Um tipo de inflamação nos rins (glomerulonefrite) que se caracteriza pelo acúmulo de uma proteína específica, o C3, com pouca ou nenhuma presença de anticorpos. Ao contrário de uma doença similar, a "doença de depósito denso", não são encontradas as alterações que ficam mais escuras em exames de alta precisão (de microscopia eletrônica).
Introdução
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Um tipo de inflamação nos rins (glomerulonefrite) que se caracteriza pelo acúmulo de uma proteína específica, o C3, com pouca ou nenhuma presença de anticorpos. Ao contrário de uma doença similar, a "doença de depósito denso", não são encontradas as alterações que ficam mais escuras em exames de alta precisão (de microscopia eletrônica).
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Entender a doença
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Sinais e sintomas
O que aparece no corpo e com que frequência cada sintoma acontece
Partes do corpo afetadas
+ 3 sintomas em outras categorias
Características mais comuns
Os sintomas variam de pessoa para pessoa. Abaixo estão as 11 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
4 genes identificados com associação a esta condição.
Involved in complement regulation. The dimerized forms have avidity for tissue-bound complement fragments and efficiently compete with the physiological complement inhibitor CFH
Secreted
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
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
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.
Variantes genéticas (ClinVar)
92 variantes patogênicas registradas no ClinVar.
Classificação de variantes (ClinVar)
Distribuição de 205 variantes classificadas pelo 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 — Glomerulonefrite C3
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Dados de DATASUS/CNES, SBGM, ABNeuro e Ministério da Saúde. Sempre confirme a disponibilidade diretamente com o estabelecimento.
Pesquisa ativa
Ensaios clínicos abertos e novidades científicas recentes
Ensaios em destaque
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2 pesquisas recrutando participantes. Converse com seu médico sobre a possibilidade de participar.
Outros ensaios clínicos
13 ensaios clínicos encontrados, 3 ativos.
Publicações mais relevantes
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%.
A pediatric case of C3 glomerulonephritis initially misclassified as IgA nephropathy with a favorable response to C3-targeted therapy.
C3 glomerulopathy is an ultra-rare kidney disease driven by dysregulation of the alternative complement pathway fluid phase C3 convertase. We report the case of a previously healthy 13-year-old girl who presented with concurrent nephrotic and nephritic syndrome and low complement C3. Her initial kidney biopsy surprisingly showed mesangioproliferative glomerulonephritis with dominating IgA deposits resulting in a diagnosis of IgA nephropathy. Despite standard immunosuppressive treatment with prednisone and mycophenolic acid plus angiotensin-converting enzyme inhibitors, she achieved only partial remission and subsequently relapsed, exhibiting severe, persistent C3 consumption (6-8 mg/dl) and sustained C5b9 activation (1059 ng/ml). A repeat biopsy 1.2 years later established the definitive diagnosis of C3 glomerulonephritis (C3GN) with a membranoproliferative pattern. C3NEF antibodies were negative, and no pathological variant was detected in the genetic study. After 1 year and 9 months without response to immunosuppressive treatment, the C3 inhibitor pegcetacoplan was initiated. The patient achieved rapid and complete remission within 3 months, marked by normalization of proteinuria, from 2747 mg/day (urine protein-to-creatinine ratio [UPCR] 2.66 mg/mg) pre-treatment to 19 mg/d (UPCR 0.02 mg/mg) and complement activation markers (from 1162 ng/ml pre-treatment to C5b9 92 ng/ml; reference value 30-150 ng/ml). This case highlights the inherent diagnostic complexity of C3GN, suggesting the critical role of repeat biopsies in treatment-refractory, complement-dysregulated glomerulonephritis. It strongly supports the potential efficacy of C3 inhibition as a targeted therapeutic approach for patients with C3GN.
C3 Glomerulopathy Diagnosis, Current Treatments, and Emerging Therapies.
C3 glomerulopathy (C3G) is characterized by prominent deposition of complement component C3 in the kidney glomeruli, leading to glomerular inflammation. C3G is a rare and complex pattern of injury caused by dysregulation of the alternative pathway of complement system and occurs in both children and adults. It can happen because of genetic and acquired factors. Kidney biopsy is the gold standard for diagnosing C3G. About 50% of cases progress to kidney failure, and traditional treatment strategies, including immunosuppression and supportive care, have demonstrated variable efficacy. In this review, we aim to provide a comprehensive overview of pathophysiology, clinical presentations, and diagnostic criteria of C3G. Additionally, we will discuss current treatment guidelines and ongoing clinical trials.
Primary membranoproliferative glomerulonephritis: natural history, pathogenesis, and treatment.
Primary membranoproliferative glomerulonephritis (MPGN) is an ultrarare disease characterized by immunofluorescence microscopy as either immune-complex mediated (IC-MPGN) or C3 glomerulopathy (C3), the latter subdivided by electron microscopy to C3 glomerulonephritis (C3GN) and dense deposit disease (DDD). Both IC-MPGN and C3G typically have obvious C3 staining differentiating them from other causes of MPGN histology. Secondary causes must be excluded, including infections, autoimmune disease, and neoplasia. Clinical presentations are variable, including urinary sediment abnormalities, nephrotic syndrome, or a rapidly progressive course. The prognosis is unfavorable with about 50% reaching kidney failure by 10 years. Recurrence following transplantation is frequent, and allograft survival is shortened. The pathogenesis involves dysregulation of the alternate pathway (AP) of complement. Possibly 20% of patients harbor pathogenic mutations in AP proteins or their regulators, and up to 80% have autoantibodies impairing normal regulation. Paraproteins are found in 20 - 40% of otherwise primary MPGN, either directly detectable on biopsy (IC-MPGN) or as dysregulators of the AP. Therapy of MPGN begins with supportive care as for all glomerulopathies. Paraproteins require clone-directed therapy. When immunosuppression is considered, complement inhibition should be first line. Two agents are now FDA approved for C3G, the oral Factor B inhibitor iptacopan and the subcutaneous C3-inhibitor pegcetacoplan, the latter also approved for IC-MPGN. If complement inhibition is unavailable, MMF/steroids may be considered. Following transplantation, protocol biopsies are needed to detect early recurrence with the intent of complement inhibition.
Proteomic Profiling of Complement Components in Glomerular Disease.
The complement system plays a central role in glomerular disease development and resolution. Currently, renal biopsies assess the presence of complement through limited immunofluorescence stains (C3 and C1q). With >50 total proteins and fragments involved in the complement cascade, this method offers a severely limited view into the mechanisms of tissue injury orchestrated by complement activation. A more comprehensive evaluation of complement components will advance the understanding of complement involvement in glomerular diseases by allowing for multiplex detection of complement cascade proteins and activation products, which can be achieved by mass spectrometry (MS). Data-independent acquisition MS was performed following extraction of proteins from tissue lysates, microdissected glomeruli, or protein G immunoprecipitates from residual kidney biopsy tissue. Cohorts included patients with lupus nephritis, membranous nephropathy and membranous lupus nephritis, diabetic glomerulosclerosis, C3 glomerulonephritis, and control biopsies. Abundances of complement components by MS correlated with immunofluorescence intensity of C1q on kidney biopsies. Increased abundances of complement classical, lectin, final common pathway, and regulatory proteins correlated with disease activity in lupus nephritis. Complement protein abundances of final common pathway components were heterogeneous between patients with the same disease state, including diabetic glomerulosclerosis and various forms of proliferative glomerulonephritis. Finally, complement proteins and their activation products can be mapped to determine which components are impacted among individuals and between disease states. In conclusion, complement proteins and some of their split products can be reliably measured by MS of kidney biopsies, which can enhance our understanding of complement-mediated tissue injury and heterogeneity in glomerular diseases.
Publicações recentes
Complement 3 Glomerulopathy (C3G) in Native and Posttransplant Kidneys: Pathophysiology, Prognosis, and Treatment.
Finding the etiology of membranoproliferative glomerulonephritis.
🥉 Relato de casoA pediatric case of C3 glomerulonephritis initially misclassified as IgA nephropathy with a favorable response to C3-targeted therapy.
C3 Glomerulopathy Diagnosis, Current Treatments, and Emerging Therapies.
Primary membranoproliferative glomerulonephritis: natural history, pathogenesis, and treatment.
📚 EuropePMC115 artigos no totalmostrando 198
A pediatric case of C3 glomerulonephritis initially misclassified as IgA nephropathy with a favorable response to C3-targeted therapy.
Pediatric nephrology (Berlin, Germany)C3 Glomerulopathy Diagnosis, Current Treatments, and Emerging Therapies.
Kidney medicinePrimary membranoproliferative glomerulonephritis: natural history, pathogenesis, and treatment.
Frontiers in nephrologyProteomic Profiling of Complement Components in Glomerular Disease.
Laboratory investigation; a journal of technical methods and pathologyRefractory C3 glomerulonephritis unresponsive to bortezomib.
NefrologiaC3 glomerulonephritis with light chain crystalline podocytopathy and non-crystalline proximal tubulopathy: a case report and integrative clinicopathologic analysis.
BMC nephrologyA case of concurrent cold agglutinin disease and C3 glomerulonephritis requiring differentiation from other iatrogenic immunodeficiency-associated lymphoproliferative disorder in a patient with rheumatoid arthritis and Sjögren's disease.
Immunological medicineA Decade of C3 Glomerulopathy-A Nationwide Cohort Study.
Kidney international reportsClinical Profile and Outcome of C3-Dominant Glomerulonephritis: Retrospective Study.
Kidney & blood pressure researchElectron Microscopy in Renal Biopsy Interpretation: When and Why It Still Matters.
CureusPegcetacoplan in idiopathic and familial pediatric C3 glomerulopathy.
Pediatric nephrology (Berlin, Germany)De novo C3 Glomerulonephritis in a Kidney Transplant Recipient Associated With a Rare CFH Variant of Unknown Significance.
Kidney international reportsC3 glomerulopathy: advancements in diagnostics and therapeutics.
Current opinion in nephrology and hypertensionEculizumab in C3 Glomerulopathy: A Systematic Review of Therapeutic Efficacy and Clinical Outcomes.
PharmaceuticsC3 Glomerulonephritis in a Child with Renal Coloboma Syndrome.
Indian journal of pediatricsDelayed Therapeutic Response to Oral Corticosteroids and Rituximab Leading to Dialysis Independence in a Patient with Treatment-resistant C3 Glomerulonephritis.
Internal medicine (Tokyo, Japan)Membranous-like glomerulopathy with masked IgG-k deposits in a pediatric patient with juvenile idiopathic arthritis.
Pediatric nephrology (Berlin, Germany)Apolipoprotein E Immunostaining Has Diagnostic Utility in Differentiating Dense Deposit Disease and C3 Glomerulonephritis: Clone-Based Evaluation of D719N, EP1373Y, and 1B2C9.
Modern pathology : an official journal of the United States and Canadian Academy of Pathology, IncC3 Glomerulonephritis Associated With Unusual IgG4 Antifactor H in IgG4-related Disease.
Kidney medicineC3 glomerulopathy post kidney transplantation: A single center experience.
World journal of transplantationClinical and Pathological Course of Recurrent C3 Glomerulonephritis from Onset to Graft Loss: A Case Report.
Case reports in nephrology and dialysisMonoclonal gammopathy-associated C3 glomerulonephritis secondary to follicular lymphoma: a case report.
Frontiers in immunologyTruncated complement factor H Y402 gene therapy rescues C3 glomerulonephritis.
Molecular therapy : the journal of the American Society of Gene TherapyAcquired drivers of C3 glomerulopathy.
Clinical kidney journalC3-Dominant Infection Related Crescentic Glomerulonephritis.
Giornale italiano di nefrologia : organo ufficiale della Societa italiana di nefrologiaClinical and Pathological Characteristics of Non-AL Amyloidosis MGRS: A Single-Center Experience Over 10 Years.
Canadian journal of kidney health and diseaseGene variant C3 glomerulonephritis with chronic urinary tract infection: A case report and literature review.
MedicineC3 glomerulonephritis associated with monoclonal gammopathy of renal significance: a diagnostic and therapeutic challenge.
Jornal brasileiro de nefrologiaPegcetacoplan for the Treatment of Paediatric C3 Glomerulonephritis: A Case Report.
Nephrology (Carlton, Vic.)Efficacy of complement inhibition with pegcetacoplan in children with C3 glomerulopathy.
Pediatric nephrology (Berlin, Germany)The Difficulties of Treating Complement-3-Mediated Glomerulopathy.
American journal of therapeuticsIdiopathic inflammatory myopathy and C3 glomerulopathy: a rare association.
Journal of nephrologyC3 Glomerulonephritis Associated with Monoclonal Gammopathy.
Indian journal of nephrologyDe Novo C3 Glomerulonephritis of Allograft Associated With Factor H Autoantibody in a Patient with Systemic Lupus Erythematosus: A Case Report.
Transplantation proceedingsC3 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)C3 glomerulopathy is highly prevalent in French Polynesia.
Journal of translational autoimmunityMembranoproliferative Glomerulonephritis over 20 Years at a Tertiary Referral Center in the UK.
Glomerular diseasesNovel use of Siltuximab in a patient with VEXAS Syndrome.
Annals of hematologyMultiple Pyoderma Gangrenosum Overlying AV Fistula Treated With Colchicine: A Case Report.
Canadian journal of kidney health and diseaseTruncated Complement Factor H Y402 Gene Therapy Cures C3 Glomerulonephritis.
bioRxiv : the preprint server for biologyGlomerular injury induced by vinyl carbamate in A/J inbred mice: a novel model of membranoproliferative glomerulonephritis.
Frontiers in pharmacologyOutcome of Glomerular Disease Manifesting After Vaccination Against Severe Acute Respiratory Syndrome Coronavirus 2.
CureusKidney involvement in myelodysplastic syndromes.
Clinical kidney journalA Practical Method for Synthesizing Iptacopan.
Molecules (Basel, Switzerland)Clinical characteristics and outcomes of immune-complex membranoproliferative glomerulonephritis and C3 glomerulopathy in Japanese children.
Pediatric nephrology (Berlin, Germany)Apolipoprotein E is enriched in dense deposits and is a marker for dense deposit disease in C3 glomerulopathy.
Kidney internationalAtypical Hemolytic Uremic Syndrome: A Nationwide Colombian Pediatric Series.
Global pediatric healthC3 Glomerulonephritis Presenting With Nephritic and Nephrotic Syndromes: Spontaneous Remission After Six Months on Dialysis.
CureusSyphilis-Related Nephropathy: A Rare Manifestation of a Re-emerging Disease.
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 arkhivC3 glomerulopathies: dense deposit disease and C3 glomerulonephritis.
Frontiers in medicineComplement gene mutations in children with C3 glomerulopathy: do they affect the response to mycophenolate mofetil?
Pediatric nephrology (Berlin, Germany)Glomerular Diseases Across Lifespan: Key Differences in Diagnostic and Therapeutic Approaches.
Seminars in nephrologyC3 glomerulopathy in a patient with a history of post-infectious glomerulonephritis.
Pediatric nephrology (Berlin, Germany)Evaluation of the significance of complement-related genes mutations in atypical postinfectious glomerulonephritis: a pilot study.
International urology and nephrologyMorphological 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 morphologyExpert Discussion on Challenges in C3G Diagnosis: A Podcast Article on Best Practices in Kidney Biopsies.
Advances in therapy[Diagnosis and treatment of glomerular diseases with a membranoproliferative glomerulonephritis (MPGN) pattern of injury].
Wiener klinische WochenschriftAssociation of monoclonal gammopathy of undetermined significance and C3 glomerulopathy.
Internal medicine journalRecurrent C3 Glomerulonephritis along with BK-Virus-Associated Nephropathy after Kidney Transplantation: A Case Report.
Medicina (Kaunas, Lithuania)Anti-factor H antibody-positive C3 glomerulonephritis secondary to poststreptococcal acute glomerulonephritis with diabetic nephropathy.
CEN case reportsImmunoelectron microscopy findings in a patient with C3 glomerulonephritis.
Clinical nephrology. Case studiesA 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)Pembrolizumab induced-C3 glomerulonephritis and RBC cast nephropathy: a case report.
BMC nephrologyCKD therapy to improve outcomes of immune-mediated glomerular diseases.
Nephrology, dialysis, transplantation : official publication of the European Dialysis and Transplant Association - European Renal AssociationDiagnostic Problems in C3 Glomerulopathy.
BiomedicinesUtilizing therapeutic drug monitoring to optimize therapy with eculizumab and mycophenolate mofetil in a child with C3 glomerulonephritis.
Pediatric nephrology (Berlin, Germany)A Monoclonal Antibody That Provides a Model for C3 Nephritic Factors.
Monoclonal antibodies in immunodiagnosis and immunotherapyComplement detection in kidney biopsies - utility and challenges.
Current opinion in nephrology and hypertensionC3 Glomerulopathy: A Review with Emphasis on Ultrastructural Features.
Glomerular diseasesEculizumab as a treatment for C3 glomerulopathy: a single-center retrospective study.
BMC nephrologyRenal injury in scleromyxoedema due to monoclonal gammopathy associated C3 glomerulonephritis.
BMJ case reports[Remissions and progression of C3 glomerulopathy].
Terapevticheskii arkhivC3 glomerulonephritis accompanied with lupus nephritis.
NefrologiaKidney Involvement in Pediatric COVID-19 Cases: A Single-Center Experience.
Turkish archives of pediatricsRecurrent C3 Glomerulonephritis with an ADAMTS 13 Gene Variant: A Case Report and Literature Review.
Saudi journal of kidney diseases and transplantation : an official publication of the Saudi Center for Organ Transplantation, Saudi ArabiaEndocarditis-Associated C3-Dominant Glomerulonephritis in a Patient With a Solitary Kidney.
CureusC3 Glomerulonephritis Post COVID-19 Vaccination: A Case Report and Review of the Literature.
The Brown journal of hospital medicineC3 glomerulopathy: Understanding an ultra-rare complement-mediated renal disease.
American journal of medical genetics. Part C, Seminars in medical geneticsComplement Factor I Variants in Complement-Mediated Renal Diseases.
Frontiers in immunologyHeterozygous laminin β2 mutation in C3 glomerulopathy.
Saudi journal of kidney diseases and transplantation : an official publication of the Saudi Center for Organ Transplantation, Saudi ArabiaGross hematuria, edema, and hypocomplementemia in a 9-year-old boy: Answers.
Pediatric nephrology (Berlin, Germany)Case Report: Chronic Lymphocytic Leukemia With Recurrent Complement-Mediated Thrombotic Microangiopathy and C3 Glomerulonephritis.
Frontiers in medicineRare Case of C3 Glomerulopathy in a Patient of Type 1 Diabetes Mellitus.
Saudi journal of kidney diseases and transplantation : an official publication of the Saudi Center for Organ Transplantation, Saudi ArabiaCharacteristics and Outcome of Biopsy-proven Malignant Hypertension with Severe Kidney Injury: A Retrospective Study.
Indian journal of nephrologyRetinal findings in glomerulonephritis.
Clinical & experimental optometryA case of C3 glomerulopathy with nephritis-associated plasmin receptor positivity without a history of streptococcal infection.
CEN case reportsHistopathological and ultrastructural monitoring of remission induction in crescentic C3 glomerulonephritis.
Journal of nephrologyComplete Renal Recovery in Pediatric Patient with C3 Glomerulonephritis: A Case Report.
Case reports in nephrology and dialysisRepository Corticotropin in Treating de novo C3 Glomerulonephritis after Transplantation.
Glomerular diseasesC3 glomerulonephritis associated with monoclonal gammopathy: a retrospective case series study from a single institute in China.
Renal failureMycoplasma pneumoniae Infection Associated C3 Glomerulopathy Presenting as Severe Crescentic Glomerulonephritis.
Case reports in nephrologyFHR-5 Serum Levels and CFHR5 Genetic Variations in Patients With Immune Complex-Mediated Membranoproliferative Glomerulonephritis and C3-Glomerulopathy.
Frontiers in immunologyMyeloperoxidase immunohistochemical staining can identify glomerular endothelial cell injury in dense deposit disease.
Pediatric nephrology (Berlin, Germany)Demographic, clinical characteristics and treatment outcomes of immune-complex membranoproliferative glomerulonephritis and C3 glomerulonephritis in Japan: A retrospective analysis of data from the Japan Renal Biopsy Registry.
PloS oneMonoclonal Gammopathy of Renal Significance Causes C3 Glomerulonephritis Via Monoclonal IgG Kappa Inhibition of Complement Factor H.
Kidney international reportsLong-term follow-up including extensive complement analysis of a pediatric C3 glomerulopathy cohort.
Pediatric nephrology (Berlin, Germany)Blood oxygen level-dependent imaging for evaluating C3 glomerulonephritis.
Pediatrics international : official journal of the Japan Pediatric Societyvon Willebrand factor variants in C3 glomerulopathy: A Chinese cohort study.
Clinical immunology (Orlando, Fla.)Co-existence of Alport syndrome and C3 glomerulonephritis in a proband with family history.
European journal of medical researchC3 glomerulonephritis and systemic lupus erythematosus: A report of a patient treated with eculizumab and review of the literature.
LupusGeneration and Characterization of Mouse Models of C3 Glomerulonephritis With CFI D288G and P467S Mutations.
Frontiers in physiologyMurine Factor H Co-Produced in Yeast With Protein Disulfide Isomerase Ameliorated C3 Dysregulation in Factor H-Deficient Mice.
Frontiers in immunologyC3 glomerulonephritis associated with ANCA positivity: a case report.
BMC nephrologyC3 Glomerulopathy, a pathology with scarce evidence. A case report.
Giornale italiano di nefrologia : organo ufficiale della Societa italiana di nefrologiaClinico-pathological Profile and Outcome of C-3 Glomerulopathy in Indian Children.
Indian journal of nephrologyC3 Glomerulonephritis Associated with Monoclonal Gammopathy of Renal Significance.
Acta medica portuguesaLongitudinal change in proteinuria and kidney outcomes in C3 glomerulopathy.
Nephrology, dialysis, transplantation : official publication of the European Dialysis and Transplant Association - European Renal AssociationC3 glomerulonephritis along with light chain proximal tubulopathy without crystal deposits in multiple myeloma: a case report.
World journal of surgical oncologyGlomerular C4d deposition in proliferative glomerular diseases.
Indian journal of pathology & microbiologyMonoclonal Gammopathy of Renal Significance: Clinical and Histological Efficacy of a Bortezomib-Based Regimen.
Frontiers in medicineA Rare Case of C3 Glomerulonephritis Presenting as Pulmonary Renal Syndrome.
HCA healthcare journal of medicineFunction and Dysfunction of Complement Factor H During Formation of Lipid-Rich Deposits.
Frontiers in immunologyValidation of a Histologic Scoring Index for C3 Glomerulopathy.
American journal of kidney diseases : the official journal of the National Kidney FoundationCat-Scratch Disease Masquerading as C3 Glomerulonephritis.
Kidney international reportsLong-Term Outcomes of C3 Glomerulopathy and Immune-Complex Membranoproliferative Glomerulonephritis in Children.
Kidney international reportsTreatment of C3 Glomerulopathy in Adult Kidney Transplant Recipients: A Systematic Review.
Medical sciences (Basel, Switzerland)Pediatric C3 glomerulopathy: a 12-year single-center experience.
Pediatric nephrology (Berlin, Germany)C3-glomerulonephritis in New Zealand - a case series.
BMC nephrologyTherapy and outcomes of C3 glomerulopathy and immune-complex membranoproliferative glomerulonephritis.
Pediatric nephrology (Berlin, Germany)Long-term data on two sisters with C3GN due to an identical, homozygous CFH mutation and autoantibodies.
Clinical nephrologyRecurrent Glomerulonephritis after Renal Transplantation: The Clinical Problem.
International journal of molecular sciencesGlomerular galactose-deficient IgA1 expression analysis in pediatric patients with glomerular diseases.
Scientific reportsComplement in Hemolysis- and Thrombosis- Related Diseases.
Frontiers in immunologyGenetic and pathological findings in a boy with psoriasis and C3 glomerulonephritis: A case report and literature review.
Molecular genetics & genomic medicineDiverse Clinical Presentations of C3 Dominant Glomerulonephritis.
Frontiers in medicineThe impact of reclassification of C3 predominant glomerulopathies on diagnostic accuracy, outcome and prognosis in patients with C3 glomerulonephritis.
BMC nephrologyC3 Glomerulopathy: Pathogenesis and Treatment.
Advances in chronic kidney diseaseUse of Bortezomib in the Treatment of C3 Glomerulonephritis Refractory to Eculizumab and Rituximab.
Kidney international reportsUpdate on C3 Glomerulopathy: A Complement-Mediated Disease.
NephronValidation of distinct pathogenic patterns in a cohort of membranoproliferative glomerulonephritis patients by cluster analysis.
Clinical kidney journalClinicopathological Significance and Renal Outcomes of Light Microscopic Patterns in Complement Component 3 Glomerulopathy.
NephronInfection-related Glomerulonephritis and C3 Glomerulonephritis - Similar Yet Dissimilar: A Case Report and Brief Review of Current Literature.
CureusThe glomerular crescent: triggers, evolution, resolution, and implications for therapy.
Current opinion in nephrology and hypertensionConcurrent anti-neutrophil cytoplasmic antibody-associated glomerulonephritis and IgG4-associated tubulointerstitial nephritis with C3 glomerulonephritis: A case report.
MedicineA Narrative Review on C3 Glomerulopathy: A Rare Renal Disease.
International journal of molecular sciencesC3 glomerulonephritis in a patient treated with anti-PD-1 antibody.
European journal of cancer (Oxford, England : 1990)Monoclonal immunoglobulin mediates complement activation in monoclonal gammopathy associated-C3 glomerulonephritis.
BMC nephrologyOutcome of C3 glomerulopathy patients: largest single-centre experience from South Asia.
Journal of nephrologyComplement dysregulation in glomerulonephritis.
Seminars in immunologyC4 nephritic factor in patients with immune-complex-mediated membranoproliferative glomerulonephritis and C3-glomerulopathy.
Orphanet journal of rare diseasesPoor allograft outcome in Indian patients with post-transplant C3 glomerulopathy.
Clinical kidney journalClinicopathological features of C3 glomerulopathy in children: a single-center experience.
Pediatric nephrology (Berlin, Germany)Eculizumab in post-transplant C3 glomerulonephritis caused by a C3 mutation .
Clinical nephrologyUtility of immunohistochemistry with C3d in C3 glomerulopathy.
Modern pathology : an official journal of the United States and Canadian Academy of Pathology, IncRenal pathologic spectrum and clinical outcome of monoclonal gammopathy of renal significance: A large retrospective case series study from a single institute in China.
Nephrology (Carlton, Vic.)Glomerular C4d Staining Does Not Exclude a C3 Glomerulopathy.
Kidney international reportsA novel mutation in complement 2 accompanied by susceptibility variants in C3 glomerulonephritis: A case study.
NefrologiaC5 Convertase Blockade in Membranoproliferative Glomerulonephritis: A Single-Arm Clinical Trial.
American journal of kidney diseases : the official journal of the National Kidney FoundationDifferential contribution of C5aR and C5b-9 pathways to renal thrombic microangiopathy and macrovascular thrombosis in mice carrying an atypical hemolytic syndrome-related factor H mutation.
Kidney international[Membranoproliferative glomerulonephritis and C3 glomerulopathy].
Der InternistClinical course and outcome after kidney transplantation in patients with C3 glomerulonephritis due to CFHR5 nephropathy.
Nephrology, dialysis, transplantation : official publication of the European Dialysis and Transplant Association - European Renal AssociationWidening spectrum of renal involvement in psoriasis: First reported case of C3 glomerulonephritis in a psoriatic patient.
Saudi journal of kidney diseases and transplantation : an official publication of the Saudi Center for Organ Transplantation, Saudi ArabiaC3 glomerulopathy - understanding a rare complement-driven renal disease.
Nature reviews. NephrologyC3 Glomerulonephritis: A Rare Etiology of the Pulmonary Renal Syndrome.
Kidney medicineNephrotoxicity of immune checkpoint inhibitors beyond tubulointerstitial nephritis: single-center experience.
Journal for immunotherapy of cancerC3 Glomerulonephritis With Multiple Mutations in Complement Factor H.
Iranian journal of kidney diseasesCorticosteroid therapy alone for the treatment of C3 glomerulonephritis in association with monoclonal gammopathy .
Clinical nephrologyKidney Transplantation in C3 Glomerulopathy: A Case Series.
American journal of kidney diseases : the official journal of the National Kidney FoundationPredictive factors for poor outcome in pediatric C3 glomerulonephritis.
Fukushima journal of medical scienceComplement your knowledge with a rare cause of pauci-immune glomerulonephritis.
Clinical rheumatologyComplement 3 glomerulonephritis in rheumatoid arthritis: A case report and follow-up.
Experimental and therapeutic medicineA Case of Switch from C3 Glomerulonephritis to Proliferative Glomerulonephritis with Monoclonal IgG Deposits.
Annals of clinical and laboratory scienceBe on Target: Strategies of Targeting Alternative and Lectin Pathway Components in Complement-Mediated Diseases.
Frontiers in immunologyMembranoproliferative glomerulonephritis and C3 glomerulopathy in children: change in treatment modality? A report of a case series.
Clinical kidney journalA case of recurrent proliferative glomerulonephritis with monoclonal IgG deposits or de novo C3 glomerulonephritis after kidney transplantation.
Nephrology (Carlton, Vic.)C3 glomerulonephritis and thrombotic microangiopathy of renal allograft after pulmonary infection in a male with concomitant two complement factor I gene variations: a case report.
BMC nephrologyC3 glomerulonephritis associated with monoclonal gammopathy of renal significance: case report.
BMC nephrologyC3 glomerulopathy associated with monoclonal Ig is a distinct subtype.
Kidney internationalFavorable effect of bortezomib in dense deposit disease associated with monoclonal gammopathy: a case report.
BMC nephrologyC3 glomerulopathy in cystic fibrosis: a case report.
BMC nephrologyGenetic analysis of the complement pathway in C3 glomerulopathy.
Nephrology, dialysis, transplantation : official publication of the European Dialysis and Transplant Association - European Renal AssociationClinicopathological Spectrum of Glomerular Diseases in Adolescents: A Single-center Experience over 4 Years.
Indian journal of nephrologyC3 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 AssociationTreating C3 glomerulopathy with eculizumab.
BMC nephrologyC3 glomerulonephritis and dense deposit disease share a similar disease course in a large United States cohort of patients with C3 glomerulopathy.
Kidney internationalFurther delineation of the SOX18-related Hypotrichosis, Lymphedema, Telangiectasia syndrome (HTLS).
European journal of medical geneticsHigh-dose melphalan and autologous hematopoietic stem cell transplant in patient with C3 glomerulonephritis associated with monoclonal gammopathy .
Clinical nephrologyKidney Diseases Associated With Alternative Complement Pathway Dysregulation and Potential Treatment Options.
The American journal of the medical sciencesMultimodal imaging of retinal pigment epithelial detachments in patients with C3 glomerulopathy: case report and review of the literature.
BMC ophthalmologyRecurrent allograft C3 glomerulonephritis and unsuccessful eculizumab treatment.
Clinical immunology (Orlando, Fla.)Cluster Analysis Identifies Distinct Pathogenetic Patterns in C3 Glomerulopathies/Immune Complex-Mediated Membranoproliferative GN.
Journal of the American Society of Nephrology : JASNMidterm Outcomes of 12 Renal Transplant Recipients Treated With Eculizumab to Prevent Atypical Hemolytic Syndrome Recurrence.
TransplantationC4 Nephritic Factors in C3 Glomerulopathy: A Case Series.
American journal of kidney diseases : the official journal of the National Kidney FoundationA novel CFHR1-CFHR5 hybrid leads to a familial dominant C3 glomerulopathy.
Kidney internationalC5 nephritic factors drive the biological phenotype of C3 glomerulopathies.
Kidney internationalDifferent types of glomerulonephritis associated with the dysregulation of the complement alternative pathway in 2 brothers: A case report.
MedicineC3 glomerulonephritis with a severe crescentic phenotype.
Pediatric nephrology (Berlin, Germany)Rituximab for Treatment of Membranoproliferative Glomerulonephritis and C3 Glomerulopathies.
BioMed research internationalControlling the anaphylatoxin C5a in diseases requires a specifically targeted inhibition.
Clinical immunology (Orlando, Fla.)Diagnostic dilemmas in a girl with acute glomerulonephritis: Answers.
Pediatric nephrology (Berlin, Germany)C3 glomerulopathy and eculizumab: a report on four paediatric cases.
Pediatric nephrology (Berlin, Germany)Mini review: A unique case of crescentic C3 glomerulonephritis.
Nephrology (Carlton, Vic.)Durable remission of C3 glomerulonephritis with mycophenolate mofetil.
Nephrology (Carlton, Vic.)Complement related kidney diseases: Recurrence after transplantation.
World journal of transplantationC3 glomerulopathy in adults: a distinct patient subset showing frequent association with monoclonal gammopathy and poor renal outcome.
Clinical kidney journalAssociações
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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.
- Clinical Profile and Outcome of C3-Dominant Glomerulonephritis: Retrospective Study.
- A pediatric case of C3 glomerulonephritis initially misclassified as IgA nephropathy with a favorable response to C3-targeted therapy.
- C3 Glomerulopathy Diagnosis, Current Treatments, and Emerging Therapies.
- Primary membranoproliferative glomerulonephritis: natural history, pathogenesis, and treatment.
- Proteomic Profiling of Complement Components in Glomerular Disease.Laboratory investigation; a journal of technical methods and pathology· 2026· PMID 41720186mais citado
- Complement 3 Glomerulopathy (C3G) in Native and Posttransplant Kidneys: Pathophysiology, Prognosis, and Treatment.
- Finding the etiology of membranoproliferative glomerulonephritis.
Bases de dados e fontes oficiais
Identificadores e referências canônicas usadas para montar este verbete.
- ORPHA:329931(Orphanet)
- OMIM OMIM:614809(OMIM)
- MONDO:0013892(MONDO)
- GARD:16487(GARD (NIH))
- Variantes catalogadas(ClinVar)
- Busca completa no PubMed(PubMed)
- Q55784385(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
