{"orpha":137617,"name":"Nephrogenic systemic fibrosis","type":"Disease","group":"Disorder","status":"active","flags":[],"synonyms":["Nephrogenic fibrosing dermopathy"],"definition":"Nephrogenic systemic fibrosis (NSF) is a rare systemic fibrosing condition observed in renally impaired patients and characterized by a hardening and thickening of the skin with fibrotic plaques or papules, pruritus, joint pain and stiffness, muscle weakness, limitation of range of motion, and yellowed eyes. It is generally associated with administration of gadolinium-based magnetic resonance imaging contrast agents (GBCA) in patients with kidney disease.","note":null,"url":"https://raremap.alftech.space/d/ORPHA-137617/","orphanet_url":"https://www.orpha.net/en/disease/detail/137617","names":{"fr":{"name":"Fibrose systémique fibrogénique","synonyms":["Dermopathie fibrosante néphrogénique"],"definition":"La fibrose systémique fibrogénique (FSN) est une maladie fibrosante systémique rare observée chez les patients insuffisants rénaux et caractérisée par un durcissement et un épaississement de la peau accompagnés de plaques ou de papules fibreuses, un prurit, une douleur et une raideur articulaires, une faiblesse musculaire, une diminution de l'amplitude des mouvements et des yeux jaunis. Elle est généralement associée à l'administration de produits de contraste gadolinés (PCG) en imagerie à résonance magnétique chez des patients atteints d'insuffisance rénale."},"de":{"name":"Nephrogene systemische Fibrose","synonyms":["NFS","Nephrogene fibrosierende Dermopathie"],"definition":"Die Nephrogene systemische Fibrose (NSF) ist eine seltene, systemisch fibrosierende Störung bei nierengeschädigten Patienten und gekennzeichnet durch Verhärtung und Verdickung der Haut mit fibrotischen Plaques oder Papeln, Pruritus, Gelenkschmerzen und -versteifung, Muskelschwäche, eingeschränktem Bewegungsumfang und gelb verfärbte Augen. In der Regel handelt es sich um nierenkranke Patienten, die gadoliniumhaltige Kontrastmittel für die Magnetresonanz-Bildgebung erhielten."},"es":{"name":"Fibrosis sistémica nefrogénica","synonyms":["Dermopatía fibrosante nefrogénica"],"definition":"La fibrosis sistémica nefrogénica (NSF) es una enfermedad fibrosante sistémica poco frecuente observada en pacientes con insuficiencia renal y que se caracteriza por un endurecimiento y engrosamiento de la piel con placas o pápulas fibróticas, prurito, dolor en las articulaciones y rigidez, debilidad muscular, limitación del rango de movimiento, y ojos amarillentos. Está asociada generalmente con la administración de agentes de contraste basados en gadolinio utilizados en la resonancia magnética (GBCA) en pacientes con enfermedades renales."},"it":{"name":"Fibrosi sistemica nefrogenica","synonyms":["Dermopatia fibrosante nefrogenica"],"definition":"La fibrosi sistemica nefrogena (NSF) è una malattia sistemica fibrosante rara descritta nei pazienti con insufficienza renale: è caratterizzata dall'indurimento e dall'ispessimento della cute con placche o papule fibrotiche, prurito, rigidità e dolore articolare, debolezza muscolare, limitazione dei movimenti e occhi ingialliti. Si associa, in genere, alla somministrazione di agenti di contrasto impiegati nella risonanza magnetica, a base di gadolinio, in soggetti affetti da malattia renale."},"nl":{"name":"Nefrogene systemische fibrose","synonyms":["Nefrogene fibroserende dermopathie"],"definition":"Een zeldzame systemische fibroserende aandoening, waargenomen bij patiënten met nierfunctiestoornis, en gekenmerkt door verharding en verdikking van huid met fibrotische plaques of papels, pruritus, pijn en stijfheid van gewrichten, spierzwakte, beperking van bewegingsbereik, en gelige ogen. Het is doorgaans geassocieerd met toediening van contrastmiddelen voor beeldvorming met magnetische resonantie gebaseerd op gadolinium (GBCA) bij patiënten met nierziekte."},"pt":{"name":"Dermopatia fibrosante nefrogénica"},"pl":{"name":"Nefrogenne włóknienie układowe","synonyms":["Nefrogenna dermopatia włóknista"]},"cs":{"name":"Nefrogenní systémová fibróza","synonyms":["Nefrogenní fibrotizující dermopatie","NSF","NFD"]},"uk":{"name":"Нефрогенний системний фіброз","synonyms":["Нефрогенна фіброзуюча дермопатія"],"definition":"Nephrogenic systemic fibrosis (NSF) is a rare systemic fibrosing condition observed in renally impaired patients and characterized by a hardening and thickening of the skin with fibrotic plaques or papules, pruritus, joint pain and stiffness, muscle weakness, limitation of range of motion, and yellowed eyes. It is generally associated with administration of gadolinium-based magnetic resonance imaging contrast agents (GBCA) in patients with kidney disease."},"zh":{"name":"肾源性系统性纤维化","synonyms":["肾源性纤维性皮肤病"],"definition":"肾源性系统性纤维化(NSF)是一种罕见的系统性纤维化疾病，见于肾脏受损患者，其特征为皮肤硬化增厚并伴有纤维化斑块或丘疹，瘙痒，关节疼痛和僵硬，肌肉无力，活动范围受限，眼睛发黄。它通常与肾脏病患者使用钆磁共振造影剂(GBCA)有关。"},"tr":{"name":"Nefrojenik sistemik fibroz","synonyms":["Nefrojenik fibrozan dermopati"]}},"xrefs":[{"source":"ICD-10","code":"M72.8","qualifier":"NTBT","icd_relation":"attributed","validated":true},{"source":"ICD-11","code":"FB51.Y","qualifier":"NTBT","icd_relation":"index","validated":true,"icd11_uri":"1537082562"},{"source":"MONDO","code":"0015294","qualifier":"E","icd_relation":null,"validated":true},{"source":"UMLS","code":"C3888044","qualifier":"E","icd_relation":null,"validated":true},{"source":"GARD","code":"9725","qualifier":"E","icd_relation":null,"validated":true},{"source":"MedDRA","code":"10067467","qualifier":"E","icd_relation":null,"validated":true}],"associations":[],"incoming_associations":[],"preferential_parent":{"orpha":89826,"name":"Rare skin disease"},"parents":[{"orpha":90077,"name":"Other acquired skin disease","classifications":[187]}],"children":[],"genes":[],"genes_via_subtypes":[],"inheritance":[],"onset":[],"prevalence":[],"phenotypes":null}