{"Name":"Idiopathic membranous glomerulonephritis","DiseaseID__c":"GARD:0009180","id":9180,"encodedName":"idiopathic-membranous-glomerulonephritis","IsDeleted":false,"Disease_Name_Full__c":"Idiopathic membranous glomerulonephritis","Xref_IDs__c":"722119002; C0086445; C123060; MEDGEN:39226; MONDO:0013860; OMIM:614692; ORPHA:97560","USA_Estimate__c":"5,000","No_of_Specialist_Tagsa__c":1,"No_of_ClinGen_records__c":0,"No_of_GeneReviews__c":0,"No_of_HHS_records__c":0,"World_Estimate__c":"8,000 to 80,000","No_of_HRSA_records__c":0,"Evidence_Based_Score__c":0,"No_of_Disease_Descriptions__c":4,"Disease_Characteristics_Score__c":5,"No_of_Age_at_Onset__c":1,"Description_Source__c":"MONDO:0013860","Disease_Description__c":"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.","GARD_Name__c":"Idiopathic membranous glomerulonephritis","GARD_Synonym__c":"membranous nephropathy - idiopathic; primary membranous glomerulonephritis; primary membranous nephropathy","Curated_Disease_Description_Source__c":"GARD:0009180","Curated_Disease_Description__c":"Primary membranous glomerulonephritis is a kidney disease characterized by inflammation of the structures inside the kidney that help filter wastes and fluids. When the glomerular basement membrane becomes thickened, it does not work normally, allowing large amounts of protein to be lost in the urine. Symptoms develop gradually and may include swelling, fatigue, weight gain, and high blood pressure. In many cases, the underlying cause of primary membranous glomerulonephritis is not known. Some cases are associated with other conditions (lupus), infections (hepatitis B and C), cancer or as a side effect of certain medications.","Curated_USA_Estimate_Source__c":null,"Curated_USA_Estimate__c":"5,000","Age_at_Onset_Snippet_Text__c":"as an Adult","SourceID__c":"ORPHA:97560","Deprecated__c":"No","Disease_Concept_Type__c":"Rare Disease Entity","MONDO_ID__c":"MONDO:0013860","ORPHANET_ID__c":"ORPHA:97560","Replaced_By_ID__c":null,"Display_Spanish_Disease_Name__c":"Glomerulonefritis membranosa primaria","Spanish_Description_Source__c":"ORPHA:97560","Spanish_Description__c":"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.","Spanish_Disease_Name__c":"glomerulonefritis membranosa primaria","Spanish_GARD_Synonym__c":"glomerulonefritis membranosa idiopática; nefropatía membranosa primaria","Category_Linearization__c":"ORPHA:93626","icd10_id__c":null,"mesh_id__c":null,"omim_id__c":null,"snomed_id__c":null,"umls_id__c":null,"GARD_Disease__c":[{"Curated_Disease_Description__c":"Primary membranous glomerulonephritis is a kidney disease characterized by inflammation of the structures inside the kidney that help filter wastes and fluids. When the glomerular basement membrane becomes thickened, it does not work normally, allowing large amounts of protein to be lost in the urine. Symptoms develop gradually and may include swelling, fatigue, weight gain, and high blood pressure. In many cases, the underlying cause of primary membranous glomerulonephritis is not known. Some cases are associated with other conditions (lupus), infections (hepatitis B and C), cancer or as a side effect of certain medications.","Curated_Disease_Description_Source__c":"GARD:0009180","GARD_Synonym__c":"membranous nephropathy - idiopathic; primary membranous glomerulonephritis; primary membranous nephropathy","Name":"Idiopathic membranous glomerulonephritis","Curated_USA_Estimate__c":"5,000","estimateUsa":"5,000"}],"Organization_Supported_Diseases__c":[{"Account_Name__c":"NephCure Kidney International","Website__c":"https://nephcure.org/"},{"Account_Name__c":"Halpin Foundation","Website__c":"http://www.halpinfoundation.org/index.html"}],"GARD_Disease_Tag__c":[{"Tag_Name__c":"Nephrology","Tag_Category__c":"Account;Disease Category;Specialist","category_description":"Kidney diseases affect the kidneys' ability to remove waste and water from blood, create urine, or make certain hormones.","curated_tag_name":"Kidney diseases"}],"Age_At_Onset__c":[{"Age_At_Onset__c":"Adult","Provided_By__c":"ORPHA:97560"}],"External_Identifier_Disease__c":[{"URL__c":"https://www.ncbi.nlm.nih.gov/medgen/?term=39226","Source__c":"C0086445","Xref__c":"MEDGEN:39226"},{"URL__c":"https://www.orpha.net/en/disease/detail/97560","Source__c":"C0086445; MONDO:0013860; ORPHA:97560","Xref__c":"ORPHA:97560"},{"URL__c":"https://uts.nlm.nih.gov/uts/umls/concept/C0086445","Source__c":"C0086445","Xref__c":"C0086445"},{"URL__c":"https://evsexplore.semantics.cancer.gov/evsexplore/concept/ncit/C123060","Source__c":"C0086445; MONDO:0013860","Xref__c":"C123060"},{"URL__c":"https://www.omim.org/entry/614692","Source__c":"MONDO:0013860; ORPHA:97560","Xref__c":"OMIM:614692"},{"URL__c":"https://browser.ihtsdotools.org/?perspective=full&conceptId1=722119002","Source__c":"C0086445; MONDO:0013860","Xref__c":"722119002"},{"URL__c":"http://purl.obolibrary.org/obo/MONDO_0013860","Source__c":"GARD:0009180","Xref__c":"MONDO:0013860"}],"GARD_Disease_Feature__c":[{"Provided_By__c":"ORPHA:97560","HPO_Frequency__c":"Frequent (30-79%)","Feature__r":{"HPO_Description__c":"The presence of autoantibodies (immunoglobulins) in the blood circulation that react against the phospholipase A2 receptor.","HPO_Feature_URL__c":"https://hpo.jax.org/browse/term/HP:0034002","HPO_Synonym__c":"Anti-PLA2R antibody positivity","HPO_Name__c":"Anti-phospholipase A2 receptor antibody positivity","Feature_System__c":"Immune System","HPO_Feature_Type__c":"Lab"}}],"tags":{"Account":["Nephrology"],"Disease Category":["Nephrology"],"Specialist":["Nephrology"]},"synonyms":["membranous nephropathy - idiopathic"," primary membranous glomerulonephritis"," primary membranous nephropathy"]}