{"orpha":91137,"name":"Immunotactoid or fibrillary glomerulopathy","type":"Clinical group","group":"Group of disorders","status":"active","flags":[],"synonyms":["Immunotactoid or fibrillary glomerulonephritis"],"definition":"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), 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.","note":null,"url":"https://raremap.alftech.space/d/ORPHA-91137/","orphanet_url":"https://www.orpha.net/en/disease/detail/91137","names":{"fr":{"name":"Glomérulopathie immunotactoïde ou fibrillaire","synonyms":["Glomérulonéphrite immunotactoïde ou fibrillaire"],"definition":"Groupe de néphropathies glomérulaires très rares, comprenant la glomérulopathie immunotactoïde (GIT) et la glomérulopathie fibrillaire non amyloïde (GFNA), qui se caractérisent par l'accumulation mésangiale de dépôts microtubulaires monoclonaux ou de dépôts fibrillaires polyclonaux. Sur le plan clinique, ces deux maladies se caractérisent par une protéinurie de type néphrotique, une hématurie et une insuffisance rénale conduisant, dans de nombreux cas, à une insuffisance rénale terminale. La GTI est plus susceptible de se manifester en association avec une maladie lymphoproliférative, une hypocomplémentémie, une dysprotéinémie, une gammapathie monoclonale ou une cryoglobulinémie occulte sous-jacentes. La GFNA est 10&nbsp;fois plus fréquente que la GTI."},"de":{"name":"Glomerulopathie, immunotaktoide oder fibrilläre","synonyms":["Glomerulonephritis, immunotaktoide oder fibrilläre"],"definition":"Die immunotaktische oder fibrilläre Glomerulopathie ist eine Gruppe sehr seltener glomerulärer Erkrankungen, bestehend aus immunotaktischer Glomerulopathie (ITG) und nicht amyloider fibrillärer Glomerulopathie (nicht amyloide FGP) (s. dort), die durch mesangiale Ansammlungen monoklonaler mikrotubulärer oder polyklonaler fibrillärer Ablagerungen gekennzeichnet sind. Beide zeigen klinisch eine im nephrotischen Bereich liegende Proteinurie, eine Hämaturie und eine Niereninsuffizienz, die in vielen Fällen zu Nierenversagen führen. ITG manifestiert sich häufiger mit einer zugrunde liegenden lymphoproliferativen Erkrankung, Hypokomplementämie, Dysproteinämie, monoklonaler Gammopathie oder okkulter Kryoglobulinämie. Die nicht amyloide FGP ist 10-mal häufiger als die ITG."},"es":{"name":"Glomerulopatía inmunotactoide o fibrilar","synonyms":["Glomerulonefritis fibrilar o inmunotactoide","Glomerulonefritis inmunotactoide o fibrilar"],"definition":"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."},"it":{"name":"Glomerulopatia immunotattoide o fibrillare","definition":"La glomerulopatia fibrillare o immunotattoide appartiene al gruppo delle malattie glomerulari molto rare, che comprende la glomerulopatia immunotattoide (ITG) e la glomerulopatia fibrillare non amiloide (FGP non amiloide; si vedano questi termini). Si tratta di malattie caratterizzate dall'accumulo mesangiale di depositi fibrillari microtubulari o policlonali. Entrambe condividono alcuni segni clinici come la proteinuria di tipo nefrosico, l'ematuria e l'insufficienza renale che, in molti casi, esita nel blocco renale. La ITG spesso si associa ad una malattia linfoproliferativa preesistente, ad ipocomplementemia, disproteinemia, gammopatia monoclonale o crioglobulinemia occulta. La FGP non amiloide è dieci volte più frequente della ITG."},"nl":{"name":"Immunotactoïde of fibrillaire glomerulopathie","synonyms":["Immunotactoïde of fibrillaire glomerulonefritis"],"definition":"Immunotactoïde of fibrillaire glomerulopathie is een groep zeer zeldzame glomerulaire ziekten, bestaande uit immunotactoïde glomerulopathie (ITG) en niet-amyloïde fibrillaire glomerulopathie (niet-amyloïde FGP) (zie deze termen), die gekarakteriseerd worden door monoklonale microtubulaire of polyklonale fibrillaire afzettingen in het mesangium. Klinisch vertonen beide ziekten proteïnurie in het nefrotische bereik, hematurie en nierinsufficiëntie, die in vele gevallen leidt tot nierfalen. ITG manifesteert zich vaker met onderliggende lymfoproliferatieve ziekte, hypocomplementemie, dysproteïnemie, monoklonale gammopathie of occulte cryoglobulinemie. Niet-amyloïde FGP komt 10 maal meer voor dan ITG."},"pt":{"name":"Glomerulopatia imunotactóide ou fibrilar","synonyms":["Glomerulonefrite imunotactóide ou fibrilar"],"definition":"As glomerulopatias imunotactóides ou fibrilares são doenças renais de depósito muito raras, que frequentemente são agrupadas."},"pl":{"name":"Glomerulopatia immunotaktoidalna lub włókienkowa","synonyms":["Immunotactoid or fibrillary glomerulonephritis"],"definition":"Glomerulopatia immunotaktoidalna lub włókienkowa to grupa bardzo rzadkich chorób kłębuszków nerkowych, do której należy glomerulopatia immunotaktoidalna (ITG) i nieamyloidowa glomerulopatia włókienkowa (nieamyloidowa FGP) , które charakteryzują się odkładaniem się w mezangium monoklonalnych depozytów mikrotubularnych lub poliklonalnych depozytów włókienkowych. W obydwu jednostkach występuje białkomocz pochodzenia nerkowego, krwiomocz i niedoczynność nerek w wielu przypadkach prowadząca do niewydolności. ITG jest najczęściej przejawem leżącej u jej podstaw choroby limfoproliferacyjnej, hipokomplementemii, dysproteinemii, gammopatii monoklonalnej lub utajonej krioglobulinemii. Nieamyloidowa FGP występuje 10 razy częściej niż ITG."},"cs":{"name":"Imunotaktoidní nebo fibrilární glomerulopatie","synonyms":["Imunotaktoidní nebo fibrilární glomerulonefritida"]},"uk":{"name":"Імунотактоїдна або фібрилярна гломерулопатія","synonyms":["Імунотактоїдний або фібрилярний гломерулонефрит"],"definition":"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."},"zh":{"name":"免疫触须样或纤维性肾小球肾病","synonyms":["免疫触须样或纤维样肾小球病"]},"tr":{"name":"İmmünotaktoid veya fibriler glomerülopati","synonyms":["İmmünotaktoid veya fibriler glomerülonefrit"]}},"xrefs":[{"source":"MONDO","code":"0019605","qualifier":"E","icd_relation":null,"validated":true},{"source":"UMLS","code":"C5680195","qualifier":"E","icd_relation":null,"validated":true},{"source":"GARD","code":"12741","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":[{"orpha":97567,"name":"Immunotactoid glomerulopathy","type":"Disease"},{"orpha":97566,"name":"Non-amyloid fibrillary glomerulopathy","type":"Disease"}],"genes":[],"genes_via_subtypes":[],"inheritance":["Not applicable"],"onset":["Adult"],"prevalence":[{"type":"Point prevalence","qualification":"Class only","class":"<1 / 1 000 000","value":0.0,"area":"Europe","status":"Not yet validated","source":"ORPHANET"}],"phenotypes":null}