{"orpha":183,"name":"Eosinophilic granulomatosis with polyangiitis","type":"Disease","group":"Disorder","status":"active","flags":[],"synonyms":["Churg-Strauss syndrome","EGPA","Granulomatous allergic angiitis"],"definition":"A rare systemic vasculitis of small vessels characterized by asthma, blood and tissue eosinophilia and vasculitis manifestations.","note":null,"url":"https://raremap.alftech.space/d/ORPHA-183/","orphanet_url":"https://www.orpha.net/en/disease/detail/183","names":{"fr":{"name":"Granulomatose éosinophilique avec polyangéite","synonyms":["Angéite allergique granulomateuse","EGPA","Syndrome de Churg-Strauss"],"definition":"Vascularite systémique rare des petits vaisseaux caractérisée par l'asthme, l'éosinophilie sanguine et tissulaire et des manifestations de vascularite."},"de":{"name":"Eosinophile Granulomatose mit Polyangiitis","synonyms":["Angiitis, allergische granulomatöse","Churg-Strauss-Syndrom","EGPA"],"definition":"Eine systemische Vaskulitis der kleinen Gefäße, die durch Asthma, Blut- und Gewebe-Eosinophilie und Vaskulitis-Manifestationen gekennzeichnet ist."},"es":{"name":"Granulomatosis eosinofílica con poliangeítis","synonyms":["Síndrome de Churg-Strauss"],"definition":"Es una vasculitis sistémica de pequeños vasos, poco frecuente, caracterizada por asma, eosinofilia sanguínea y tisular y manifestaciones vasculíticas."},"it":{"name":"Granulomatosi eosinofilica con poliangioite","synonyms":["Sindrome di Churg-Strauss"],"definition":"La granulomatosi eosinofila con poliangioite (EGPA) è una vasculite sistemica rara dei vasi di piccole dimensioni, caratterizzata da asma, eosinofilia del sangue e dei tessuti, e dai sintomi di una vasculite."},"nl":{"name":"Eosinofiele granulomatose met polyangiitis","synonyms":["EGPA","Granulomateuze allergische angiitis","Syndroom van Churg-Strauss"],"definition":"Een zeldzame systemische vasculitis van kleine bloedvaten, gekarakteriseerd door astma, eosinofilie van bloed en weefsel, en manifestaties van vasculitis."},"pt":{"name":"Granulomatose eosinofílica com poliangeíte","synonyms":["Doença de Churg-Strauss"]},"pl":{"name":"Eozynofilowa ziarniniakowatość z zapaleniem naczyń","synonyms":["Alergiczne ziarniniakowe zapalenie naczyń","EGPA","Zespół Churga i Straussa"]},"cs":{"name":"Eozinofilní granulomatóza s polyangiitidou","synonyms":["EGPA","Alergická granulomatózní angiitida","Churgův-Straussové syndrom","Churg-Straussové syndrom"]},"uk":{"name":"Еозинофільний гранулематоз з поліангіїтом","synonyms":["Синдром Чарга-Стросса","EGPA","Гранулематозний алергічний ангіїт"],"definition":"A rare systemic vasculitis of small vessels characterized by asthma, blood and tissue eosinophilia and vasculitis manifestations."},"zh":{"name":"嗜酸性肉芽肿性血管炎","synonyms":["肉芽肿性过敏性血管炎"]},"tr":{"name":"Polianjiitli eozinofilik granülomatoz","synonyms":["EGPA","Churg-Strauss sendromu","Granülomatöz alerjik anjit"]}},"xrefs":[{"source":"ICD-10","code":"M30.1","qualifier":"E","icd_relation":"specific","validated":true},{"source":"ICD-11","code":"4A44.A2","qualifier":"E","icd_relation":"specific","validated":true,"icd11_uri":"835880885"},{"source":"MONDO","code":"0015943","qualifier":"E","icd_relation":null,"validated":true},{"source":"MeSH","code":"D015267","qualifier":"E","icd_relation":null,"validated":true},{"source":"UMLS","code":"C0008728","qualifier":"E","icd_relation":null,"validated":true},{"source":"GARD","code":"6111","qualifier":"E","icd_relation":null,"validated":true},{"source":"MedDRA","code":"10048594","qualifier":"E","icd_relation":null,"validated":true}],"associations":[],"incoming_associations":[],"preferential_parent":{"orpha":98023,"name":"Rare systemic or rheumatologic disease"},"parents":[{"orpha":156152,"name":"Anti-neutrophil cytoplasmic antibody-associated vasculitis","classifications":[184,196,198,231,233]},{"orpha":324767,"name":"Non-familial rare disease with dilated cardiomyopathy","classifications":[146,233]},{"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":[],"genes":[],"genes_via_subtypes":[],"inheritance":["Not applicable"],"onset":["Adolescent","Adult","Elderly"],"prevalence":[{"type":"Point prevalence","qualification":"Value and class","class":"1-9 / 100 000","value":1.56,"area":"Europe","status":"Validated","source":"ORPHANET_25805746[PMID]"},{"type":"Annual incidence","qualification":"Value and class","class":"<1 / 1 000 000","value":0.09,"area":"Sweden","status":"Validated","source":"19797309[PMID]"},{"type":"Point prevalence","qualification":"Value and class","class":"1-9 / 100 000","value":1.13,"area":"France","status":"Validated","source":"20039171[PMID]_25805746[PMID]"},{"type":"Point prevalence","qualification":"Value and class","class":"1-9 / 100 000","value":1.4,"area":"Sweden","status":"Validated","source":"17553910[PMID]_25805746[PMID]"},{"type":"Annual incidence","qualification":"Value and class","class":"<1 / 1 000 000","value":0.09,"area":"United Kingdom","status":"Validated","source":"21798892[PMID]_25805746[PMID]"},{"type":"Point prevalence","qualification":"Value and class","class":"1-9 / 100 000","value":1.3,"area":"Norway","status":"Validated","source":"9805179[PMID]"},{"type":"Annual incidence","qualification":"Value and class","class":"<1 / 1 000 000","value":0.09,"area":"Spain","status":"Validated","source":"11156552[PMID]"},{"type":"Point prevalence","qualification":"Value and class","class":"1-9 / 100 000","value":2.23,"area":"Australia","status":"Validated","source":"18771432[PMID]_25805746[PMID]"},{"type":"Annual incidence","qualification":"Value and class","class":"1-9 / 1 000 000","value":0.18,"area":"Europe","status":"Validated","source":"ORPHANET_19797309[PMID]_18565978[PMID]_11156552[PMID]"},{"type":"Point prevalence","qualification":"Value and class","class":"1-9 / 100 000","value":1.5,"area":"Worldwide","status":"Validated","source":"18771432[PMID]_9805179[PMID]_17553910[PMID]_14872461[PMID]"},{"type":"Annual incidence","qualification":"Value and class","class":"<1 / 1 000 000","value":0.05,"area":"Norway","status":"Validated","source":"11760724[PMID]_25805746[PMID]"},{"type":"Annual incidence","qualification":"Value and class","class":"1-9 / 1 000 000","value":0.11,"area":"Germany","status":"Validated","source":"15696553[PMID]_25805746[PMID]"},{"type":"Annual incidence","qualification":"Value and class","class":"1-9 / 1 000 000","value":0.13,"area":"Lithuania","status":"Validated","source":"15647446[PMID]_25805746[PMID]"},{"type":"Annual incidence","qualification":"Value and class","class":"1-9 / 1 000 000","value":0.12,"area":"France","status":"Validated","source":"20039171[PMID]_25805746[PMID]"},{"type":"Annual incidence","qualification":"Value and class","class":"<1 / 1 000 000","value":0.06,"area":"Turkey","status":"Validated","source":"25805746[PMID]"},{"type":"Annual incidence","qualification":"Value and class","class":"<1 / 1 000 000","value":0.014,"area":"Peru","status":"Validated","source":"25805746[PMID]"},{"type":"Point prevalence","qualification":"Value and class","class":"1-9 / 100 000","value":2.4,"area":"Germany","status":"Validated","source":"24425780[PMID]_25805746[PMID]"},{"type":"Point prevalence","qualification":"Value and class","class":"1-9 / 100 000","value":1.78,"area":"Japan","status":"Validated","source":"24289197[PMID]_25805746[PMID]"},{"type":"Annual incidence","qualification":"Value and class","class":"1-9 / 1 000 000","value":0.4,"area":"United States","status":"Validated","source":"28881446[PMID]"}],"phenotypes":{"validation_status":"y","online":"y","validation_date":"2024-10-07","source":"30725784[PMID]","terms":[{"hpo":"HP:0002099","label":"Asthma","frequency":"Very frequent (99-80%)","criteria":null},{"hpo":"HP:0002960","label":"Autoimmunity","frequency":"Very frequent (99-80%)","criteria":null},{"hpo":"HP:0007009","label":"Central nervous system degeneration","frequency":"Very frequent (99-80%)","criteria":null},{"hpo":"HP:0012649","label":"Increased inflammatory response","frequency":"Very frequent (99-80%)","criteria":null},{"hpo":"HP:0001880","label":"Increased total eosinophil count","frequency":"Very frequent (99-80%)","criteria":null},{"hpo":"HP:0009830","label":"Peripheral neuropathy","frequency":"Very frequent (99-80%)","criteria":null},{"hpo":"HP:0002113","label":"Pulmonary infiltrates","frequency":"Very frequent (99-80%)","criteria":null},{"hpo":"HP:0000979","label":"Purpura","frequency":"Very frequent 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positivity","frequency":"Frequent (79-30%)","criteria":null},{"hpo":"HP:0033557","label":"Anti-proteinase 3 antibody positivity","frequency":"Frequent (79-30%)","criteria":null},{"hpo":"HP:0002829","label":"Arthralgia","frequency":"Frequent (79-30%)","criteria":null},{"hpo":"HP:0032230","label":"Cytoplasmic antineutrophil antibody positivity","frequency":"Frequent (79-30%)","criteria":null},{"hpo":"HP:0002015","label":"Dysphagia","frequency":"Frequent (79-30%)","criteria":null},{"hpo":"HP:0032071","label":"Eosinophilic pneumonia","frequency":"Frequent (79-30%)","criteria":null},{"hpo":"HP:0012378","label":"Fatigue","frequency":"Frequent (79-30%)","criteria":null},{"hpo":"HP:0001288","label":"Gait disturbance","frequency":"Frequent (79-30%)","criteria":null},{"hpo":"HP:0000790","label":"Hematuria","frequency":"Frequent (79-30%)","criteria":null},{"hpo":"HP:0000822","label":"Hypertension","frequency":"Frequent (79-30%)","criteria":null},{"hpo":"HP:0001639","label":"Hypertrophic 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