ArticleAmerican journal of kidney diseases : the official journal of the National Kidney Foundation2017
C4 Nephritic Factors in C3 Glomerulopathy: A Case Series.
Article in American journal of kidney diseases : the official journal of the National Kidney Foundation, 2017. The graph could read no effect estimate from its abstract, so it casts no vote on the map. Cited by 33 papers.
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Who cites it
33 citing papers in PubMed, 50 citations in OpenAlex.
- Distinct Pharmacodynamic Complement Biomarker Signatures Define Iptacopan and Pegcetacoplan Treatment in C3 Glomerulopathy.Kidney international reports · 2026Article
- C3 glomerulopathy in children: experience at a resource-limited center.Clinical and experimental pediatrics · 2025Article
- Acquired drivers of C3 glomerulopathy.Clinical kidney journal · 2025Review
- Clinical Remission and Reduction of Circulating Nephritic Factors by Combining Rituximab With Belimumab in a Case of Complement Factor 3 Glomerulopathy.Kidney international reports · 2024Article
- Defining Nephritic Factors as Diverse Drivers of Systemic Complement Dysregulation in C3 Glomerulopathy.Kidney international reports · 2024Article
- Efficacy and Safety of Iptacopan in Patients With C3 Glomerulopathy.Kidney international reports · 2023Article
- Clinico-Pathogenic Similarities and Differences between Infection-Related Glomerulonephritis and C3 Glomerulopathy.International journal of molecular sciences · 2023Review
- Stage II of Chronic Kidney Disease-A Tipping Point in Disease Progression?Biomedicines · 2022Article
- Association between HLA alleles and sub-phenotype of childhood steroid-sensitive nephrotic syndrome.World journal of pediatrics : WJP · 2022Article
- Challenges in diagnostic testing of nephritic factors.Frontiers in immunology · 2022Review
- Complement C4, Infections, and Autoimmune Diseases.Frontiers in immunology · 2021Review
- Detection of C3 Nephritic Factor by Hemolytic Assay.Methods in molecular biology (Clifton, N.J.) · 2021Article
- Gain-of-Function Mutations R249C and S250C in Complement C2 Protein Increase C3 Deposition in the Presence of C-Reactive Protein.Frontiers in immunology · 2021Article
- FHR-5 Serum Levels andFrontiers in immunology · 2021Article
- The Benefits of Complement Measurements for the Clinical Practice.Methods in molecular biology (Clifton, N.J.) · 2021Review
- Different Aspects of Classical Pathway Overactivation in Patients With C3 Glomerulopathy and Immune Complex-Mediated Membranoproliferative Glomerulonephritis.Frontiers in immunology · 2021Article
- Long-Term Outcomes of C3 Glomerulopathy and Immune-Complex Membranoproliferative Glomerulonephritis in Children.Kidney international reports · 2020Article
- The Immunopathology of Complement Proteins and Innate Immunity in Autoimmune Disease.Clinical reviews in allergy & immunology · 2020Review
- Validation of distinct pathogenic patterns in a cohort of membranoproliferative glomerulonephritis patients by cluster analysis.Clinical kidney journal · 2020Article
- Clinicopathological features of C3 glomerulopathy in children: a single-center experience.Pediatric nephrology (Berlin, Germany) · 2020Article
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Authors and funding
12 authors at 6 institutions in 2 countries.
Funding
Abstract
backgroundC3 glomerulopathy (C3G) defines a group of rare complement-mediated kidney diseases with a shared underlying pathophysiology: dysregulation of complement in the fluid phase and glomerular microenvironment. Dysregulation can be driven by autoantibodies to C3 and C5 convertases. STUDY
designCase series. SETTING &
participants168 patients with C3G (dense deposit disease, 68; C3 glumerulonephritis, 100) selected from our C3G biobank. OUTCOMES: Patient-purified immunoglobulin Gs were tested for C4 nephritic factors (C4NeFs). These autoantibodies recognize C4b2a, the C3 convertase of the classical pathway of complement. MEASUREMENTS: C4NeFs were detected using a modified hemolytic assay.
resultsC4NeFs were identified in 5 patients, 4 of whom had C3 glomerulonephritis. C4NeFs were associated with dysregulation of C3 and C5 convertases, and they appear to stabilize these convertases in a dose-dependent manner. C4NeFs also appear to protect C4b2a from decay mediated by soluble CR1 and C4 binding protein. The stabilizing activity of the autoantibodies was further demonstrated by using heat treatment to inactivate complement. C4NeFs were not detected in 150 patients with another complement-mediated kidney disease, atypical hemolytic uremic syndrome. They were also absent in 300 apparently healthy controls. LIMITATIONS: In addition to C4NeFs, 2 patients had positive findings for other autoantibodies: one patient also had autoantibodies to factor H; the other patient also had autoantibodies to C3bBb (C3NeFs).
conclusionsThe finding of C4NeFs in a small percentage of patients with C3G highlights the challenge in identifying autoantibodies that drive complement dysregulation and underscores the complexity of the autoantibody repertoire that can be identified in these patients.
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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.