{"orpha":329918,"name":"C3 glomerulopathy","type":"Clinical subtype","group":"Subtype of disorder","status":"active","flags":[],"synonyms":["Non-Ig-mediated MPGN","Non-Ig-mediated membranoproliferative glomerulonephritis","Non-immunoglobulin-mediated MPGN","Non-immunoglobulin-mediated membranoproliferative glomerulonephritis"],"definition":"A form of primary membranoproliferative glomerulonephritis characterized by the presence in renal biopsy samples of a glomerulonephritis with sole (or at least dominant) glomerular immunofluorescence staining for C3. Non-specific alterations or proliferative patterns with C3-dominant glomerular staining are also possible. Based upon electron microscopic findings, C3 glomerulopathy (C3G) may be further classified as C3 glomerulonephritis (C3GN) or Dense deposit disease (DDD).","note":null,"url":"https://raremap.alftech.space/d/ORPHA-329918/","orphanet_url":"https://www.orpha.net/en/disease/detail/329918","names":{"fr":{"name":"Glomérulopathie à dépôts de C3","synonyms":["GNMP non médiée par les Ig","GNMP non médiée par les immunoglobulines","Glomérulonéphrite membranoproliférative non médiée par les Ig","Glomérulonéphrite membranoproliférative non médiée par les immunoglobulines","MPGN non médiée par les Ig","MPGN non médiée par les immunoglobulines"],"definition":"Forme de glomérulonéphrite membranoproliférative primaire caractérisée par la présence d'une glomérulonéphrite dans les échantillons de biopsie rénale analysés en immunofluorescence mettant en évidence essentiellement (ou, du moins, de manière prédominante) un marquage caractéristique de C3. Des altérations non spécifiques ou des schémas prolifératifs avec une coloration glomérulaire à dominante C3 sont également possibles. En fonction de l'aspect des dépôts en microscopie électronique, la glomérulopathie à dépôt de C3 (C3G) peut être classée en glomérulonéphrite à dépôts de C3 (GNC3) ou en maladie de dépôt dense (DDD)."},"de":{"name":"C3-Glomerulopathie","synonyms":["Glomerulonephritis, membranoproliferative, nicht Immunoglobulin-vermittelte"],"definition":"Ein Subtyp der primären membranoproliferativen Glomerulonephritis, die dadurch gekennzeichnet ist, dass in Nierenbiopsieproben eine Glomerulonephritis mit ausschließlicher (oder zumindest dominierender) glomerulärer C3-Immunfluoreszenzfärbung vorliegt. Unspezifische Veränderungen oder proliferative Muster mit C3-dominanter glomerulärer Anfärbung sind ebenfalls möglich. Aufgrund elektronenmikroskopischer Befunde kann die C3-Glomerulopathie (C3G) weiter klassifiziert werden als C3-Glomerulonephritis (C3GN) oder Dense Deposit Disease (DDD)."},"es":{"name":"Glomerulopatía C3","synonyms":["Glomerulonefritis membranoproliferativa no mediada por Ig","Glomerulonefritis membranoproliferativa no mediada por inmunoglobulinas","MPGN no mediada por Ig","MPGN no mediada por inmunoglobulinas"],"definition":"Es una forma de glomerulonefritis membranoproliferativa primaria caracterizada por la presencia de glomerulonefritis en la biopsia renal con tinción de inmunofluorescencia glomerular única (o al menos dominante) para C3. También son posibles las alteraciones inespecíficas o los patrones proliferativos con tinción glomerular dominante de C3. Según los hallazgos de microscopía electrónica, la glomerulopatía C3 (GC3) puede clasificarse adicionalmente como glomerulonefritis C3 (GNC3) o enfermedad por depósitos densos (EDD)."},"it":{"name":"Glomerulopatia da C3","synonyms":["Glomerulonefrite membranoproliferativa non-Ig-mediata","Glomerulopatia C3","MPGN non mediata dalle immunoglobuline","MPGN non-Ig-mediata"],"definition":"Si tratta di una forma di glomerulonefrite membranoproliferativa primitiva, caratterizzata dalla presenza, nei campioni delle biopsie renali analizzati con l'immunofluorescenza, di una glomerulonefrite con colorazione glomerulare esclusivamente (o prevalentemente) positiva per C3. Sono possibili anche alterazioni aspecifiche o proliferazioni con colorazione glomerulare dominante per C3. L'analisi al microscopio elettronico permette di classificare ulteriormente la glomerulopatia da C3 (C3G) in glomerulonefrite da C3 (C3GN) o malattia da depositi densi (DDD)."},"nl":{"name":"C3-glomerulopathie","synonyms":["Niet-Ig-gemedieerde MPGN","Niet-Ig-gemedieerde membranoproliferatieve glomerulonefritis","Niet-immunoglobuline-gemedieerde MPGN","Niet-immunoglobuline-gemedieerde membranoproliferatieve glomerulonefritis"],"definition":"Een vorm van primaire membranoproliferatieve glomerulonefritis, gekenmerkt door de aanwezigheid in nierbiopt van glomerulonefritis met uitsluitend (of minstens dominante) glomerulaire immunofluorescentiekleuring voor C3. Aspecifieke veranderingen of proliferatieve patronen met C3-dominante glomerulaire kleuring zijn ook mogelijk. Op basis van bevindingen van elektronenmicroscopie kan C3-glomerulopathie (C3G) verder geclassificeerd worden als C3-glomerulonefritis (C3GN) of dense deposit-ziekte (DDD)."},"pt":{"name":"Glomerulopatia C3","synonyms":["Glomerulonefrite membranoproliferativa não mediada por imunoglobulina","Glomerulonefrite membranoproliferativa não mediada por Ig"],"definition":"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)."},"pl":{"name":"Błoniasto-rozplemowe kłębuszkowe zapalenie nerek nie wywoływane przez immunoglobuliny","synonyms":["Błoniasto-rozplemowe kłębuszkowe zapalenie nerek nie wywoływane przez Ig","Glomerulopatia C3","MPGN nie związane z Ig","MPGN nie związane z przeciwciałami MPGN"]},"cs":{"name":"Membranoproliferativní glomerulonefritida nezprostředkovaná imunoglobuliny","synonyms":["Glomerulopatie se sníženým C3","MPGN nezprostředkovaná Ig","Membranoproliferativní glomerulonefritida nezprostředkovaná Ig","MPGN nezprostředkovaná imunoglobuliny"]},"uk":{"name":"С3 гломерулопатія","synonyms":["Не-Ig-опосередкований мембранопроліферативний гломерулонефрит","MPGN без Ig-опосередкування","Неімуноглобулін-опосередкований мембранопроліферативний гломерулонефрит","Неімуноглобулін-опосередкований MPGN"],"definition":"A form of primary membranoproliferative glomerulonephritis characterized by the presence in renal biopsy samples of a glomerulonephritis with sole (or at least dominant) glomerular immunofluorescence staining for C3. Non-specific alterations or proliferative patterns with C3-dominant glomerular staining are also possible. Based upon electron microscopic findings, C3 glomerulopathy (C3G) may be further classified as C3 glomerulonephritis (C3GN) or Dense deposit disease (DDD)."},"zh":{"name":"C3肾小球病","synonyms":["非免疫球蛋白介导膜性肾小球肾炎"]},"tr":{"name":"C3 glomerülopati","synonyms":["Ig-aracılı olmayan MPGN","İmmünoglobulin-aracılı olmayan MPGN","Ig-aracılı olmayan membranoproliferatif glomerülonefrit","İmmünoglobulin-aracılı olmayan membranoproliferatif glomerülonefrit"]}},"xrefs":[{"source":"OMIM","code":"609814","qualifier":"BTNT","icd_relation":null,"validated":true},{"source":"OMIM","code":"614809","qualifier":"BTNT","icd_relation":null,"validated":true},{"source":"ICD-10","code":"N03.5","qualifier":"NTBT","icd_relation":"attributed","validated":true},{"source":"ICD-11","code":"MF8Y","qualifier":"NTBT","icd_relation":"attributed","validated":true},{"source":"MONDO","code":"0018013","qualifier":"E","icd_relation":null,"validated":true},{"source":"UMLS","code":"C4087273","qualifier":"E","icd_relation":null,"validated":true},{"source":"MedDRA","code":"10077827","qualifier":"E","icd_relation":null,"validated":true}],"associations":[],"incoming_associations":[],"preferential_parent":{"orpha":93626,"name":"Rare renal disease"},"parents":[{"orpha":54370,"name":"Primary membranoproliferative glomerulonephritis","classifications":[156,188,233]}],"children":[{"orpha":329931,"name":"C3 glomerulonephritis","type":"Histopathological subtype"},{"orpha":93571,"name":"Dense deposit disease","type":"Histopathological subtype"}],"genes":[],"genes_via_subtypes":[],"inheritance":["Multigenic/multifactorial"],"onset":["All ages"],"prevalence":[{"type":"Annual incidence","qualification":"Value and class","class":"1-9 / 1 000 000","value":0.15,"area":"Europe","status":"Validated","source":"27056062[PMID]"}],"phenotypes":{"validation_status":"y","online":"y","validation_date":"2026-05-12","source":"20301598[PMID]_30692664[PMID]_30077216[PMID]_38076230[PMID]_30692664[PMID]_32943008[PMID]","terms":[{"hpo":"HP:0000790","label":"Hematuria","frequency":"Very frequent (99-80%)","criteria":null},{"hpo":"HP:0030888","label":"C3 nephritic factor positivity","frequency":"Frequent (79-30%)","criteria":null},{"hpo":"HP:0012622","label":"Chronic kidney disease","frequency":"Frequent (79-30%)","criteria":null},{"hpo":"HP:0005421","label":"Decreased circulating complement C3 concentration","frequency":"Frequent (79-30%)","criteria":null},{"hpo":"HP:0003259","label":"Elevated circulating creatinine concentration","frequency":"Frequent (79-30%)","criteria":null},{"hpo":"HP:0000822","label":"Hypertension","frequency":"Frequent (79-30%)","criteria":null},{"hpo":"HP:0000793","label":"Membranoproliferative glomerulonephritis","frequency":"Frequent (79-30%)","criteria":null},{"hpo":"HP:0012574","label":"Mesangial hypercellularity","frequency":"Frequent (79-30%)","criteria":null},{"hpo":"HP:0012593","label":"Nephrotic range proteinuria","frequency":"Frequent (79-30%)","criteria":null},{"hpo":"HP:0000093","label":"Proteinuria","frequency":"Frequent (79-30%)","criteria":null},{"hpo":"HP:0003774","label":"Stage 5 chronic kidney disease","frequency":"Frequent (79-30%)","criteria":null},{"hpo":"HP:0030469","label":"Abnormal dark-adapted electroretinogram","frequency":"Occasional (29-5%)","criteria":null},{"hpo":"HP:0001919","label":"Acute kidney injury","frequency":"Occasional (29-5%)","criteria":null},{"hpo":"HP:0034001","label":"Anti-Complement factor H antibody positivity","frequency":"Occasional (29-5%)","criteria":null},{"hpo":"HP:0002960","label":"Autoimmunity","frequency":"Occasional (29-5%)","criteria":null},{"hpo":"HP:0008653","label":"Crescentic glomerulonephritis","frequency":"Occasional (29-5%)","criteria":null},{"hpo":"HP:0045042","label":"Decreased circulating complement C4 concentration","frequency":"Occasional (29-5%)","criteria":null},{"hpo":"HP:0011510","label":"Drusen","frequency":"Occasional (29-5%)","criteria":null},{"hpo":"HP:0009125","label":"Lipodystrophy","frequency":"Occasional (29-5%)","criteria":null},{"hpo":"HP:0000100","label":"Nephrotic syndrome","frequency":"Occasional (29-5%)","criteria":null},{"hpo":"HP:0031047","label":"Paraproteinemia","frequency":"Occasional (29-5%)","criteria":null},{"hpo":"HP:0000572","label":"Visual loss","frequency":"Occasional (29-5%)","criteria":null},{"hpo":"HP:0030506","label":"Yellow/white lesions of the retina","frequency":"Occasional (29-5%)","criteria":null}]}}