsies stained positive for C5b-9 in glomeruli, tubular basement membranes, and vessel walls, albeit at lower intensity than in C3 glomerulopathy. This indication of physiologic levels of C5b-9 activation in normal kidney potentially explains the localization of deposits in patients with dysregulation of the alternative complement pathway. All post-treatment biopsies showed de novo monoclonal staining for IgG-κ in the same distribution as C3 and C5b-9, mimicking monoclonal Ig deposition disease (MIDD). Staining of the γ heavy chain was restricted to the IgG2 and IgG4 subclasses, suggesting the binding of monoclonal eculizumab to C5 in renal tissues. The long-term effects of this apparent drug-tissue interaction are unknown.
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Registered trials
Read under generation 80e0d062 · epoch 390. Bibliography from PubMed, PubMed Central and OpenAlex; grants from NIH RePORTER; trial links from ClinicalTrials.gov; estimates, votes and beliefs from the OpenQuestion graph.