{"orpha":727,"name":"Microscopic polyangiitis","type":"Disease","group":"Disorder","status":"active","flags":[],"synonyms":["MPA","Micropolyangiitis","Microscopic polyarteritis"],"definition":"A rare inflammatory, necrotizing, systemic vasculitis that affects predominantly small vessels (i.e. small arteries, arterioles, capillaries, venules) in multiple organs, including the kidney, the lungs, the skin and the peripheral nerves.","note":null,"url":"https://raremap.alftech.space/d/ORPHA-727/","orphanet_url":"https://www.orpha.net/en/disease/detail/727","names":{"fr":{"name":"Polyangéite microscopique","synonyms":["MPA","Micropolyangéite","PAM","Polyartérite microscopique"],"definition":"Vascularite inflammatoire nécrosante systémique rare qui touche principalement les vaisseaux de petit calibre de multiples organes (petites artères, artérioles, capillaires, veinules)."},"de":{"name":"Polyangiitis, mikroskopische","synonyms":["MPA","Mikropolyangiitis","Mikroskopische Panarteriitis"],"definition":"Eine seltene entzündliche, nekrotisierende, systemische Vaskulitis, die vorwiegend kleine Gefäße (d. h. kleine Arterien, Arteriolen, Kapillaren, Venolen) in mehreren Organen betrifft, darunter die Niere, die Lunge, die Haut und die peripheren Nerven."},"es":{"name":"Poliangeitis microscópica","synonyms":["MPA","Micropoliangeítis","Poliangeítis microscópica","Poliarteritis microscópica"],"definition":"Es una vasculitis sistémica, inflamatoria y necrotizante, poco frecuente, que afecta predominantemente a los vasos pequeños (es decir, arterias pequeñas, arteriolas, capilares, vénulas) en múltiples órganos, incluyendo el riñón, los pulmones, la piel y los nervios periféricos."},"it":{"name":"Poliangioite microscopica","synonyms":["MPA","Micropoliangioite","Poliarterite microscopica"],"definition":"La poliangioite microscopica (MPA) è una vasculite sistemica necrotizzante infiammatoria rara, che interessa per lo più i piccoli vasi (piccole arterie, arteriole, capillari, venule) in diversi organi (reni, polmoni, cute e nervi periferici)."},"nl":{"name":"Microscopische polyangiitis","synonyms":["MPA","Micropolyangiitis","Microscopische polyarteriitis"],"definition":"Een zeldzame inflammatoire necrotiserende systemische vasculitis die vooral kleine bloedvaten aantast (i.e. kleine arteriën, arteriolen, capillairen, venulen) in meerdere organen, waaronder nier, long, huid en perifere zenuwen."},"pt":{"name":"Poliangeíte microscópica","definition":"A poliangeíte microscópica (MPA) é uma vasculite sistémica, inflamatória e necrotizante que afecta predominantemente pequenos vasos (ou seja, pequenas artérias, arteríolas, capilares, vénulas) de múltiplos órgãos."},"pl":{"name":"Mikroskopowe zapalenie naczyń","synonyms":["Mikroskopowe zapalenie tętnic","MPA"]},"cs":{"name":"Mikroskopická polyangiitida","synonyms":["MPA","Mikropolyangiitida","Mikroskopická polyarteritida"]},"uk":{"name":"Мікроскопічний поліангіїт","synonyms":["Мікрополіангіїт","Мікроскопічний поліартеріїт","MPA"],"definition":"A rare inflammatory, necrotizing, systemic vasculitis that affects predominantly small vessels (i.e. small arteries, arterioles, capillaries, venules) in multiple organs, including the kidney, the lungs, the skin and the peripheral nerves."},"zh":{"name":"显微镜下多血管炎","synonyms":["显微镜下多动脉炎"]},"tr":{"name":"Mikroskobik polianjiit","synonyms":["MPA","Mikropolianjit","Mikroskobik poliarterit"]}},"xrefs":[{"source":"ICD-10","code":"M31.7","qualifier":"E","icd_relation":"specific","validated":true},{"source":"ICD-11","code":"4A44.A0","qualifier":"E","icd_relation":"specific","validated":true,"icd11_uri":"999231798"},{"source":"MONDO","code":"0019124","qualifier":"E","icd_relation":null,"validated":true},{"source":"MeSH","code":"D055953","qualifier":"E","icd_relation":null,"validated":true},{"source":"UMLS","code":"C2347126","qualifier":"E","icd_relation":null,"validated":true},{"source":"GARD","code":"3652","qualifier":"E","icd_relation":null,"validated":true},{"source":"MedDRA","code":"10063344","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":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":["Adult","Childhood"],"prevalence":[{"type":"Annual incidence","qualification":"Value and class","class":"1-9 / 100 000","value":1.0,"area":"Europe","status":"Validated","source":"[EXPERT]"},{"type":"Annual incidence","qualification":"Value and class","class":"1-9 / 1 000 000","value":0.26,"area":"Germany","status":"Validated","source":"15696553[PMID]_25805746[PMID]"},{"type":"Point prevalence","qualification":"Value and class","class":"1-9 / 100 000","value":4.2843,"area":"Worldwide","status":"Validated","source":"25805746[PMID]_ORPHANET"},{"type":"Annual incidence","qualification":"Value and class","class":"1-9 / 100 000","value":1.16,"area":"Spain","status":"Validated","source":"11156552[PMID]"},{"type":"Point prevalence","qualification":"Value and class","class":"1-9 / 100 000","value":9.4,"area":"Sweden","status":"Validated","source":"17553910[PMID]"},{"type":"Annual incidence","qualification":"Value and class","class":"1-9 / 1 000 000","value":0.5,"area":"Australia","status":"Validated","source":"18771432[PMID]"},{"type":"Point prevalence","qualification":"Value and class","class":"1-9 / 100 000","value":2.5,"area":"France","status":"Validated","source":"14872461[PMID]"},{"type":"Annual incidence","qualification":"Value and class","class":"1-9 / 1 000 000","value":0.27,"area":"Norway","status":"Validated","source":"11760724[PMID]"},{"type":"Point prevalence","qualification":"Value and class","class":"1-9 / 100 000","value":3.7,"area":"New Zealand","status":"Validated","source":"16368729[PMID]"},{"type":"Annual incidence","qualification":"Value and class","class":"1-9 / 1 000 000","value":0.74,"area":"United Kingdom","status":"Not yet validated","source":"11156552[PMID]_22258386[PMID]_10693883[PMID]_ORPHANET"},{"type":"Point prevalence","qualification":"Value and class","class":"1-9 / 100 000","value":6.3,"area":"United Kingdom","status":"Validated","source":"22258386[PMID]_25805746[PMID]"},{"type":"Point prevalence","qualification":"Value and class","class":"1-9 / 100 000","value":2.8,"area":"Germany","status":"Validated","source":"24425780[PMID]_25805746[PMID]"},{"type":"Point prevalence","qualification":"Value and class","class":"1-9 / 100 000","value":3.91,"area":"Australia","status":"Validated","source":"18771432[PMID]_25805746[PMID]"},{"type":"Point prevalence","qualification":"Value and class","class":"1-9 / 100 000","value":1.38,"area":"Japan","status":"Validated","source":"17699321[PMID]_25805746[PMID]"},{"type":"Annual incidence","qualification":"Value and class","class":"1-9 / 100 000","value":2.4,"area":"Kuwait","status":"Validated","source":"9306338[PMID]_25805746[PMID]"},{"type":"Annual incidence","qualification":"Value and class","class":"1-9 / 100 000","value":1.82,"area":"Japan","status":"Validated","source":"21798892[PMID]_25805746[PMID]"},{"type":"Annual incidence","qualification":"Value and class","class":"1-9 / 1 000 000","value":0.71,"area":"Canada","status":"Validated","source":"23838025[PMID]_25805746[PMID]"},{"type":"Annual incidence","qualification":"Value and class","class":"1-9 / 100 000","value":1.02,"area":"Greece","status":"Validated","source":"19604432[PMID]_25805746[PMID]"},{"type":"Annual incidence","qualification":"Value and class","class":"1-9 / 1 000 000","value":0.3,"area":"Lithuania","status":"Validated","source":"15647446[PMID]_25805746[PMID]"},{"type":"Annual incidence","qualification":"Value and class","class":"1-9 / 100 000","value":1.01,"area":"Sweden","status":"Validated","source":"19797309[PMID]_25805746[PMID]"},{"type":"Annual incidence","qualification":"Value and class","class":"1-9 / 100 000","value":1.6,"area":"United States","status":"Validated","source":"28881446[PMID]"}],"phenotypes":{"validation_status":"y","online":"y","validation_date":"2024-10-03","source":"30285379[PMID]","terms":[{"hpo":"HP:0002960","label":"Autoimmunity","frequency":"Very frequent (99-80%)","criteria":null},{"hpo":"HP:0008653","label":"Crescentic glomerulonephritis","frequency":"Very frequent (99-80%)","criteria":null},{"hpo":"HP:0010783","label":"Erythema","frequency":"Very frequent (99-80%)","criteria":null},{"hpo":"HP:0001945","label":"Fever","frequency":"Very frequent (99-80%)","criteria":null},{"hpo":"HP:0000099","label":"Glomerulonephritis","frequency":"Very frequent (99-80%)","criteria":null},{"hpo":"HP:0100820","label":"Glomerulopathy","frequency":"Very frequent (99-80%)","criteria":null},{"hpo":"HP:0000790","label":"Hematuria","frequency":"Very frequent (99-80%)","criteria":null},{"hpo":"HP:0002105","label":"Hemoptysis","frequency":"Very frequent (99-80%)","criteria":null},{"hpo":"HP:0012649","label":"Increased inflammatory response","frequency":"Very frequent (99-80%)","criteria":null},{"hpo":"HP:0100520","label":"Oliguria","frequency":"Very frequent (99-80%)","criteria":null},{"hpo":"HP:0000083","label":"Renal insufficiency","frequency":"Very frequent (99-80%)","criteria":null},{"hpo":"HP:0000988","label":"Skin rash","frequency":"Very frequent (99-80%)","criteria":null},{"hpo":"HP:0002633","label":"Vasculitis","frequency":"Very frequent (99-80%)","criteria":null},{"hpo":"HP:0002027","label":"Abdominal pain","frequency":"Frequent (79-30%)","criteria":null},{"hpo":"HP:0012614","label":"Abnormal urine cytology","frequency":"Frequent (79-30%)","criteria":null},{"hpo":"HP:0001903","label":"Anemia","frequency":"Frequent (79-30%)","criteria":null},{"hpo":"HP:0033559","label":"Anti-myeloperoxidase antibody positivity","frequency":"Frequent (79-30%)","criteria":null},{"hpo":"HP:0034104","label":"Anti-neutrophil elastase antibody 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:0002014","label":"Diarrhea","frequency":"Frequent (79-30%)","criteria":null},{"hpo":"HP:0003565","label":"Elevated erythrocyte sedimentation rate","frequency":"Frequent (79-30%)","criteria":null},{"hpo":"HP:0012378","label":"Fatigue","frequency":"Frequent (79-30%)","criteria":null},{"hpo":"HP:0002239","label":"Gastrointestinal hemorrhage","frequency":"Frequent (79-30%)","criteria":null},{"hpo":"HP:0005244","label":"Gastrointestinal infarctions","frequency":"Frequent (79-30%)","criteria":null},{"hpo":"HP:0033505","label":"Livedo reticularis","frequency":"Frequent (79-30%)","criteria":null},{"hpo":"HP:0032018","label":"Multiple mononeuropathy","frequency":"Frequent (79-30%)","criteria":null},{"hpo":"HP:0003326","label":"Myalgia","frequency":"Frequent (79-30%)","criteria":null},{"hpo":"HP:0002017","label":"Nausea and vomiting","frequency":"Frequent (79-30%)","criteria":null},{"hpo":"HP:0002586","label":"Peritonitis","frequency":"Frequent (79-30%)","criteria":null},{"hpo":"HP:0004396","label":"Poor appetite","frequency":"Frequent (79-30%)","criteria":null},{"hpo":"HP:0200042","label":"Skin ulcer","frequency":"Frequent (79-30%)","criteria":null},{"hpo":"HP:0001933","label":"Subcutaneous hemorrhage","frequency":"Frequent (79-30%)","criteria":null},{"hpo":"HP:0004936","label":"Venous thrombosis","frequency":"Frequent (79-30%)","criteria":null},{"hpo":"HP:0008046","label":"Abnormal retinal vascular morphology","frequency":"Occasional (29-5%)","criteria":null},{"hpo":"HP:0011675","label":"Arrhythmia","frequency":"Occasional (29-5%)","criteria":null},{"hpo":"HP:0001369","label":"Arthritis","frequency":"Occasional (29-5%)","criteria":null},{"hpo":"HP:0100749","label":"Chest pain","frequency":"Occasional (29-5%)","criteria":null},{"hpo":"HP:0001635","label":"Congestive heart failure","frequency":"Occasional (29-5%)","criteria":null},{"hpo":"HP:0000965","label":"Cutis marmorata","frequency":"Occasional (29-5%)","criteria":null},{"hpo":"HP:0025420","label":"Diffuse alveolar hemorrhage","frequency":"Occasional (29-5%)","criteria":null},{"hpo":"HP:0100534","label":"Episcleritis","frequency":"Occasional (29-5%)","criteria":null},{"hpo":"HP:0000421","label":"Epistaxis","frequency":"Occasional (29-5%)","criteria":null},{"hpo":"HP:0100758","label":"Gangrene","frequency":"Occasional (29-5%)","criteria":null},{"hpo":"HP:0031363","label":"Palpable purpura","frequency":"Occasional (29-5%)","criteria":null},{"hpo":"HP:0001733","label":"Pancreatitis","frequency":"Occasional (29-5%)","criteria":null},{"hpo":"HP:0003401","label":"Paresthesia","frequency":"Occasional (29-5%)","criteria":null},{"hpo":"HP:0001701","label":"Pericarditis","frequency":"Occasional (29-5%)","criteria":null},{"hpo":"HP:0009830","label":"Peripheral neuropathy","frequency":"Occasional (29-5%)","criteria":null},{"hpo":"HP:0025188","label":"Retinal vasculitis","frequency":"Occasional (29-5%)","criteria":null},{"hpo":"HP:0000246","label":"Sinusitis","frequency":"Occasional (29-5%)","criteria":null},{"hpo":"HP:0001482","label":"Subcutaneous nodule","frequency":"Occasional (29-5%)","criteria":null},{"hpo":"HP:0000554","label":"Uveitis","frequency":"Occasional (29-5%)","criteria":null},{"hpo":"HP:0001824","label":"Weight loss","frequency":"Occasional (29-5%)","criteria":null}]}}