{"orpha":97560,"name":"Primary membranous glomerulonephritis","type":"Disease","group":"Disorder","status":"active","flags":[],"synonyms":["Idiopathic membranous glomerulonephritis","Primary membranous nephropathy"],"definition":"A rare glomerular disease, histologically characterized by thickening of the capillary wall, with immune deposits predominantly containing IgG4 and C3 on the sub-epithelial side, and typically manifesting with nephrotic syndrome.","note":null,"url":"https://raremap.alftech.space/d/ORPHA-97560/","orphanet_url":"https://www.orpha.net/en/disease/detail/97560","names":{"fr":{"name":"Glomérulonéphrite extra membraneuse primitive","synonyms":["Glomérulonéphrite extra-membraneuse idiopathique","Néphropathie extra-membraneuse primitive"],"definition":"Rare néphropathie glomérulaire primitive, caractérisée histologiquement par un épaississement des parois capillaires avec des dépôts immuns principalement d'IgG4 et de C3 sur le versant sous-épithélial et des manifestations typiques de syndrome néphrotique."},"de":{"name":"Glomerulonephritis, primäre membranöse","synonyms":["Glomerulonephritis, idiopathische membranöse","Nephropathie, primäre membranöse"],"definition":"Eine seltene glomeruläre Erkrankung, die histologisch durch eine Verdickung der Kapillarwände mit subepithelialer Ablagerung von vorwiegend IgG4- und C3-haltigen Immunkomplexen gekennzeichnet ist und sich meist mit einem nephrotischen Syndrom manifestiert."},"es":{"name":"Glomerulonefritis membranosa primaria","synonyms":["Glomerulonefritis membranosa idiopática","Nefropatía membranosa primaria"],"definition":"Es una enfermedad glomerular poco frecuente caracterizada histológicamente por engrosamiento de la pared capilar, con depósitos inmunes de localización subepitelial integrados predominantemente por IgG4 y C3 , y que se manifiesta típicamente con síndrome nefrótico."},"it":{"name":"Glomerulonefrite membranosa primitiva","definition":"È una glomerulopatia rara caratterizzata, dal punto di vista istologico, dall'ispessimento delle pareti dei capillari, con depositi immuni contenenti soprattutto IgG4 e C3 nella porzione sottoepiteliale. Di solito la malattia si manifesta con una sindrome nefrosica."},"nl":{"name":"Primaire membraneuze glomerulonefritis","synonyms":["Idiopathische membraneuze glomerulonefritis","Primaire membraneuze nefropathie"],"definition":"Een zeldzame glomerulaire ziekte die histologisch gekarakteriseerd wordt door verdikking van capillaire wanden, met afzetting aan de subepitheliale zijde van voornamelijk IgG4- en C3-houdende immuuncomplexen, en die zich doorgaans manifesteert met nefrotisch syndroom."},"pt":{"name":"Glomerulonefrite membranosa, idiopática"},"pl":{"name":"Idiopatyczne błoniaste kłębuszkowe zapalenie nerek","synonyms":["Idiopathic membranous glomerulonephritis","Primary membranous nephropathy"]},"cs":{"name":"Idiopatická membranózní glomerulonefritida","synonyms":["Idiopathic membranous glomerulonephritis","Primary membranous nephropathy"]},"uk":{"name":"Первинний мембранозний гломерулонефрит","synonyms":["Ідіопатичний мембранозний гломерулонефрит","Первинна мембранозна нефропатія"],"definition":"A rare glomerular disease, histologically characterized by thickening of the capillary wall, with immune deposits predominantly containing IgG4 and C3 on the sub-epithelial side, and typically manifesting with nephrotic syndrome."},"zh":{"name":"原发性膜性肾小球肾炎","synonyms":["特发性膜性肾小球肾炎"],"definition":"一种罕见的肾小球疾病，组织学特征为毛细血管壁增厚伴上皮下侧主要为IgG4和C3的免疫沉淀物，典型表现为肾病综合征。"},"tr":{"name":"Primer membranöz glomerülonefrit","synonyms":["Primer membranöz nefropati","İdiyopatik membranöz glomerülonefrit"]}},"xrefs":[{"source":"OMIM","code":"614692","qualifier":"E","icd_relation":null,"validated":true},{"source":"ICD-10","code":"N05.2","qualifier":"NTBT","icd_relation":"attributed","validated":true},{"source":"ICD-11","code":"GB40","qualifier":"NTBT","icd_relation":"attributed","validated":true},{"source":"MONDO","code":"0013860","qualifier":"E","icd_relation":null,"validated":true},{"source":"UMLS","code":"C0086445","qualifier":"E","icd_relation":null,"validated":true},{"source":"GARD","code":"9180","qualifier":"E","icd_relation":null,"validated":true}],"associations":[],"incoming_associations":[],"preferential_parent":{"orpha":93626,"name":"Rare renal disease"},"parents":[{"orpha":93548,"name":"Glomerular disease","classifications":[188,233]}],"children":[],"genes":[],"genes_via_subtypes":[],"inheritance":[],"onset":["Adult"],"prevalence":[{"type":"Annual incidence","qualification":"Value and class","class":"1-9 / 1 000 000","value":0.9194,"area":"Europe","status":"Validated","source":"21068142[PMID]_ORPHANET"},{"type":"Annual incidence","qualification":"Value and class","class":"1-9 / 1 000 000","value":0.8103,"area":"Worldwide","status":"Validated","source":"21068142[PMID]_ORPHANET"},{"type":"Point prevalence","qualification":"Class only","class":"Unknown","value":0.0,"area":"Worldwide","status":"Validated","source":"21068142[PMID]_34593809[PMID]_ORPHANET"}],"phenotypes":{"validation_status":"y","online":"y","validation_date":"2024-03-04","source":"34277215[PMID]_34169209[PMID]","terms":[{"hpo":"HP:0034002","label":"Anti-phospholipase A2 receptor antibody positivity","frequency":"Frequent (79-30%)","criteria":null}]}}