{"orpha":439762,"name":"Systemic polyarteritis nodosa","type":"Clinical subtype","group":"Subtype of disorder","status":"active","flags":[],"synonyms":["Systemic PAN","Systemic periarteritis nodosa"],"definition":"Systemic polyarteritis nodosa (PAN) is a chronic systemic necrotizingvasculitis of adults and childrenaffecting small- and medium-sized vessels and characterized by formation of microaneurysms leading to serious generalized disease and multi-organ involvement.","note":null,"url":"https://raremap.alftech.space/d/ORPHA-439762/","orphanet_url":"https://www.orpha.net/en/disease/detail/439762","names":{"fr":{"name":"Périartérite noueuse systémique","synonyms":["PAN systémique","Polyartérite noueuse systémique"],"definition":"Vascularite nécrosante systémique chronique de l'adulte et de l'enfant affectant les vaisseaux de petit et de moyen calibre, caractérisée par la formation de micro-anévrysmes entraînant une maladie généralisée grave et l'atteinte de plusieurs organes."},"de":{"name":"Systemische Polyarteriitis nodosa","synonyms":["Periarteriitis nodosa, systemische","Systemische PAN"],"definition":"Die systemische Polyarteriitis nodosa (PAN; siehe diesen Begriff) ist eine chronische systemische nekrotisierende Vaskulitis bei Erwachsenen und Kindern, die kleine und mittelgroße Gefäße betrifft und durch die Bildung von Mikroaneurysmen gekennzeichnet ist, die zu einer schweren generalisierten Erkrankung und einer Beteiligung mehrerer Organe führen."},"es":{"name":"Poliarteritis nodosa sistémica","synonyms":["PAN sistémica","Periarteritis nodosa sistémica"],"definition":"Es una vasculitis necrotizante sistémica crónica de adultos y niños que afecta a los vasos pequeños y medianos. Se caracteriza por la formación de microaneurismas que conducen a enfermedad generalizada grave con afectación multiorgánica."},"it":{"name":"Poliarterite nodosa sistemica","synonyms":["PAN sistemica","Periarterite nodosa sistemica"],"definition":"La poliarterite nodosa sistemica (PAN; si veda questo termine) è una vasculite necrotizzante sistemica cronica degli adulti e dei bambini, che colpisce i vasi di piccole e medie dimensioni. È caratterizzata dalla formazione di microaneurismi che esitano in una malattia grave generalizzata e nell'interessamento multiorgano."},"nl":{"name":"Systemische polyarteriitis nodosa","synonyms":["Systemische PAN","Systemische periarteriitis nodosa"],"definition":"Systemische polyarteriitis nodosa (PAN; zie deze term) is een chronische, systemische, necrotiserende vasculitis van volwassenen en kinderen die kleine en middelgrote bloedvaten treft en die gekarakteriseerd wordt door vorming van microaneurysmata, wat leidt tot ernstige gegeneraliseerde ziekte en betrokkenheid van meerdere organen."},"pt":{"name":"Poliarterite nodosa sistémica"},"pl":{"name":"Układowe guzkowe zapalenie tętnic","synonyms":["Układowe PAN"]},"cs":{"name":"Systémová polyarteritis nodosa","synonyms":["Systémová PAN","Systémová periarteritis nodosa"]},"uk":{"name":"Системний вузликовий поліартеріїт","synonyms":["Системний PAN","Системний вузликовий периартеріїт"],"definition":"Systemic polyarteritis nodosa (PAN; see this term) is a chronic systemic necrotizingvasculitis of adults and childrenaffecting small- and medium-sized vessels and characterized by formation of microaneurysms leading to serious generalized disease and multi-organ involvement."},"zh":{"name":"系统性结节性多动脉炎","synonyms":["系统性结节性血管周围炎"],"definition":"全身性结节性多发性动脉炎（PAN；见本术语）是一种成人和儿童的慢性全身性坏死性血管炎，会影响中小型血管，其特征是形成微动脉瘤，导致严重的全身性疾病和多器官受累。"},"tr":{"name":"Sistemik poliarteritis nodoza","synonyms":["Sistemik PAN","Sistemik periarterit nodosa"]}},"xrefs":[{"source":"ICD-10","code":"M30.0","qualifier":"NTBT","icd_relation":"attributed","validated":true},{"source":"ICD-11","code":"4A44.4","qualifier":"NTBT","icd_relation":"attributed","validated":true},{"source":"MONDO","code":"0018596","qualifier":"E","icd_relation":null,"validated":true},{"source":"UMLS","code":"C5848156","qualifier":"E","icd_relation":null,"validated":true}],"associations":[],"incoming_associations":[],"preferential_parent":{"orpha":98023,"name":"Rare systemic or rheumatologic disease"},"parents":[{"orpha":439737,"name":"Primary polyarteritis nodosa","classifications":[181,188,196,198,231,233]}],"children":[],"genes":[],"genes_via_subtypes":[],"inheritance":["Not applicable"],"onset":[],"prevalence":[],"phenotypes":null}