{"orpha":52759,"name":"Vasculitis","type":"Category","group":"Group of disorders","status":"active","flags":[],"synonyms":["Systemic vasculitis"],"definition":"Vasculitis represents a clinically heterogenous group of diseases of multifactorial etiology characterized by inflammation of either large-sized vessels (large-vessel vasculitis, e.g. Giant-cell arteritis and Takayasu arteritis), medium-sized vessels (medium-vessel vasculitis e.g. polyarteritis nodosa and Kawasaki disease), or small-sized vessels (small-vessel vasculitis, e.g. granulomatosis with polyangiitis, microscopic polyangiitis, immunoglobulin A vasculitis, and cutaneous leukocytoclastic angiitis). Vasculitis occurs at any age, may be acute or chronic, and manifests with general symptoms such as fever, weight loss and fatigue, as well as more specific clinical signs depending on the type of vessels and organs affected. The degree of severity is variable, ranging from life or sight threatening disease (e.g. Behçet disease) to relatively minor skin disease.","note":null,"url":"https://raremap.alftech.space/d/ORPHA-52759/","orphanet_url":"https://www.orpha.net/en/disease/detail/52759","names":{"fr":{"name":"Vascularite","synonyms":["Vascularite systémique"],"definition":"Groupe cliniquement hétérogène de maladies d'étiologie multifactorielle, caractérisées par l'inflammation des gros vaisseaux (vascularite des vaisseaux de gros calibre, telle que l'artérite à cellules géantes et l'artérite de Takayasu), des vaisseaux de moyen calibre (vascularite des vaisseaux de moyen calibre, telle que la périartérite noueuse et la maladie de Kawasaki) ou des petits vaisseaux (vascularite des vaisseaux de petit calibre, comme la granulomatose avec polyangéite, la polyangéite microscopique, la vascularite à immunoglobulines&nbsp;A et l'angéite cutanée leucocytoclasique). La vascularite survient à tous les âges, elle peut être aigüe ou chronique, et se manifeste par des symptômes généraux tels qu'une fièvre, une perte de poids et une fatigue, ainsi que par des symptômes plus spécifiques, selon le type de vaisseaux et l'organe atteints. Le degré de sévérité de la maladie est variable, allant d'une forme résultant en une déficience visuelle ou potentiellement létale (maladie de Behçet), à une forme cutanée relativement mineure."},"de":{"name":"Vaskulitis","synonyms":["Vaskulitis, systemische"],"definition":"Die Vaskulitis umfasst eine klinisch heterogene Gruppe von Krankheiten mit multifaktorieller Ätiologie. Sie ist gekennzeichnet durch Entzündung der großen Gefäße (<i>large-vessel vasculitis</i>, z.B. Riesenzell-Arteriitis und Takayasu-Arteriitis; s. diese Termini), der mittelgroßen Gefäße (<i>medium-vessel vasculitis</i>, z.B. Polyarteriitis nodosa und Kawasaki-Krankheit; s. diese Termini) oder der kleinen Gefäße (<i>small-vessel vasculitis</i>, z.B. Wegener- Granulomatose, Mikroskopische Polyangiitis, Immunoglobulin A-Vaskulitis und Kutane leukozytoklastische Angiitis; s. diese Termini). Die Krankheit kann in jedem Alter und akut oder chronisch auftreten. Sie äußert sich mit Allgemeinsymptomen wie Fieber, Gewichtsverlust und Mattigkeit und, abhängig von Gefäßtyp und betroffenen Organen, mit spezifischeren Symptomen. Der Schweregrad ist unterschiedlich und reicht von lebensbedrohlich und Visusverlust (z.B. Behçet-Krankheit; s. dort) bis zu relativ gutartiger Hautkrankheit."},"es":{"name":"Vasculitis","definition":"El término vasculitis representa un grupo clínicamente heterogéneo de enfermedades de etiología multifactorial caracterizadas por la inflamación de vasos de gran tamaño (vasculitis macrovascular, p. ej. arteritis de células gigantes y arteritis de Takayasu; ver estos términos), vasos de tamaño medio (vasculitis de vasos medios, p. ej. periarteritis nodosa y enfermedad de Kawasaki; ver estos términos), o vasos pequeños (vasculitis microvascular, p. ej. granulomatosis de Wegener, poliangeítis microscópica, vasculitis por inmunoglobulina A, y vasculitis cutánea; ver estos términos). La vasculitis se presenta a cualquier edad, puede ser aguda o crónica y se manifiesta con síntomas generales como fiebre, pérdida de peso y cansancio, así como con síntomas clínicos más específicos en función del tipo de vasos y órganos afectados. El grado de gravedad es variable, y va desde enfermedades que amenazan la visión o potencialmente mortales (p. ej. la enfermedad de Behçet) a enfermedades cutáneas relativamente leves."},"it":{"name":"Vasculite","definition":"Le vasculiti sono un gruppo eterogeneo di malattie multifattoriali, caratterizzate dall'infiammazione dei grandi vasi (vasculite dei grandi vasi, come l'arterite a cellule giganti e l'arterite di Takayasu; si vedano questi termini), dei vasi medi (vasculite dei vasi medi, come la poliarterite nodosa e la malattia di Kawasaki; si vedano questi termini), o dei piccoli vasi (vasculite dei piccoli vasi, come la granulomatosi di Wegener, la poliangite microscopica, la vasculite associata alle immunoglobuline A, e l'angite leucocitoclastica cutanea; si vedano questi termini). La vasculite esordisce a tutte le età, può essere acuta o cronica, e si manifesta con sintomi generici come la febbre, la perdita di peso e l'affaticamento, ma anche con segni clinici specifici che dipendono dal tipo dei vasi e degli organi interessati. La gravità è variabile: dalla malattia potenzialmente letale o pericolosa per la vista (malattia di Behçet; si veda questo termine), alla malattia cutanea relativamente lieve."},"nl":{"name":"Vasculitis","synonyms":["Bloedvatontsteking","Systemische vasculitis"],"definition":"Vasculitis vertegenwoordigt een klinisch heterogene groep van ziekten met een multifactoriële etiologie die worden gekarakteriseerd door ontsteking van grote bloedvaten (vasculitis van de grote bloedvaten, e.g. reuzencel-arteritis en Takayasu arteritis; zie deze termen), middelgrote bloedvaten (vasculitis van de middelgrote bloedvaten, e.g. polyarteritis nodosa en ziekte van Kawasaki; zie deze termen), of kleine bloedvaten (vasculitis van de kleine bloedvaten, e.g. Wegener granulomatose, microscopische polyangiitis, immunoglobuline A-vasculitis, en cutane leukocytoclastische angiitis; zie deze termen). Vasculitis komt voor op elke leeftijd, kan acuut of chronisch zijn, en manifesteert zich met algemene symptomen zoals koorts, gewichtsverlies en moeheid, alsook meer specifieke klinische kenmerken afhankelijk van het type bloedvaten en de organen die getroffen worden. De ernst kan variëren, gaande van zicht- of levensbedreigende ziekte (ziekte van Behçet; zie deze term) tot relatief geringe huidziekte."},"pt":{"name":"Vasculite"},"pl":{"name":"Zapalenie naczyń","synonyms":["Systemowe zapalenie naczyń"]},"cs":{"name":"Vaskulitida","synonyms":["Systémová vaskulitida"]},"uk":{"name":"Васкуліт","synonyms":["Системний васкуліт"],"definition":"Vasculitis represents a clinically heterogenous group of diseases of multifactorial etiology characterized by inflammation of either large-sized vessels (large-vessel vasculitis, e.g. Giant-cell arteritis and Takayasu arteritis; see these terms), medium-sized vessels (medium-vessel vasculitis e.g. polyarteritis nodosa and Kawasaki disease; see these terms), or small-sized vessels (small-vessel vasculitis, e.g. granulomatosis with polyangiitis, microscopic polyangiitis, immunoglobulin A vasculitis, and cutaneous leukocytoclastic angiitis; see these terms). Vasculitis occurs at any age, may be acute or chronic, and manifests with general symptoms such as fever, weight loss and fatigue, as well as more specific clinical signs depending on the type of vessels and organs affected. The degree of severity is variable, ranging from life or sight threatening disease (e.g. Behçet disease, see this term) to relatively minor skin disease."},"zh":{"name":"血管炎","synonyms":["系统性血管炎"]},"tr":{"name":"Vaskülit","synonyms":["Sistemik vaskülit"]}},"xrefs":[{"source":"ICD-11","code":"4A44","qualifier":"E","icd_relation":"specific","validated":true,"icd11_uri":"572581721"},{"source":"MONDO","code":"0018882","qualifier":"E","icd_relation":null,"validated":true},{"source":"MONDO","code":"18882","qualifier":"E","icd_relation":null,"validated":true},{"source":"MeSH","code":"D014657","qualifier":"E","icd_relation":null,"validated":true},{"source":"UMLS","code":"C0042384","qualifier":"E","icd_relation":null,"validated":true},{"source":"MedDRA","code":"10047115","qualifier":"E","icd_relation":null,"validated":true}],"associations":[],"incoming_associations":[],"preferential_parent":{"orpha":98023,"name":"Rare systemic or rheumatologic disease"},"parents":[{"orpha":98023,"name":"Rare systemic or rheumatologic disease","classifications":[196]},{"orpha":68362,"name":"Rare vascular disease","classifications":[198]}],"children":[{"orpha":156140,"name":"Predominantly large-vessel vasculitis","type":"Clinical group"},{"orpha":156143,"name":"Predominantly medium-vessel vasculitis","type":"Clinical group"},{"orpha":156146,"name":"Predominantly small-vessel vasculitis","type":"Clinical group"},{"orpha":445197,"name":"Secondary vasculitis","type":"Category"},{"orpha":251328,"name":"Unclassified vasculitis","type":"Disease"}],"genes":[],"genes_via_subtypes":[],"inheritance":[],"onset":[],"prevalence":[{"type":"Point prevalence","qualification":"Value and class","class":"1-9 / 100 000","value":6.3,"area":"Europe","status":"Not yet validated","source":"ORPHANET"}],"phenotypes":null}