{"orpha":34145,"name":"Immunoglobulin A nephropathy","type":"Disease","group":"Disorder","status":"active","flags":[],"synonyms":["Berger disease","IgA nephropathy"],"definition":"A rare glomerular disease, histologically characterized by glomerular mesangial deposits of IgA, often accompanied by IgG and complement C3 as well as mesangioproliferative changes, clinically mostly manifesting as oligosymptomatic glomerulonephritis, possibly infection-triggered macrohematuria and a variable course ranging from spontaneous remission to slow or rarely rapid progression to kidney failure.","note":null,"url":"https://raremap.alftech.space/d/ORPHA-34145/","orphanet_url":"https://www.orpha.net/en/disease/detail/34145","names":{"fr":{"name":"Néphropathie à immunoglobuline A","synonyms":["Maladie de Berger","Néphropathie à IgA"],"definition":"Glomérulopathie rare caractérisée sur le plan histologique par des dépôts mésangiaux glomérulaires d'IgA, souvent accompagnés d'IgG et de complément C3, ainsi que par des lésions de prolifération mésangiale, se manifestant le plus souvent sur le plan clinique par une glomérulonéphrite oligosymptomatique, éventuellement une macrohématurie déclenchée par une infection, ainsi que par une évolution variable allant d'une rémission spontanée à une progression lente ou, dans de rares cas, rapide, vers une insuffisance rénale."},"de":{"name":"Immunglobulin A-Nephropathie","synonyms":["Berger-Krankheit","IgA-Nephropathie"],"definition":"Eine seltene glomeruläre Erkrankung, die histologisch durch glomeruläre mesangiale Ablagerungen von IgA gekennzeichnet ist, häufig begleitet von IgG und Komplement C3 sowie mesangioproliferativen Veränderungen, klinisch meist manifestierend als oligosymptomatische Glomerulonephritis, möglicherweise infektionsausgelöste Makrohämaturie und einem variablen Verlauf, der von spontaner Remission bis zu langsamem oder selten schnellem Fortschreiten bis zur Niereninsuffizienz reicht."},"es":{"name":"Nefropatía por inmunoglobulina A","synonyms":["Enfermedad de Berger","Nefropatía por IgA"],"definition":"Es una enfermedad glomerular poco frecuente caracterizada histológicamente por depósitos de IgA en el mesangio glomerular, a menudo acompañados de IgG y del componente 3 del complemento (C3), así como de cambios mesangioproliferativos. Clínicamente se manifiesta como glomerulonefritis oligosintomática, macrohematuria posiblemente desencadenada por infección y un curso variable que va desde la remisión espontánea hasta la progresión lenta, o rara vez rápida, a insuficiencia renal."},"it":{"name":"Nefropatia da immunoglobulina A","synonyms":["Malattia di Berger","Nefropatia da IgA"]},"nl":{"name":"Immunoglobuline A-nefropathie","synonyms":["IgA-nefropathie","Ziekte van Berger"],"definition":"Een zeldzame glomerulaire aandoening, histologisch gekenmerkt door glomerulaire mesangiale afzettingen van IgA, vaak vergezeld van IgG en complement C3 alsook mesangioproliferatieve veranderingen, en met als klinische manifestaties oligosymptomatische glomerulonefritis, mogelijk door infectie uitgelokte macrohematurie en een variabel verloop gaande van spontane remissie tot trage of in zeldzame gevallen snelle progressie naar nierfalen."},"pt":{"name":"Nefropatia por imunoglobulina A","synonyms":["Doença Berger","Nefropatia de IgA"],"definition":"Doença glomerular rara, caracterizada histologicamente por depósitos mesangiais glomerulares de IgA, frequentemente acompanhados por IgG e complemento C3, bem como alterações mesangioproliferativas, manifestando-se clinicamente principalmente como glomerulonefrite oligossintomática, possivelmente macrohematúria desencadeada por infeção e um curso variável que vai de remissão espontânea a progressão lenta ou raramente rápida para insuficiência renal."},"pl":{"name":"Choroba Bergera","synonyms":["Nefropatia IgA"]},"cs":{"name":"Imunoglobulin A nefropatie","synonyms":["Berger disease","IgA nephropathy"]},"uk":{"name":"Імуноглобулінова нефропатія А","synonyms":["Хвороба Бергера","Нефропатія IgA"],"definition":"A rare glomerular disease, histologically characterized by glomerular mesangial deposits of IgA, often accompanied by IgG and complement C3 as well as mesangioproliferative changes, clinically mostly manifesting as oligosymptomatic glomerulonephritis, possibly infection-triggered macrohematuria and a variable course ranging from spontaneous remission to slow or rarely rapid progression to kidney failure."},"zh":{"name":"欧洲非罕见病：贝格尔疾病","synonyms":["IgA肾病"]},"tr":{"name":"AVRUPA'DA NADİR OLMAYAN: Berger hastalığı","synonyms":["IgA nefropati"]}},"xrefs":[{"source":"ICD-10","code":"N02","qualifier":"NTBT","icd_relation":"index","validated":true},{"source":"ICD-11","code":"MF8Y","qualifier":"NTBT","icd_relation":"index","validated":true,"icd11_uri":"30371100"},{"source":"MONDO","code":"0005342","qualifier":"E","icd_relation":null,"validated":true},{"source":"MONDO","code":"5342","qualifier":"E","icd_relation":null,"validated":true},{"source":"UMLS","code":"C0017661","qualifier":"E","icd_relation":null,"validated":true},{"source":"MedDRA","code":"10021263","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":[],"prevalence":[],"phenotypes":{"validation_status":"y","online":"y","validation_date":"2024-01-19","source":"34676611[PMID]_31371641[PMID]_30855802[PMID]_32400945[PMID]","terms":[{"hpo":"HP:0001919","label":"Acute kidney injury","frequency":"Frequent (79-30%)","criteria":null},{"hpo":"HP:0000794","label":"IgA deposition in the glomerulus","frequency":"Frequent (79-30%)","criteria":null},{"hpo":"HP:0012587","label":"Macroscopic hematuria","frequency":"Frequent (79-30%)","criteria":null},{"hpo":"HP:0002907","label":"Microscopic hematuria","frequency":"Frequent (79-30%)","criteria":null},{"hpo":"HP:0012595","label":"Mild proteinuria","frequency":"Frequent (79-30%)","criteria":null},{"hpo":"HP:0001541","label":"Ascites","frequency":"Occasional (29-5%)","criteria":null},{"hpo":"HP:0002608","label":"Celiac disease","frequency":"Occasional (29-5%)","criteria":null},{"hpo":"HP:0001394","label":"Cirrhosis","frequency":"Occasional (29-5%)","criteria":null},{"hpo":"HP:0030830","label":"Crackles","frequency":"Occasional (29-5%)","criteria":null},{"hpo":"HP:0000282","label":"Facial edema","frequency":"Occasional (29-5%)","criteria":null},{"hpo":"HP:0031504","label":"Foamy urine","frequency":"Occasional (29-5%)","criteria":null},{"hpo":"HP:0033316","label":"Glomerular crescent formation","frequency":"Occasional (29-5%)","criteria":null},{"hpo":"HP:0000099","label":"Glomerulonephritis","frequency":"Occasional (29-5%)","criteria":null},{"hpo":"HP:0000822","label":"Hypertension","frequency":"Occasional (29-5%)","criteria":null},{"hpo":"HP:0003261","label":"Increased circulating IgA level","frequency":"Occasional (29-5%)","criteria":null},{"hpo":"HP:0000083","label":"Renal insufficiency","frequency":"Occasional (29-5%)","criteria":null},{"hpo":"HP:0012593","label":"Nephrotic range proteinuria","frequency":"Very rare (<4-1%)","criteria":null}]}}