Uma forma de glomerulonefrite membranoproliferativa primária caracterizada pela presença em amostras de biópsia renal de uma glomerulonefrite com coloração de imunofluorescência glomerular única (ou pelo menos dominante) para C3. Alterações inespecíficas ou padrões proliferativos com coloração glomerular C3-dominante também são possíveis de ocorrer. Com base nos achados da microscopia eletrónica, a glomerulopatia C3 (C3G) pode ser classificada como glomerulonefrite C3 (C3GN) ou doença de depósito denso (DDD).
Introdução
O que você precisa saber de cara
Uma forma de glomerulonefrita membranoproliferativa primária descrita pela presença em amostras de biópsia renal de uma glomerulonefrita com coloração de imunofluorescência glomerular única (ou pelo menos dominante) para C3. Alterações inespecíficas ou padrões proliferativos com coloração glomerular C3-dominante também são possíveis de ocorrer. Com base nos achados da microscopia eletrônica, a glomerulopatia C3 (C3G) pode ser encontrada como glomerulonefrite C3 (C3GN) ou doença de depósito denso (DDD).
Escala de raridade
<1/50kMuito rara
1/20kRara
1/10kPouco freq.
1/5kIncomum
1/2k
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Sinais e sintomas
O que aparece no corpo e com que frequência cada sintoma acontece
Partes do corpo afetadas
+ 11 sintomas em outras categorias
Características mais comuns
Os sintomas variam de pessoa para pessoa. Abaixo estão as 34 características clínicas mais associadas, ordenadas por frequência.
Linha do tempo da pesquisa
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Genética e causas
O que está alterado no DNA e como passa nas famílias
Genes associados
5 genes identificados com associação a esta condição. Padrão de herança: Multigenic/multifactorial.
Involved in complement regulation. The dimerized forms have avidity for tissue-bound complement fragments and efficiently compete with the physiological complement inhibitor CFH
Secreted
Glycoprotein that plays an essential role in maintaining a well-balanced immune response by modulating complement activation. Acts as a soluble inhibitor of complement, where its binding to self markers such as glycan structures prevents complement activation and amplification on cell surfaces (PubMed:21285368, PubMed:21317894, PubMed:25402769). Accelerates the decay of the complement alternative pathway (AP) C3 convertase C3bBb, thus preventing local formation of more C3b, the central player of
Secreted
Basal laminar drusen
Drusen are extracellular deposits that accumulate below the retinal pigment epithelium on Bruch membrane. Basal laminar drusen refers to an early adult-onset drusen phenotype that shows a pattern of uniform small, slightly raised yellow subretinal nodules randomly scattered in the macula. In later stages, these drusen often become more numerous, with clustered groups of drusen scattered throughout the retina. In time these small basal laminar drusen may expand and ultimately lead to a serous pigment epithelial detachment of the macula that may result in vision loss.
Involved in complement regulation. The dimerized forms have avidity for tissue-bound complement fragments and efficiently compete with the physiological complement inhibitor CFH. Can associate with lipoproteins and may play a role in lipid metabolism
Secreted
Hemolytic uremic syndrome, atypical, 1
An atypical form of hemolytic uremic syndrome. It is a complex genetic disease characterized by microangiopathic hemolytic anemia, thrombocytopenia, renal failure and absence of episodes of enterocolitis and diarrhea. In contrast to typical hemolytic uremic syndrome, atypical forms have a poorer prognosis, with higher death rates and frequent progression to end-stage renal disease.
Might be involved in complement regulation
Secreted
Hemolytic uremic syndrome, atypical, 1
An atypical form of hemolytic uremic syndrome. It is a complex genetic disease characterized by microangiopathic hemolytic anemia, thrombocytopenia, renal failure and absence of episodes of enterocolitis and diarrhea. In contrast to typical hemolytic uremic syndrome, atypical forms have a poorer prognosis, with higher death rates and frequent progression to end-stage renal disease.
Involved in complement regulation. The dimerized forms have avidity for tissue-bound complement fragments and efficiently compete with the physiological complement inhibitor CFH. Can associate with lipoproteins and may play a role in lipid metabolism
Secreted
Variantes genéticas (ClinVar)
92 variantes patogênicas registradas no ClinVar.
Vias biológicas (Reactome)
1 via biológica associada aos genes desta condição.
Diagnóstico
Os sinais que médicos procuram e os exames que confirmam
Tratamento e manejo
Remédios, cuidados de apoio e o que precisa acompanhar
Onde tratar no SUS
Hospitais de referência no Brasil e o protocolo oficial do SUS (PCDT)
🇧🇷 Atendimento SUS — Glomerulopatia C3
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Dados de DATASUS/CNES, SBGM, ABNeuro e Ministério da Saúde. Sempre confirme a disponibilidade diretamente com o estabelecimento.
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Ensaios em destaque
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Outros ensaios clínicos
30 ensaios clínicos encontrados, 12 ativos.
Publicações mais relevantes
Treatment of atypical hemolytic uremic syndrome and C3 glomerulopathy in mice by hepatic expression of factor D.
Atypical hemolytic uremic syndrome (aHUS) and C3 glomerulopathy (C3G) are diseases driven by dysregulation of the alternative pathway (AP) complement. Current treatments of these diseases involve the use of frequent and often large doses of monoclonal antibodies, peptide inhibitors, or chemical inhibitors of complement proteins, with various limitations. In this study, we describe ectopic expression of factor D (FD) in the liver as a potential novel therapy for these indications. Unlike most plasma complement proteins, which are synthesized in the liver, FD, a serine protease in the AP, is synthesized primarily in fat tissues. Newly synthesized FD is released largely as an inactive proenzyme that requires activation by mannose-binding lectin-associated serine protease 3 (MASP3) to form the mature enzyme. We found serendipitously that ectopic expression of mature FD, but not pro-FD, in the mouse liver led to C3-dependent depletion of plasma factor B (FB) and abolished AP complement activity. Coexpression of C3, FB, and mature FD in cultured Hepa1-6 cells caused intracellular FB activation and consumption, probably within the secretory vesicles where all 3 proteins may colocalize. Hepatic FD-mediated local FB activation and depletion effectively prevented disease development in murine models of aHUS and C3G. Our results suggest that segregated tissue production of FD, FB, and C3, as well as FD regulation by MASP3, is necessary to prevent in situ FB activation and depletion. Ectopic expression of mature FD in the liver is an effective way to inhibit FB expression and AP complement activity, with potential therapeutic applications in diseases such as aHUS and C3G.
Clinical Profile and Outcome of C3-Dominant Glomerulonephritis: Retrospective Study.
Complement 3 (C3)-dominant glomerulonephritis (GN) are rare diseases resulting from alternative complement pathway dysregulation; they include C3 glomerulopathy (C3G), paraprotein-associated GN, and C3-dominant infection-related GN (IRGN). To our knowledge, long-term follow-up studies of clinical profile and outcomes of this rare disorder are sparse. We studied kidney histopathology baseline findings, outcomes, treatment, and its complications of C3-dominant GN in our setting. We studied the clinical, pathological profiles, and outcomes of patients with C3-dominant GN. This study was a single centre, retrospective, case record based observational study at a tertiary care hospital in Southern India. Consecutive patients of C3-dominant GN on kidney biopsy from 2013 to 2023 were included. Demography, laboratory and histopathological data, treatment and outcomes were studied. Of 2,175 kidney biopsies, 141 (6.48%) showed C3-dominant GN; 74 (52.5%) C3G, 67 (47.5%) IRGN. Median age was 43 years (IQR 29-59.5), males 90 (63.8%). Preceding skin/throat infections were seen in 32/141 (22.7%). At presentation, median serum creatinine was 1.7 mg/dL (IQR: 1.2-3.6), eGFR <60 mL/min/1.73 m2 in 91/141 (64.5%), 111/141 (78.7%) had low serum C3 levels. Nephrotic proteinuria was seen in 65/141 (46%), Crescents in 45 (31.9%). Remission was partial in 38/141 (27%), complete in 45/141 (31.9%) and 31/141 (22%) progressed to end-stage kidney disease (ESKD). On immunosuppression, commonest infection was pneumonia in 15/70 (21.4%) and 27/141 (19.1%) died at an average follow-up of 25.7 months. Diabetes mellitus, percentage sclerosis, and presence of crescents predicted development of CKD stage 5. Over 10 years, C3-dominant GN represented 6.48% of kidney biopsies. Nephrotic proteinuria and kidney failure are common at presentation with 58% achieving some remission, 22% going on to ESKD and mortality of 19%.
Pegcetacoplan in C3 Glomerulopathy and Immune-Complex MPGN. Reply.
Pegcetacoplan in C3 Glomerulopathy and Immune-Complex MPGN.
Autoantibodies Targeting Complement Regulating Factors Induced C3 Glomerulopathy Resambling C4 Dense Deposit Disease: A Case Report.
Dense deposit disease (DDD), a subgroup of C3 glomerulopathy, is a rare glomerular disease characterised histologically by predominant C3 staining on immunofluorescence microscopy along with intramembranous osmiophilic dense deposits. C4 DDD is a recently identified form of complement-mediated glomerulonephritis, characterised by intense C4d staining with minimal or absent staining for C3 and immunoglobulin, and dense deposits along the glomerular basement membrane. We present a 27-year-old female patient who exhibited proteinuria during pregnancy. Following delivery, the patient continued to experience persistent non-nephrotic-range proteinuria, along with microscopic hematuria and dramatically decreased serum C3 level, while maintaining preserved renal function. Renal biopsy revealed mesangial proliferative glomerulonephritis with a focal membranoproliferative glomerulonephritis pattern by light microscopy. Electron microscopy demonstrated high-density banded deposits in the glomerular basement membrane, while immunofluorescence exhibited negative C3c and immunoglobulin staining. Immunohistochemical analysis showed diffuse expression of C3d and C4d in the capillary loops, mesangial region, focal tubular basement membrane, and focal arteriolar wall. Further investigation revealed elevated circulating levels of autoantibodies against complement factor H and the C3 convertase. Mass spectrometry confirmed that C3 was the predominant component of the deposits. Ultimately, this patient was diagnosed as DDD mediated by complement factor H autoantibodies and C3 nephritic factor.
Publicações recentes
New Anticomplement Drugs in Nephrology - Mechanism and Indication.
Clinical Images: Anti-MDA5 dermatomyositis with rapidly progressive interstitial lung disease presenting with rapidly progressive glomerulonephritis due to C3 glomerulopathy.
🥉 Relato de casoFinding the etiology of membranoproliferative glomerulonephritis.
A Case Series of Elevated Donor-Derived Cell-Free DNA Leading to Diagnosis of Posttransplant Glomerulonephritis.
Autoantibodies Targeting Complement Regulating Factors Induced C3 Glomerulopathy Resambling C4 Dense Deposit Disease: A Case Report.
📚 EuropePMC325 artigos no totalmostrando 197
Autoantibodies Targeting Complement Regulating Factors Induced C3 Glomerulopathy Resambling C4 Dense Deposit Disease: A Case Report.
Nephrology (Carlton, Vic.)A pediatric case of C3 glomerulonephritis initially misclassified as IgA nephropathy with a favorable response to C3-targeted therapy.
Pediatric nephrology (Berlin, Germany)Thrombosis in paroxysmal nocturnal hemoglobinuria in the complement inhibitor era: mechanisms, risk stratification, and clinical management.
International journal of hematologyRecurrent C3 glomerulopathy after prolonged remission.
Pediatric nephrology (Berlin, Germany)Pegcetacoplan in C3 Glomerulopathy and Immune-Complex MPGN. Reply.
The New England journal of medicinePegcetacoplan in C3 Glomerulopathy and Immune-Complex MPGN.
The New England journal of medicineA novel fusion protein reduces kidney complement in experimental C3 glomerulopathy.
Clinical and experimental immunologyC3 Glomerulopathy Diagnosis, Current Treatments, and Emerging Therapies.
Kidney medicinePrimary membranoproliferative glomerulonephritis: natural history, pathogenesis, and treatment.
Frontiers in nephrologyA young woman with membranoproliferative glomerulonephritis and persistent hypocomplementemia: a case report.
BMC nephrologyReal-World Analysis of Patients With C3 Glomerulopathy in the United States.
Kidney international reports[C3 glomerulopathy: An immune-mediated kidney disease].
MMW Fortschritte der MedizinEfficacy of iptacopan in idiopathic immune complex-mediated membranoproliferative glomerulonephritis refractory to conventional treatment.
NefrologiaMonoclonal gammopathy of renal significance in western China: A large cohort study dominated by amyloidosis with distinct clinical outcomes.
Clinics (Sao Paulo, Brazil)Complement 3 Glomerulopathy Revisited: Bridging Pathogenesis and Therapy in the New Era.
Advances in kidney disease and healthCase Report: Possible C3 nephritic factor-driven complement-mediated severe hemolytic anemia and acute kidney injury in a child with Bordetella parapertussis infection.
Frontiers in immunologyTreatment of atypical hemolytic uremic syndrome and C3 glomerulopathy in mice by hepatic expression of factor D.
Blood advancesCurrent and Emerging Therapies for C3 Glomerulopathy and Primary (Idiopathic) Immune Complex Membranoproliferative Glomerulonephritis.
Kidney international reportsA Decade of C3 Glomerulopathy-A Nationwide Cohort Study.
Kidney international reportsClinical Profile and Outcome of C3-Dominant Glomerulonephritis: Retrospective Study.
Kidney & blood pressure researchRecurrence of C3 Glomerulopathy and Immune Complex-Mediated Membranoproliferative Glomerulonephritis After Kidney Transplantation: Challenges and Opportunities.
Kidney international reportsDelphi Consensus on Surrogate End Points in C3 Glomerulopathy and Primary Immune Complex-Mediated Membranoproliferative Glomerulonephritis.
Kidney international reportsDisentangling disease heterogeneity in C3 glomerulopathies: a slippery road.
Kidney internationalProbing the surrogate validity of proteinuria thresholds in C3 glomerulopathy/immune-complex membranoproliferative glomerulonephritis: unresolved biases and mechanistic gaps.
Kidney internationalLaboratory findings in patients treated with complement factor C3 inhibitor pegcetacoplan.
Clinical chemistry and laboratory medicineA very rare cause of oliguric acute kidney disease: crescentic C3 glomerulopathy.
Oxford medical case reportsModern challenges in infection prevention: encapsulated organisms in the era of novel complement inhibitors.
Kidney internationalComplement activation drives a compartmentalized innate immune response in C3 glomerulopathy contributing to the disease phenotype.
Kidney internationalNatural History and Clinical Associations with Long-Term Outcomes in Primary C3 Glomerulopathy and Immune Complex-Mediated Membranoproliferative GN.
Clinical journal of the American Society of Nephrology : CJASNElectron Microscopy in Renal Biopsy Interpretation: When and Why It Still Matters.
CureusPegcetacoplan in idiopathic and familial pediatric C3 glomerulopathy.
Pediatric nephrology (Berlin, Germany)Real-World Patient Characteristics, Burden, and Quality of Life in C3 Glomerulopathy and Primary Immune Complex Membranoproliferative Glomerulonephritis.
Kidney360Pegcetacoplan for Treatment of C3 Glomerulopathy and Immune-Complex MPGN.
The New England journal of medicineTrial of Pegcetacoplan in C3 Glomerulopathy and Immune-Complex MPGN.
The New England journal of medicineNovel treatment strategies for C3 glomerulopathy: complement blockade.
International urology and nephrologyC3 glomerulopathy: advancements in diagnostics and therapeutics.
Current opinion in nephrology and hypertensionDiagnosis and treatment in adult patients with C3 glomerulopathy in Japan: a real-world survey.
Clinical and experimental nephrologyComplement Profiling in Glomerular Disease: Insights from Laser Microdissection and Mass Spectrometry.
Clinical journal of the American Society of Nephrology : CJASNEculizumab in C3 Glomerulopathy: A Systematic Review of Therapeutic Efficacy and Clinical Outcomes.
PharmaceuticsFrom Data to Drug: The Translational Impact of RaDaR, the UK National Registry of Rare Kidney Diseases.
Nephrology, dialysis, transplantation : official publication of the European Dialysis and Transplant Association - European Renal AssociationSpectrum and clinical profile of glomerular diseases in a Nepalese cohort, a tertiary care center study: focus on comparing IgA nephropathy with other primary glomerulonephritis.
BMC nephrologyHierarchical clustering uncovered disease patterns and further untangled complexities in immune complex-mediated idiopathic MPGN and C3 glomerulopathy.
Kidney internationalIn situ assessment of glomerular C3/C5 convertases indicates ongoing complement activation in IgA nephropathy.
Nephrology, dialysis, transplantation : official publication of the European Dialysis and Transplant Association - European Renal AssociationIptacopan treatment for recurrent dense deposit disease after kidney transplant: A case report.
SAGE open medical case reportsOral iptacopan therapy in patients with C3 glomerulopathy: a randomised, double-blind, parallel group, multicentre, placebo-controlled, phase 3 study.
Lancet (London, England)Recurrence of Glomerular Diseases (GN) After Kidney Transplantation: A Narrative Review.
Journal of clinical medicineA rare co-existence of celiac disease and C3 glomerulopathy.
NefrologiaIptacopan/LNP023 and rituximab as rescue therapy in a patient with systemic lupus erythematosus-associated atypical haemolytic uraemic syndrome.
Journal of nephrologyA 14-year-old boy with C3 glomerulopathy and Sjögren's syndrome identified by a school urinary screening program.
Fukushima journal of medical scienceCorrigendum to "The Role of von Willebrand Factor in the Pathogenesis of C3 Glomerulopathy" [Kidney International Reports Volume 10, Issue 6, June 2025, Pages 1929-1938].
Kidney international reportsClinical Presentation, Treatment Patterns, Burden of Disease, and the Association of Proteinuria with Clinical Outcomes in C3 Glomerulopathy and Primary Immune Complex Membranoproliferative Glomerulonephritis: A Systematic Review.
NephronAtypical presentation of post-streptococcal glomerulonephritis in a child with genetically confirmed susceptibility to C3-glomerulopathy: a case report and brief review of the literature.
BMC nephrologySpectrum of renal diseases caused by monoclonal immunoglobulin: experience from an Australian tertiary referral centre over a 10-year period.
PathologyTargeting the Roots of Kidney Disease: Systematic Review of the Therapies Targeting the Complement System.
Medicina (Kaunas, Lithuania)Gene-Environment Interaction: Lessons From Complement-Mediated Kidney Disease.
Seminars in nephrologyThe Role of von Willebrand Factor in the Pathogenesis of C3 Glomerulopathy.
Kidney international reportsQuantifying association of early proteinuria and estimated glomerular filtration rate changes with long-term kidney failure in C3 glomerulopathy and immune-complex membranoproliferative glomerulonephritis using the United Kingdom RaDaR Registry.
Kidney internationalAdvances in Complement Inhibitory Strategies for the Treatment of Glomerular Disease: A Rapidly Evolving Field.
Journal of clinical medicineComplement System Inhibitors in Nephrology: An Update-Narrative Review.
International journal of molecular sciencesC3 glomerulopathy post kidney transplantation: A single center experience.
World journal of transplantationClinical and Histologic Predictors of Kidney Outcomes in C3 Glomerulopathy and Idiopathic Membranoproliferative GN.
Clinical journal of the American Society of Nephrology : CJASNSuccessful Management of C3 Glomerulopathy Recurrence Post-Kidney Transplantation with Iptacopan: A Case Report.
International journal of molecular sciencesC3 glomerulopathy and acute kidney injury after mRNA-1273 (Moderna) COVID-19 vaccine and genetical analysis.
Clinical nephrologyFactor H-related 1 and heparan sulfate architecture contribute to complement dysregulation in C3 glomerulopathy.
Frontiers in immunologySpatially resolved transcriptomic profiling for glomerular and tubulointerstitial gene expression in C3 glomerulopathy.
Clinical kidney journalCFHR5 Nephropathy Case Report: A Novel Variant Characterized by Tubulointerstitial Kidney Disease.
Kidney & blood pressure researchKidney transplant in patients with C3 glomerulopathy.
Clinical kidney journalComplement-Mediated Kidney Disease and Living Donor Transplantation: Tailoring Approaches to Improve Outcomes.
Current transplantation reportsExpanding the spectrum of genetic causes of DNA-specific exonuclease TREX1 variants in thrombotic microangiopathy.
Kidney internationalPregnancy outcomes in C3 glomerulopathy: a retrospective review.
BMC nephrologyThe 4 functional segments of Factor H: Role in physiological target recognition and contribution to disease.
Journal of immunology (Baltimore, Md. : 1950)C-model: A comprehensive enhanced pharmacokinetic/pharmacodynamic simulation environment targeting the complement system.
British journal of pharmacologyMalignancies and glomerulonephritis: when to suspect and when to screen?
Clinical kidney journalMonoclonal gammopathy-associated C3 glomerulonephritis secondary to follicular lymphoma: a case report.
Frontiers in immunologyMycoplasma Infection Associated C3 Glomerulopathy.
Indian journal of pediatricsComparative Analysis of Proteinuria and Longitudinal Outcomes in Immune Complex Membranoproliferative Glomerulonephritis and C3 Glomerulopathy.
Kidney international reportsTruncated complement factor H Y402 gene therapy rescues C3 glomerulonephritis.
Molecular therapy : the journal of the American Society of Gene TherapyThe Recurrence of Systemic Diseases in Kidney Transplantation.
Journal of clinical medicineSelective factor B inhibition with iptacopan: transforming C3 glomerulopathy management.
International urology and nephrologyIdeZ protease does not prevent convertase stabilization by C3 nephritic factors in C3 glomerulopathy.
Nephrology, dialysis, transplantation : official publication of the European Dialysis and Transplant Association - European Renal AssociationUpdates on Glomerular Diseases: A Summary of Inaugural GlomCon Hawaii 2024.
Glomerular diseasesFactor H-related 2 levels dictate FHR dimer composition.
Scientific reportsEffective long-term treatment with moss-produced factor H by overcoming the antibody response in a mouse model of C3G.
Frontiers in immunologyClinical characteristics and short term outcomes of childhood immune complex membranoproliferative glomerulonephritis and C3 glomerulopathy: a single centre retrospective study.
BMC nephrologyAcquired drivers of C3 glomerulopathy.
Clinical kidney journalAssessing C3 glomerulopathy outcomes in children: how concerned should we be?
Pediatric nephrology (Berlin, Germany)Iptacopan Reduces Proteinuria and Stabilizes Kidney Function in C3 Glomerulopathy.
Kidney international reportsKidney Transplantation in Children and Adolescents With C3 Glomerulopathy or Immune Complex Membranoproliferative Glomerulonephritis: An International Survey of Current Practice.
Pediatric transplantationClinical features and outcomes of immune complex-membranoproliferative glomerulonephritis and C3 glomerulopathy: a multicenter observational cohort study analyzing kidney biopsy cases.
Kidney research and clinical practiceC3 glomerulopathy associated with mycoplasma pneumoniae infection and positive IgA staining.
BMC nephrologyNovel immunochromatographic test for rapid detection of anti-factor H autoantibodies with an assessment of its clinical relevance.
Frontiers in immunologyC3 glomerulonephritis associated with monoclonal gammopathy of renal significance: a diagnostic and therapeutic challenge.
Jornal brasileiro de nefrologiaWhy Is C5a Inflammatory Complement Inhibition Not Enough to Improve C3 Glomerulopathy?
Journal of the American Society of Nephrology : JASNPegcetacoplan for the Treatment of Paediatric C3 Glomerulonephritis: A Case Report.
Nephrology (Carlton, Vic.)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.
ImmunoHorizonsAdverse drug events (ADEs) risk signal mining related to eculizumab based on the FARES database.
Frontiers in pharmacologyEfficacy of complement inhibition with pegcetacoplan in children with C3 glomerulopathy.
Pediatric nephrology (Berlin, Germany)Anticomplement Therapies for C3 Glomerulopathy and Immune-Complex Membranoproliferative Glomerulonephritis Recurrence - A Dawn of New Hope.
Kidney international reportsEfficacy and Safety of Pegcetacoplan in Kidney Transplant Recipients With Recurrent Complement 3 Glomerulopathy or Primary Immune Complex Membranoproliferative Glomerulonephritis.
Kidney international reportsOutcome of Patients Transplanted for C3 Glomerulopathy and Primary Immune Complex-Mediated Membranoproliferative Glomerulonephritis.
Kidney international reportsThe Difficulties of Treating Complement-3-Mediated Glomerulopathy.
American journal of therapeuticsRecurrence of Glomerular Diseases after Kidney Transplantation: What Do We Know New?
NephronWhen a NeF Is Not Enough: Improving Assays in C3 Glomerulopathy.
Journal of the American Society of Nephrology : JASNEstablishing the Future Direction of Clinical Outcomes in C3 Glomerulopathy: Perspectives From a Patient and a Physician.
Kidney medicineMonoclonal gammopathy is present in one fourth of patients undergoing renal biopsy but is pathogenic only in half of them.
Journal of nephrologyIdiopathic inflammatory myopathy and C3 glomerulopathy: a rare association.
Journal of nephrologyMinimal Change Disease and Focal Segmental Glomerulosclerosis Are Associated with Good Kidney Prognosis and Thrombotic Microangiopathy with Poor Kidney Survival in Patients with COVID-19-Associated Nephropathies.
American journal of nephrologyOutcomes of Covid-19 Vaccine-Associated Glomerular Diseases (CVAGD) - A Case Series from India.
Indian journal of nephrologyLate Recurrence of C3 Glomerulopathy After SARS-CoV-2 Infection in a Long-Term Kidney Transplant Recipient: A Case Report.
The American journal of case reportsDe Novo C3 Glomerulonephritis of Allograft Associated With Factor H Autoantibody in a Patient with Systemic Lupus Erythematosus: A Case Report.
Transplantation proceedingsC3 Glomerulopathy: A Current Perspective in an Evolving Landscape.
Glomerular diseasesComplement in Kidney Transplantation.
Transplantation reviews (Orlando, Fla.)C3 glomerulopathy in children: experience at a resource-limited center.
Clinical and experimental pediatricsC3 glomerulopathy in children: a European longitudinal study evaluating outcome.
Pediatric nephrology (Berlin, Germany)[What is proven in the treatment of complement-mediated kidney diseases?].
Innere Medizin (Heidelberg, Germany)C3 glomerulopathy is highly prevalent in French Polynesia.
Journal of translational autoimmunityAcquired and genetic drivers of C3 and C5 convertase dysregulation in C3 glomerulopathy and immunoglobulin-associated MPGN.
Nephrology, dialysis, transplantation : official publication of the European Dialysis and Transplant Association - European Renal AssociationAcquired and genetic determinants of disease phenotype and therapeutic strategies in C3 glomerulopathy and immunoglobulin-associated MPGN.
Nephrology, dialysis, transplantation : official publication of the European Dialysis and Transplant Association - European Renal AssociationC3 glomerulopathy: a kidney disease mediated by alternative pathway deregulation.
Frontiers in nephrologyChildhood onset C3 glomerulopathy: recurrence after kidney transplantation-a case series.
Frontiers in pediatricsPrognostic factors and validation of the histologic chronicity score for C3 glomerulopathy: a registry analysis.
Clinical kidney journalSafety and Efficacy of Avacopan in Patients with Complement 3 Glomerulopathy: Randomized, Double-Blind Clinical Trial.
Journal of the American Society of Nephrology : JASNTruncated Complement Factor H Y402 Gene Therapy Cures C3 Glomerulonephritis.
bioRxiv : the preprint server for biologyFunctional Characterization of Anti-C3bBb Autoantibodies and C3 Glomerulopathy Phenotype.
Journal of the American Society of Nephrology : JASNGlomerular injury induced by vinyl carbamate in A/J inbred mice: a novel model of membranoproliferative glomerulonephritis.
Frontiers in pharmacologyHow Stem and Progenitor Cells Can Affect Renal Diseases.
CellsSuccessfully treated C3 glomerulopathy in which protein and genetic analyses were useful for diagnosis.
CEN case reportsCorrigendum: Genetic investigation of Nordic patients with complement-mediated kidney diseases.
Frontiers in immunologyMonoclonal gammopathy of renal significance from the perspective of nephrologists.
Blood researchFirst Successful Treatment of a Patient with a Primary Immune Complex-Membranoproliferative Glomerulonephritis with Iptacopan: A Case Report.
Case reports in nephrology and dialysisC3 Glomerulopathy Recurs Early after Kidney Transplantation in Serial Biopsies Performed within the First 2 Years after Transplantation.
Clinical journal of the American Society of Nephrology : CJASNKidney transplantation in children and adolescents with C3 glomerulopathy or immune complex membranoproliferative glomerulonephritis: a real-world study within the CERTAIN research network.
Pediatric nephrology (Berlin, Germany)Prognosis is still poor in patients with posttransplant C3 glomerulopathy despite eculizumab use.
Clinical kidney journalUpdate on C3 Glomerulopathy.
Advances in kidney disease and healthUnsupervised Clustering of Membranoproliferative Glomerulonephritis and C3 Glomerulopathy Patients Discovers Distinct Patient Groups unlike the Current Classification.
NephronUpdates on C3 Glomerulopathy in Kidney Transplantation: Pathogenesis and Treatment Options.
International journal of molecular sciencesProof of concept of a new plasma complement Factor H from waste plasma fraction.
Frontiers in immunologyApolipoprotein E in dense deposits: a parting of the ways in C3 glomerulopathy.
Clinical kidney journalUrinary complement biomarkers in immune-mediated kidney diseases.
Frontiers in immunologyComprehensive functional characterization of complement factor I rare variant genotypes identified in the SCOPE geographic atrophy cohort.
The Journal of biological chemistryThe role of complement in kidney disease: conclusions from a Kidney Disease: Improving Global Outcomes (KDIGO) Controversies Conference.
Kidney internationalDeveloping Therapies for C3 Glomerulopathy: Report of the Kidney Health Initiative C3 Glomerulopathy Trial Endpoints Work Group.
Clinical journal of the American Society of Nephrology : CJASNIgM autoantibodies to complement factor H in C3 glomerulopathy.
Journal of nephrologyMultiple Myeloma as a Potential Cause of Low Complements in Patients With Acute Kidney Injury.
CureusMoss-produced human complement factor H with modified glycans has an extended half-life and improved biological activity.
Frontiers in immunologyPrimary immunodeficiency as a cause of immune-mediated kidney diseases.
Nephrology, dialysis, transplantation : official publication of the European Dialysis and Transplant Association - European Renal AssociationFactor B Inhibition with Iptacopan in Recurrent C3 Glomerulopathy Following Kidney Transplant: A Report of Two Cases.
Kidney medicineComplement Terminal Pathway Activation and Intrarenal Immune Response in C3 Glomerulopathy.
Journal of the American Society of Nephrology : JASNClinical characteristics and outcomes of immune-complex membranoproliferative glomerulonephritis and C3 glomerulopathy in Japanese children.
Pediatric nephrology (Berlin, Germany)Recurrent disease after pediatric renal transplantation.
Pediatric transplantationC3 Dominant Collapsing Focal Segmental Glomerulosclerosis - A Report of Two Rare Cases.
Indian journal of nephrologyClinical and pathological features of 52 patients with glomerulonephritis with dominant C3.
Zhong nan da xue xue bao. Yi xue ban = Journal of Central South University. Medical sciencesSuccessful Treatment of Posttransplant Monoclonal Gammopathy-associated C3 Glomerulopathy With Plasma Cell Clone-directed Therapy.
Transplantation directDiagnostic Challenges and Emerging Pathogeneses of Selected Glomerulopathies.
Pediatric and developmental pathology : the official journal of the Society for Pediatric Pathology and the Paediatric Pathology SocietyC3 concentrations can be normal in patients with C3 glomerulopathy secondary to C3 nephritic factor.
Journal of clinical pathologyC3 Glomerulopathy: Novel Treatment Paradigms.
Kidney international reportsDigital Ischemia as a Rare Manifestation of C3 Glomerulopathy Associated With Monoclonal Gammopathy.
Kidney international reportsApolipoprotein E is enriched in dense deposits and is a marker for dense deposit disease in C3 glomerulopathy.
Kidney internationalHot Spot of Complement Factor I Rare Variant p.Ile357Met in Patients With Hemolytic Uremic Syndrome.
American journal of kidney diseases : the official journal of the National Kidney FoundationRecurrent C3 glomerulopathy after kidney transplantation.
Transplantation reviews (Orlando, Fla.)Triple-fusion protein (TriFu): A potent, targeted, enzyme-like inhibitor of all three complement activation pathways.
The Journal of biological chemistryEculizumab dose tapering should take into account the nonlinearity of its pharmacokinetics.
British journal of clinical pharmacologyEfficacy of GalNAc C3 siRNAs in factor H-deficient mice with C3 glomerulopathy.
Molecular immunologyDefining Nephritic Factors as Diverse Drivers of Systemic Complement Dysregulation in C3 Glomerulopathy.
Kidney international reportsRationale and design of the Japanese Biomarkers in Nephrotic Syndrome (J-MARINE) study.
Clinical and experimental nephrologyHistologic and Clinical Factors Associated with Kidney Outcomes in IgA Vasculitis Nephritis.
Clinical journal of the American Society of Nephrology : CJASNAnti-factor B antibodies in atypical hemolytic uremic syndrome.
Pediatric nephrology (Berlin, Germany)C3 Glomerulonephritis Presenting With Nephritic and Nephrotic Syndromes: Spontaneous Remission After Six Months on Dialysis.
Cureus[Comparative characteristics of the complement system in patients with C3-glomerulopathy and atypical hemolytic uremic syndrome of chronic course who suffered an acute episode of thrombotic microangiopathy].
Terapevticheskii arkhivEfficacy and Safety of Iptacopan in Patients With C3 Glomerulopathy.
Kidney international reportsThe Improved Kidney Risk Score in ANCA-Associated Vasculitis for Clinical Practice and Trials.
Journal of the American Society of Nephrology : JASN[Translated article] Pharmacokinetics of eculizumab in adult and pediatric patients with atypical hemolytic uremic syndrome and C3 glomerulopathy.
Farmacia hospitalaria : organo oficial de expresion cientifica de la Sociedad Espanola de Farmacia HospitalariaComplement gene mutations in children with C3 glomerulopathy: do they affect the response to mycophenolate mofetil?
Pediatric nephrology (Berlin, Germany)Clinical Safety and Efficacy of Pegcetacoplan in a Phase 2 Study of Patients with C3 Glomerulopathy and Other Complement-Mediated Glomerular Diseases.
Kidney international reportsLow C3 in a 4-month-old baby: is it a problem?
Pediatric nephrology (Berlin, Germany)Overlapping Atypical Hemolytic Uremic Syndrome and C3 Glomerulopathy with Mutation in CFI in a Japanese Patient: A Case Report.
Internal medicine (Tokyo, Japan)Clinical research advances of CFHR5 nephropathy: a recent review.
European review for medical and pharmacological sciencesC3 glomerulopathy in a patient with a history of post-infectious glomerulonephritis.
Pediatric nephrology (Berlin, Germany)Morphological and etiological analyses of C3 and non-C3 glomerulonephritis in primary membranoproliferative glomerulonephritis using periodic acid-methenamine silver stain electron microscopy: a retrospective multicentered study.
Medical molecular morphologyEtiology and Outcomes of Rapidly Progressive Glomerulonephritis in Children: A Retrospective Cohort Study.
Indian pediatricsFrom Patterns to Proteins: Mass Spectrometry Comes of Age in Glomerular Disease.
Journal of the American Society of Nephrology : JASNGenetic investigation of Nordic patients with complement-mediated kidney diseases.
Frontiers in immunologyComplement inhibitors in pediatric kidney diseases: new therapeutic opportunities.
Pediatric nephrology (Berlin, Germany)[Diagnosis and treatment of glomerular diseases with a membranoproliferative glomerulonephritis (MPGN) pattern of injury].
Wiener klinische WochenschriftDaratumumab in the treatment of C3 glomerulopathy with monoclonal gammopathy: a case report and literature review.
Frontiers in medicineExpansion of Anticomplement Therapy Indications from Rare Genetic Disorders to Common Kidney Diseases.
Annual review of medicineAssociation of monoclonal gammopathy of undetermined significance and C3 glomerulopathy.
Internal medicine journalRare Variants in Complement Gene in C3 Glomerulopathy and Immunoglobulin-Mediated Membranoproliferative GN.
Clinical journal of the American Society of Nephrology : CJASNC3G and Ig-MPGN-treatment standard.
Nephrology, dialysis, transplantation : official publication of the European Dialysis and Transplant Association - European Renal AssociationRecurrent C3 Glomerulonephritis along with BK-Virus-Associated Nephropathy after Kidney Transplantation: A Case Report.
Medicina (Kaunas, Lithuania)Pioglitazone, a PPAR-y agonist, as one of the new therapeutic candidates for C3 glomerulopathy.
Pediatric nephrology (Berlin, Germany)Liver factor B silencing to cure C3 glomerulopathy: Evidence from a mouse model of complement dysregulation.
Molecular immunologyTargeted therapy in glomerular diseases.
Journal of the Formosan Medical Association = Taiwan yi zhiDesign and Rationale of the APPELHUS Phase 3 Open-Label Study of Factor B Inhibitor Iptacopan for Atypical Hemolytic Uremic Syndrome.
Kidney international reportsTargeting the Complement Pathway in Kidney Transplantation.
Journal of the American Society of Nephrology : JASNA 78-Year-Old Man with Chronic Kidney Disease and Monoclonal Gammopathy Who Developed Post-Transplant C3 Glomerulopathy - Recurrence or De Novo? A Case Report and Literature Review.
The American journal of case reportsSuccessful treatment with avacopan (CCX168) in a pediatric patient with C3 glomerulonephritis.
Pediatric nephrology (Berlin, Germany)Associações
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Referências e fontes
Bases de dados externas citadas neste artigo
Publicações científicas
Artigos indexados no PubMed ligados a esta doença no grafo RarasNet — título, periódico e PMID direto da fonte, sem intermediação de IA.
- Treatment of atypical hemolytic uremic syndrome and C3 glomerulopathy in mice by hepatic expression of factor D.
- Clinical Profile and Outcome of C3-Dominant Glomerulonephritis: Retrospective Study.
- Pegcetacoplan in C3 Glomerulopathy and Immune-Complex MPGN. Reply.
- Pegcetacoplan in C3 Glomerulopathy and Immune-Complex MPGN.
- Autoantibodies Targeting Complement Regulating Factors Induced C3 Glomerulopathy Resambling C4 Dense Deposit Disease: A Case Report.
- New Anticomplement Drugs in Nephrology - Mechanism and Indication.
- Clinical Images: Anti-MDA5 dermatomyositis with rapidly progressive interstitial lung disease presenting with rapidly progressive glomerulonephritis due to C3 glomerulopathy.
- Finding the etiology of membranoproliferative glomerulonephritis.
- A Case Series of Elevated Donor-Derived Cell-Free DNA Leading to Diagnosis of Posttransplant Glomerulonephritis.
Bases de dados e fontes oficiais
Identificadores e referências canônicas usadas para montar este verbete.
- ORPHA:329918(Orphanet)
- MONDO:0018013(MONDO)
- GARD:17507(GARD (NIH))
- Variantes catalogadas(ClinVar)
- Busca completa no PubMed(PubMed)
- Q56014073(Wikidata)
Dados compilados pelo RarasNet a partir de fontes abertas (Orphanet, OMIM, MONDO, PubMed/EuropePMC, ClinicalTrials.gov, DATASUS, PCDT/MS). Este conteúdo é informativo e não substitui avaliação médica.
Conteúdo mantido por Agente Raras · Médicos e pesquisadores podem colaborar
