{"orpha":767,"name":"Polyarteritis nodosa","type":"Disease","group":"Disorder","status":"active","flags":[],"synonyms":["Küssmaul-Maier disease","PAN","Periarteritis nodosa"],"definition":"A rare, clinically heterogeneous, systemic disease characterized by necrotizing inflammatory lesions affecting medium-sized blood vessels. It most commonly affects skin, joints, peripheral nerves and the gastrointestinal tract.","note":null,"url":"https://raremap.alftech.space/d/ORPHA-767/","orphanet_url":"https://www.orpha.net/en/disease/detail/767","names":{"fr":{"name":"Périartérite noueuse","synonyms":["Maladie de Küssmaul-Maier","PAN","Panartérite noueuse","Polyartérite noueuse"],"definition":"Maladie systémique rare à expression clinique hétérogène, caractérisée par des lésions inflammatoires nécrosantes affectant des vaisseaux sanguins de calibre moyen. La peau, les articulations, les nerfs périphériques et le tractus gastro-intestinal sont les cibles privilégiées."},"de":{"name":"Polyarteriitis nodosa","synonyms":["Küssmaul-Maier-Krankheit","PAN"],"definition":"Eine seltene systemische Erkrankung mit heterogenem klinischen Bild, die durch nekrotisierende entzündliche Läsionen der mittelgroßen Blutgefäße gekennzeichnet ist. Am häufigsten sind Haut, Gelenke, periphere Nerven und der Gastrointestinaltrakt betroffen."},"es":{"name":"Poliarteritis nodosa","synonyms":["Enfermedad de Küssmaul-Maier","PAN","Periarteritis nodosa"],"definition":"Es una enfermedad sistémica poco frecuente, clínicamente heterogénea, caracterizada por lesiones inflamatorias necrotizantes que afectan a vasos sanguíneos medianos, principalmente de la piel, las articulaciones, los nervios periféricos y el tracto gastrointestinal."},"it":{"name":"Poliarterite nodosa","synonyms":["Malattia di Küssmaul-Maier","PAN","Periartrite nodosa"],"definition":"La poliarterite nodosa (PAN) è una malattia sistemica rara, clinicamente eterogenea, caratterizzata da lesioni infiammatorie necrotizzanti che interessano i vasi sanguigni di medie dimensioni. Di solito la malattia colpisce la cute, le articolazioni, i nervi periferici e il tratto gastrointestinale."},"nl":{"name":"Polyarteriitis nodosa","synonyms":["PAN","Periarteriitis nodosa","Ziekte van Küssmaul-Maier"],"definition":"Een zeldzame, klinisch heterogene, systemische aandoening, gekarakteriseerd door necrotiserende inflammatoire laesies die middelgrote bloedvaten aantasten. De aandoening treft doorgaans huid, gewrichten, perifere zenuwen, en maag-darmstelsel."},"pt":{"name":"Poliarterite nodosa"},"pl":{"name":"Guzkowe zapalenie tętnic","synonyms":["Choroba Küssmaula i Maiera","PAN"]},"cs":{"name":"Polyarteritis nodosa","synonyms":["Küssmaulova-Maierova nemoc","PAN","Periarteritis nodosa","Küssmaul-Maierova nemoc"]},"uk":{"name":"Вузликовий поліартеріїт","synonyms":["Хвороба Кюссмауля-Майєра","PAN","Вузликовий периартеріїт"],"definition":"A rare, clinically heterogeneous, systemic disease characterized by necrotizing inflammatory lesions affecting medium-sized blood vessels. It most commonly affects skin, joints, peripheral nerves and the gastrointestinal tract."},"zh":{"name":"结节性多动脉炎","synonyms":["Küssmaul-Maier 综合征"]},"tr":{"name":"Poliarteritis nodosa","synonyms":["TAVA","Periarteritis nodoza","Küssmaul-Maier hastalığı"]}},"xrefs":[{"source":"ICD-10","code":"M30.0","qualifier":"E","icd_relation":"specific","validated":true},{"source":"ICD-11","code":"4A44.4","qualifier":"E","icd_relation":"specific","validated":true,"icd11_uri":"1419332129"},{"source":"MONDO","code":"0019170","qualifier":"E","icd_relation":null,"validated":true},{"source":"MeSH","code":"D010488","qualifier":"E","icd_relation":null,"validated":true},{"source":"UMLS","code":"C0031036","qualifier":"E","icd_relation":null,"validated":true},{"source":"GARD","code":"7360","qualifier":"E","icd_relation":null,"validated":true},{"source":"MedDRA","code":"10036024","qualifier":"E","icd_relation":null,"validated":true}],"associations":[],"incoming_associations":[{"type":"Referred to","orpha":93564,"name":"OBSOLETE: Pediatric polyarteritis nodosa","status":"inactive"}],"preferential_parent":{"orpha":98023,"name":"Rare systemic or rheumatologic disease"},"parents":[{"orpha":156143,"name":"Predominantly medium-vessel vasculitis","classifications":[196,198]},{"orpha":280369,"name":"Rare pediatric vasculitis","classifications":[231]},{"orpha":209007,"name":"Systemic inflammatory disease associated with an acquired peripheral neuropathy","classifications":[181]},{"orpha":567560,"name":"Systemic vasculitis associated with glomerulopathy","classifications":[188,233]}],"children":[{"orpha":439737,"name":"Primary polyarteritis nodosa","type":"Clinical subtype"},{"orpha":439746,"name":"Secondary polyarteritis nodosa","type":"Clinical subtype"}],"genes":[],"genes_via_subtypes":[],"inheritance":["Not applicable"],"onset":["All ages"],"prevalence":[{"type":"Point prevalence","qualification":"Value and class","class":"1-9 / 100 000","value":3.16,"area":"Europe","status":"Validated","source":"9805179[PMID]_14872461[PMID]_17553910[PMID]_ORPHANET"},{"type":"Point prevalence","qualification":"Value and class","class":"1-9 / 100 000","value":3.1,"area":"Sweden","status":"Validated","source":"17553910[PMID]"},{"type":"Annual incidence","qualification":"Value and class","class":"1-9 / 1 000 000","value":0.9,"area":"United Kingdom","status":"Not yet validated","source":"10693883[PMID]_11156552[PMID]"},{"type":"Point prevalence","qualification":"Value and class","class":"1-9 / 100 000","value":3.07,"area":"France","status":"Validated","source":"14872461[PMID]"},{"type":"Annual incidence","qualification":"Value and class","class":"1-9 / 1 000 000","value":0.11,"area":"Australia","status":"Validated","source":"18771432[PMID]"},{"type":"Annual incidence","qualification":"Value and class","class":"1-9 / 1 000 000","value":0.62,"area":"Spain","status":"Validated","source":"11156552[PMID]"},{"type":"Point prevalence","qualification":"Value and class","class":"1-9 / 100 000","value":3.3,"area":"Norway","status":"Validated","source":"9805179[PMID]"},{"type":"Annual incidence","qualification":"Value and class","class":"<1 / 1 000 000","value":0.05,"area":"Norway","status":"Not yet validated","source":"ORPHANET"},{"type":"Annual incidence","qualification":"Value and class","class":"<1 / 1 000 000","value":0.04,"area":"Germany","status":"Validated","source":"15696553[PMID]"}],"phenotypes":{"validation_status":"y","online":"y","validation_date":"2019-05-15","source":"29955757[PMID]_30114520[PMID]_30418125[PMID]_30952413[PMID]_26876385[PMID]","terms":[{"hpo":"HP:0011121","label":"Abnormal skin morphology","frequency":"Very frequent (99-80%)","criteria":null},{"hpo":"HP:0000077","label":"Abnormality of the kidney","frequency":"Frequent (79-30%)","criteria":null},{"hpo":"HP:0002829","label":"Arthralgia","frequency":"Frequent (79-30%)","criteria":null},{"hpo":"HP:0011227","label":"Elevated circulating C-reactive protein concentration","frequency":"Frequent (79-30%)","criteria":null},{"hpo":"HP:0001945","label":"Fever","frequency":"Frequent (79-30%)","criteria":null},{"hpo":"HP:0033260","label":"Livedo racemosa","frequency":"Frequent (79-30%)","criteria":null},{"hpo":"HP:0003326","label":"Myalgia","frequency":"Frequent (79-30%)","criteria":null},{"hpo":"HP:0009830","label":"Peripheral neuropathy","frequency":"Frequent (79-30%)","criteria":null},{"hpo":"HP:0005764","label":"Polyarticular arthritis","frequency":"Frequent (79-30%)","criteria":null},{"hpo":"HP:0031003","label":"Polyneuritis","frequency":"Frequent (79-30%)","criteria":null},{"hpo":"HP:0001824","label":"Weight loss","frequency":"Frequent (79-30%)","criteria":null},{"hpo":"HP:0002027","label":"Abdominal pain","frequency":"Occasional (29-5%)","criteria":null},{"hpo":"HP:0030680","label":"Abnormal cardiovascular system morphology","frequency":"Occasional (29-5%)","criteria":null},{"hpo":"HP:0002088","label":"Abnormal lung morphology","frequency":"Occasional (29-5%)","criteria":null},{"hpo":"HP:0011024","label":"Abnormality of the gastrointestinal tract","frequency":"Occasional (29-5%)","criteria":null},{"hpo":"HP:0000707","label":"Abnormality of the nervous system","frequency":"Occasional (29-5%)","criteria":null},{"hpo":"HP:0000965","label":"Cutis marmorata","frequency":"Occasional (29-5%)","criteria":null},{"hpo":"HP:0010783","label":"Erythema","frequency":"Occasional (29-5%)","criteria":null},{"hpo":"HP:0000822","label":"Hypertension","frequency":"Occasional (29-5%)","criteria":null},{"hpo":"HP:0002011","label":"Morphological central nervous system abnormality","frequency":"Occasional (29-5%)","criteria":null},{"hpo":"HP:0001701","label":"Pericarditis","frequency":"Occasional (29-5%)","criteria":null},{"hpo":"HP:0030880","label":"Raynaud phenomenon","frequency":"Occasional (29-5%)","criteria":null},{"hpo":"HP:0003390","label":"Sensory axonal neuropathy","frequency":"Occasional (29-5%)","criteria":null},{"hpo":"HP:0200042","label":"Skin ulcer","frequency":"Occasional (29-5%)","criteria":null},{"hpo":"HP:0001482","label":"Subcutaneous nodule","frequency":"Occasional (29-5%)","criteria":null},{"hpo":"HP:0000478","label":"Abnormality of the eye","frequency":"Very rare (<4-1%)","criteria":null},{"hpo":"HP:0001638","label":"Cardiomyopathy","frequency":"Very rare (<4-1%)","criteria":null},{"hpo":"HP:0002102","label":"Pleuritis","frequency":"Very rare (<4-1%)","criteria":null}]}}