{"Name":"Immunotactoid or fibrillary glomerulopathy","DiseaseID__c":"GARD:0012741","id":12741,"encodedName":"immunotactoid-or-fibrillary-glomerulopathy","IsDeleted":false,"Disease_Name_Full__c":"Immunotactoid or fibrillary glomerulopathy","Xref_IDs__c":"C5680195; MEDGEN:1842578; MONDO:0019605; ORPHA:91137","USA_Estimate__c":"1,000","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":"1 to 8,000","No_of_HRSA_records__c":0,"Evidence_Based_Score__c":0,"No_of_Disease_Descriptions__c":4,"Disease_Characteristics_Score__c":4,"No_of_Age_at_Onset__c":1,"Description_Source__c":"MONDO:0019605","Disease_Description__c":"Immunotactoid or fibrillary glomerulopathy is a group of very rare glomerular diseases, composed of immunotactoid glomerulopathy (ITG) and non-amyloid fibrillary glomerulopathy (non-amyloid FGP) (see these terms), that are characterized by mesangial deposition of monoclonal microtubular or polyclonal fibrillar deposits. Both present clinically with nephrotic range proteinuria, hematuria and renal insufficiency leading to renal failure in many cases. ITG is more likely to manifest with underlying lymphoproliferative disease, hypocomplementemia, dysproteinemia, monoclonal gammopathy or occult cryoglobulinemia. Non-amyloid FGP is 10 times more frequent than ITG.","GARD_Name__c":"Immunotactoid or fibrillary glomerulopathy","GARD_Synonym__c":"immunotactoid or fibrillary glomerulonephritis","Curated_Disease_Description_Source__c":"GARD:0012741","Curated_Disease_Description__c":"Immunotactoid or fibrillary glomerulopathy is a term that includes two conditions: immunotactoid glomerulopathy and fibrillary glomerulonephritis, which are uncommon causes of glomerular disease. Most experts feel that fibrillary glomerulonephritis and immunotactoid glomerulopathy are separate disorders but they have many similarities and some experts group these disorders together. Fibrillary glomerulonephritis and immunotactoid glomerulopathy can be distinguished from each other by electron microscopy; the 'fibrils' that characterize fibrillary glomerulonephritis are smaller and randomly oriented as opposed to the larger and organized fibrils of immunotactoid glomerulopathy which also have microtubule formations. Both disorders probably result from deposits derived from immunoglobulins but in most cases the cause is idiopathic (unknown). The signs and symptoms are similar in both diseases and may include blood (hematuria) and protein (proteinuria) in the urine, kidney insufficiency and high blood pressure. Fibrillary glomeurlonephritis is much more common than immunotactoid glomerulopathy. Both fibrillary glomerulonephritis and immunotactoid glomerulopathy have been associated with hepatitis C virus infection and with malignancy and autoimmune disease, but immunotactoid glomerulopathy patients have a greater predisposition to chronic lymphocytic leukemia and B cell lymphomas. All patients should be screened for these conditions. It is also important to rule out another disease known as amyloidosis. When the fibrils are stained with an acid dye known as \"Congo red\" the results are negative. In amyloidosis the results are positive because the dye is absorbed by the amyloids.","Curated_USA_Estimate_Source__c":null,"Curated_USA_Estimate__c":"1,000","Age_at_Onset_Snippet_Text__c":"as an Adult","SourceID__c":"ORPHA:91137","Deprecated__c":"No","Disease_Concept_Type__c":"Rare Disease Grouping","MONDO_ID__c":"MONDO:0019605","ORPHANET_ID__c":"ORPHA:91137","Replaced_By_ID__c":null,"Display_Spanish_Disease_Name__c":"Glomerulopatía inmunotactoide o fibrilar","Spanish_Description_Source__c":"ORPHA:91137","Spanish_Description__c":"Es un grupo de enfermedades glomerulares muy poco frecuentes integrado por la glomerulopatía inmunotactoide (GIT) y la glomerulopatía fibrilar no-amiloide (GPF), caracterizado por depósitos microtubulares monoclonales o fibrilares policlonales a nivel mesangial. Clínicamente se caracterizan por presentar proteinuria en rango nefrótico, hematuria e insuficiencia renal que con frecuencia progresa a enfermedad renal crónica terminal. La glomerulopatía inmunotactoide o fibrilar se suele asociar con trastornos linfoproliferativos subyacentes, hipocomplementemia, disproteinemia, gammapatía monoclonal o crioglobulinemia oculta. La GPF no-amiloide es 10 veces más frecuente que la GIT.","Spanish_Disease_Name__c":"glomerulopatía inmunotactoide o fibrilar","Spanish_GARD_Synonym__c":"glomerulonefritis fibrilar o inmunotactoide; glomerulonefritis inmunotactoide o fibrilar","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":"Immunotactoid or fibrillary glomerulopathy is a term that includes two conditions: immunotactoid glomerulopathy and fibrillary glomerulonephritis, which are uncommon causes of glomerular disease. Most experts feel that fibrillary glomerulonephritis and immunotactoid glomerulopathy are separate disorders but they have many similarities and some experts group these disorders together. Fibrillary glomerulonephritis and immunotactoid glomerulopathy can be distinguished from each other by electron microscopy; the 'fibrils' that characterize fibrillary glomerulonephritis are smaller and randomly oriented as opposed to the larger and organized fibrils of immunotactoid glomerulopathy which also have microtubule formations. Both disorders probably result from deposits derived from immunoglobulins but in most cases the cause is idiopathic (unknown). The signs and symptoms are similar in both diseases and may include blood (hematuria) and protein (proteinuria) in the urine, kidney insufficiency and high blood pressure. Fibrillary glomeurlonephritis is much more common than immunotactoid glomerulopathy. Both fibrillary glomerulonephritis and immunotactoid glomerulopathy have been associated with hepatitis C virus infection and with malignancy and autoimmune disease, but immunotactoid glomerulopathy patients have a greater predisposition to chronic lymphocytic leukemia and B cell lymphomas. All patients should be screened for these conditions. It is also important to rule out another disease known as amyloidosis. When the fibrils are stained with an acid dye known as \"Congo red\" the results are negative. In amyloidosis the results are positive because the dye is absorbed by the amyloids.","Curated_Disease_Description_Source__c":"GARD:0012741","GARD_Synonym__c":"immunotactoid or fibrillary glomerulonephritis","Name":"Immunotactoid or fibrillary glomerulopathy","Curated_USA_Estimate__c":"1,000","estimateUsa":"1,000"}],"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:91137"}],"External_Identifier_Disease__c":[{"URL__c":"https://uts.nlm.nih.gov/uts/umls/concept/C5680195","Source__c":"C5680195","Xref__c":"C5680195"},{"URL__c":"https://www.orpha.net/en/disease/detail/91137","Source__c":"C5680195; MONDO:0019605; ORPHA:91137","Xref__c":"ORPHA:91137"},{"URL__c":"https://www.ncbi.nlm.nih.gov/medgen/?term=1842578","Source__c":"C5680195","Xref__c":"MEDGEN:1842578"},{"URL__c":"http://purl.obolibrary.org/obo/MONDO_0019605","Source__c":"GARD:0012741","Xref__c":"MONDO:0019605"}],"tags":{"Account":["Nephrology"],"Disease Category":["Nephrology"],"Specialist":["Nephrology"]},"synonyms":["immunotactoid or fibrillary glomerulonephritis"]}