{"orpha":439755,"name":"Single-organ polyarteritis nodosa","type":"Clinical subtype","group":"Subtype of disorder","status":"active","flags":[],"synonyms":["Single-organ PAN","Single-organ periarteritis nodosa"],"definition":"Single-organ polyarteritis nodosa (PAN) is a rare, often mild form of PAN characterized by limited disease without generalized manifestations, most often affecting the skin (cutaneous PAN), the brain, eyes, pancreas, testicles, ureter, breasts, or ovaries. Affected patients are often younger than those with systemic PAN and relapses appear to be more common.","note":null,"url":"https://raremap.alftech.space/d/ORPHA-439755/","orphanet_url":"https://www.orpha.net/en/disease/detail/439755","names":{"fr":{"name":"Périartérite noueuse à organe unique","synonyms":["PAN à organe unique","Polyartérite noueuse à organe unique"],"definition":"Forme rare et souvent légère de périartérite noueuse caractérisée par une atteinte limitée sans manifestations généralisées, affectant le plus souvent la peau (périartérite noueuse cutanée), le cerveau, les yeux, le pancréas, les testicules, l'uretère, les seins ou les ovaires. Les patients atteints sont souvent plus jeunes que ceux atteints de périartérite noueuse systémique et les rechutes semblent plus fréquentes."},"de":{"name":"Isolierte Organvaskulitis vom Typ der Polyarteriitis nodosa","synonyms":["Einzelorgan-PAN","Periarteriitis nodosa, isoliertes Organ"]},"es":{"name":"Poliarteritis nodosa de órgano único","synonyms":["PAN de órgano único","Periarteritis nodosa de órgano único"],"definition":"Es una forma poco frecuente, con frecuencia leve, de PAN que se caracteriza por una enfermedad limitada sin manifestaciones generalizadas que afecta con mayor frecuencia a la piel (PAN cutánea), cerebro, ojos, páncreas, testículos, uréter, mamas u ovarios. Los afectados suelen ser más jóvenes que aquellos con PAN sistémico y las recaídas parecen ser más frecuentes."},"it":{"name":"Poliarterite nodosa di un solo organo","synonyms":["PAN di un solo organo","Periarterite nodosa di un solo organo"],"definition":"La poliarterite nodosa (PAN) di un solo organo è una forma rara, e spesso lieve, di PAN. È caratterizzata dalla malattia limitata in assenza di segni clinici generalizzati; interessa per lo più la cute (PAN cutanea; si veda questo termine), il cervello, gli occhi, il pancreas, i testicoli, l'uretere, le mammelle o le ovaie. Spesso i pazienti sono più giovani rispetto a quelli colpiti da PAN sistemica (si veda questo termine) e le recidive sono più comuni."},"nl":{"name":"Polyarteriitis nodosa van een enkel orgaan","synonyms":["PAN van een enkel orgaan","Periarteriitis nodosa van een enkel orgaan"],"definition":"Polyarteriitis nodosa (PAN) van een enkel orgaan is een zeldzame, vaak milde vorm van PAN die gekarakteriseerd wordt door beperkte ziekte zonder gegeneraliseerde manifestaties, meestal met aantasting van de huid (cutane PAN; zie deze term), hersenen, ogen, pancreas, testikels, ureter, borsten, of eierstokken. Getroffen patiënten zijn vaak jonger dan diegenen met systemische PAN (zie deze term) en terugvallen lijken vaker voor te komen."},"pt":{"name":"Poliarterite nodosa, orgão - único"},"pl":{"name":"Guzkowe zapalenie tętnic jednego narządu","synonyms":["PAN w obrębie jednego narządu"]},"cs":{"name":"Jednoorgánová polyarteritis nodosa","synonyms":["Jednoorgánová PAN","Jednoorgánová periarteritis nodosa"]},"uk":{"name":"Одноорганний вузликовий поліартеріїт","synonyms":["Одноорганний PAN","Одноорганний вузликовий періартеріїт"],"definition":"Single-organ polyarteritis nodosa (PAN) is a rare, often mild form of PAN characterized by limited disease without generalized manifestations, most often affecting the skin (cutaneous PAN; see this term), the brain, eyes, pancreas, testicles, ureter, breasts, or ovaries. Affected patients are often younger than those with systemic PAN (see this term) and relapses appear to be more common."},"zh":{"name":"单器官结节性多动脉炎","synonyms":["单器官结节性动脉周围炎"],"definition":"单器官结节性多发性动脉炎（PAN）是一种罕见的，通常为轻度形式的PAN，其特征是病情较局限，无全身性临床表现，最常影响皮肤（皮肤PAN；请参见此术语），大脑，眼睛，胰腺，睾丸，输尿管，乳房或卵巢。患病的患者通常比全身性PAN的患者年轻（见此术语），复发也更为常见。"},"tr":{"name":"Tek organlı poliarteritis nodoza","synonyms":["Tek organlı PAN","Tek organlı 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":"0018595","qualifier":"E","icd_relation":null,"validated":true},{"source":"UMLS","code":"C5680051","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}