{"orpha":567544,"name":"Idiopathic non-lupus full-house nephropathy","type":"Clinical syndrome","group":"Disorder","status":"active","flags":[],"synonyms":["Idiopathic non-lupus FHN"],"definition":"A rare idiopathic glomerular clinical syndrome characterized by diffuse renal lesions that are indistinguishable from the lesions observed in systemic lupus erythematosus (SLE) in the absence of circulating autoantibodies and other systemic features necessary to meet the classification criteria for SLE. Patients may present with nephrotic syndrome, abnormal urinary sediment, acute renal insufficiency, progressive glomerulonephritis, and hypertension. Some patients have been reported to develop a progression to SLE over time.","note":null,"url":"https://raremap.alftech.space/d/ORPHA-567544/","orphanet_url":"https://www.orpha.net/en/disease/detail/567544","names":{"fr":{"name":"Néphropathie full-house non lupique idiopathique","synonyms":["FHN non lupique idiopathique","FHN séronégative idiopathique"],"definition":"Syndrome clinique glomérulaire idiopathique rare caractérisé par des lésions rénales diffuses que l'on ne peut distinguer des lésions observées dans le lupus érythémateux systémique (LES) en l'absence d'auto-anticorps circulants et d'autres caractéristiques systémiques nécessaires pour répondre aux critères de classification du LES. Les patients peuvent présenter un syndrome néphrotique, un sédiment urinaire anormal, une insuffisance rénale aiguë, une glomérulonéphrite progressive et une hypertension. Une évolution vers le LES au fil du temps a été rapportée chez certains patients."},"de":{"name":"Idiopathische non-Lupus Full-House-Nephropathie","synonyms":["Idiopathische non-Lupus FHN"],"definition":"Ein seltenes idiopathisches Glomerulonephritis-Syndrom, das durch diffuse Nierenläsionen gekennzeichnet ist, die nicht von denen des systemischen Lupus erythematodes (SLE) unterschieden werden können, ohne dass zirkulierende Autoantikörper oder andere systemische Merkmale vorhanden sind, die zur Erfüllung der Klassifikationskriterien für SLE erforderlich sind. Die Patienten können sich mit einem nephrotischen Syndrom, abnormen Urinsedimenten, akuter Niereninsuffizienz, progressiver Glomerulonephritis und arterieller Hypertonie vorstellen. Bei einigen Patienten wurde berichtet, dass sich im Laufe der Zeit ein SLE entwickelt."},"es":{"name":"Nefropatía “full house” no lúpica idiopática","synonyms":["FHN no lúpica idiopática"],"definition":"Es un síndrome clínico glomerular idiopático poco frecuente caracterizado por lesiones renales difusas indistinguibles de las lesiones observadas en el lupus eritematoso sistémico (LES) en ausencia de autoanticuerpos circulantes y otras características sistémicas necesarias para cumplir los criterios de clasificación del LES. Los pacientes pueden presentar síndrome nefrótico, sedimento urinario anómalo, insuficiencia renal aguda, glomerulonefritis progresiva e hipertensión. Se ha descrito que algunos pacientes desarrollan una progresión hacia LES con el tiempo."},"it":{"name":"Nefropatia non lupica idiopatica ''full house''","synonyms":["FHN non lupica idiopatica"]},"nl":{"name":"Idiopathische niet-lupus 'full house'-nefropathie","synonyms":["Idiopathische niet-lupus FHN"],"definition":"Een zeldzaam idiopathisch glomerulair klinisch syndroom, gekenmerkt door diffuse nierlaesies die niet te onderscheiden zijn van de laesies die worden waargenomen bij systemische lupus erythematosus (SLE) in afwezigheid van circulerende auto-antilichamen en andere systemische kenmerken nodig om te voldoen aan de classificatiecriteria voor SLE. Patiënten kunnen zich presenteren met nefrotisch syndroom, abnormaal sediment in urine, acute nierinsufficiëntie, progressieve glomerulonefritis, en hypertensie. Er werden enkele patiënten gerapporteerd die na verloop van tijd progressie naar SLE ontwikkelen."},"pt":{"name":"Nefropatia não-lúpica full-house idiopática","definition":"Síndrome clínica glomerular idiopática rara caracterizada por lesões renais difusas que são indistinguíveis das lesões observadas no lúpus eritematoso sistémico (LES) na ausência de autoanticorpos circulantes e outras características sistémicas necessárias para corresponder aos critérios de classificação do LES. Os doentes podem apresentar síndrome nefrótica, sedimento urinário anómalo, insuficiência renal aguda, glomerulonefrite progressiva e hipertensão. Foi descrito que alguns doentes desenvolvem uma progressão para LES ao longo do tempo."},"pl":{"name":"Idiopatyczna nefropatia pełnoobjawowa nie związana z toczniem","synonyms":["Idiopathic non-lupus FHN"],"definition":"Rzadki idiopatyczny zespół kłębuszkowy, charakteryzujący się rozsianymi zmianami w nerkach, które są nie do odróżnienia od zmian obserwowanych w toczniu rumieniowatym układowym (SLE), przy braku krążących autoprzeciwciał i innych ogólnoustrojowych cech niezbędnych do spełnienia kryteriów klasyfikacji SLE. U pacjentów może wystąpić zespół nerczycowy, nieprawidłowy osad moczu, ostra niewydolność nerek, postępujące kłębuszkowe zapalenie nerek i nadciśnienie. U niektórych pacjentów choroba postępuje z czasem do SLE."},"cs":{"name":"Idiopatická nelupusová full-house nefropatie","synonyms":["Idiopathic non-lupus FHN"]},"uk":{"name":"Ідіопатична не вовчакова повна нефропатія","synonyms":["Ідіопатичний не вовчаковий FHN"],"definition":"A rare idiopathic glomerular clinical syndrome characterized by diffuse renal lesions that are indistinguishable from the lesions observed in systemic lupus erythematosus (SLE) in the absence of circulating autoantibodies and other systemic features necessary to meet the classification criteria for SLE. Patients may present with nephrotic syndrome, abnormal urinary sediment, acute renal insufficiency, progressive glomerulonephritis, and hypertension. Some patients have been reported to develop a progression to SLE over time."},"zh":{"name":"特发性非狼疮性满堂亮肾病","synonyms":["Idiopathic non-lupus FHN"]},"tr":{"name":"İdiyopatik lupus dışı full-house nefropati","synonyms":["İdiyopatik lupus olmayan FHN"]}},"xrefs":[{"source":"ICD-10","code":"N05","qualifier":"NTBT","icd_relation":"attributed","validated":true},{"source":"ICD-11","code":"GB4Y","qualifier":"NTBT","icd_relation":"attributed","validated":true},{"source":"MONDO","code":"0035763","qualifier":"E","icd_relation":null,"validated":true},{"source":"UMLS","code":"C5680132","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":["Not applicable"],"onset":["All ages"],"prevalence":[{"type":"Point prevalence","qualification":"Class only","class":"Unknown","value":0.0,"area":"Worldwide","status":"Validated","source":"ORPHANET"}],"phenotypes":{"validation_status":"y","online":"y","validation_date":"2021-06-16","source":"28340077[PMID]_27270291[PMID]","terms":[{"hpo":"HP:0000093","label":"Proteinuria","frequency":"Very frequent (99-80%)","criteria":null},{"hpo":"HP:0004431","label":"Complement deficiency","frequency":"Frequent (79-30%)","criteria":null},{"hpo":"HP:0005421","label":"Decreased circulating complement C3 concentration","frequency":"Frequent (79-30%)","criteria":null},{"hpo":"HP:0045042","label":"Decreased circulating complement C4 concentration","frequency":"Frequent (79-30%)","criteria":null},{"hpo":"HP:0000822","label":"Hypertension","frequency":"Frequent (79-30%)","criteria":null},{"hpo":"HP:0000100","label":"Nephrotic syndrome","frequency":"Frequent (79-30%)","criteria":null},{"hpo":"HP:0000083","label":"Renal insufficiency","frequency":"Frequent (79-30%)","criteria":null},{"hpo":"HP:0001919","label":"Acute kidney injury","frequency":"Occasional (29-5%)","criteria":null},{"hpo":"HP:0001369","label":"Arthritis","frequency":"Occasional (29-5%)","criteria":null},{"hpo":"HP:0003259","label":"Elevated circulating creatinine concentration","frequency":"Occasional (29-5%)","criteria":null},{"hpo":"HP:0012576","label":"Glomerular C3 deposition","frequency":"Occasional (29-5%)","criteria":null},{"hpo":"HP:0000099","label":"Glomerulonephritis","frequency":"Occasional (29-5%)","criteria":null},{"hpo":"HP:0001966","label":"Mesangial abnormality","frequency":"Occasional (29-5%)","criteria":null},{"hpo":"HP:0002907","label":"Microscopic hematuria","frequency":"Occasional (29-5%)","criteria":null},{"hpo":"HP:0000155","label":"Oral ulcer","frequency":"Occasional (29-5%)","criteria":null},{"hpo":"HP:0045073","label":"Serositis","frequency":"Occasional (29-5%)","criteria":null},{"hpo":"HP:0000988","label":"Skin rash","frequency":"Occasional (29-5%)","criteria":null},{"hpo":"HP:0100769","label":"Synovitis","frequency":"Occasional (29-5%)","criteria":null},{"hpo":"HP:0002715","label":"Abnormality of the immune system","frequency":"Very rare (<4-1%)","criteria":null},{"hpo":"HP:0000707","label":"Abnormality of the nervous system","frequency":"Very rare (<4-1%)","criteria":null},{"hpo":"HP:0020151","label":"Anti-dsDNA antibody positivity","frequency":"Excluded (0%)","criteria":null},{"hpo":"HP:0002960","label":"Autoimmunity","frequency":"Excluded (0%)","criteria":null}]}}