{"orpha":761,"name":"Immunoglobulin A vasculitis","type":"Disease","group":"Disorder","status":"active","flags":[],"synonyms":["Anaphylactoid purpura","Henoch-Schönlein purpura","IgA vasculitis","Purpura rheumatica","Rheumatoid purpura"],"definition":"A rare, small-vessel vasculitis characterized by skin purpura, arthritis, abdominal and/or renal involvement, IgA tissue deposits (arterioles, capillaries, and venules) and circulating IgA immune complexes.","note":null,"url":"https://raremap.alftech.space/d/ORPHA-761/","orphanet_url":"https://www.orpha.net/en/disease/detail/761","names":{"fr":{"name":"Vascularite à immunoglobulines A","synonyms":["Purpura anaphylactique","Purpura de Henoch-Schönlein","Purpura rhumatoïde","Vascularite à IgA"],"definition":"Vascularite rare des vaisseaux de petit calibre caractérisée par un purpura cutané, de l'arthrite, une atteinte abdominale et/ou rénale, des dépôts tissulaires d'IgA (artérioles, capillaires et veinules) et des complexes immuns circulants de type IgA."},"de":{"name":"Immunoglobulin A-Vaskulitis","synonyms":["IgA-Vaskulitis","Purpura Schönlein-Henoch","Purpura rheumatica","Purpura, anaphylaktoide","Rheumatische Purpura","Schoenlein-Henoch-Purpura"],"definition":"Eine seltene Vaskulitis der kleinen Gefäße, gekennzeichnet durch Hautpurpura, Arthritis, Bauch- und/oder Nierenbeteiligung, IgA-Gewebeablagerungen (Arteriolen, Kapillaren und Venolen) und zirkulierende IgA-Immunkomplexe."},"es":{"name":"Vasculitis por inmunoglobulina A","synonyms":["Púrpura anafilactoide","Púrpura de Henoch-Schönlein","Púrpura reumatoide","Púrpura reumática","Vasculitis IgA"],"definition":"Es una vasculitis poco frecuente de pequeños vasos caracterizada por púrpura cutánea, artritis, afectación abdominal y/o renal, depósitos tisulares de IgA (arteriolas, capilares y vénulas) y complejos inmunes circulantes de IgA."},"it":{"name":"Vasculite da immuglobuline A","synonyms":["Porpora anafilattoide","Porpora di Henoch-Schönlein","Porpora reumatica","Porpora reumatoide","Vasculite da IgA"],"definition":"Si tratta di una vasculite rara dei piccoli vasi, caratterizzata da porpora della cute, artrite, interessamento addominale e/o renale, depositi di IgA nei tessuti (arteriole, capillari e venule) e complessi immuni di IgA circolanti."},"nl":{"name":"Immunoglobuline A-vasculitis","synonyms":["Anafylactoïde purpura","IgA-vasculitis","Purpura rheumatica","Purpura van Henoch-Schönlein","Reumatoïde purpura"],"definition":"Een zeldzame vasculitis van kleine bloedvaten, gekarakteriseerd door purpura van huid, artritis, betrokkenheid van abdomen en/of nier, IgA-depositie in weefsels (arteriolen, capillairen en venulen) en circulerende IgA-immuuncomplexen."},"pt":{"name":"Vasculite Imunoglobulina A","synonyms":["Púrpura anafilática","Púrpura de Henoch-Schönlein","Púrpura reumatóide","Púrpura reumática","Vasculite IgA"],"definition":"A púrpura Schönlein-Henoch (SHP) é uma vasculite sistémica de IgA que afecta pequenos vasos. É caracterizada por púrpura cutânea, artrite e envolvimento abdominal e/ou renal."},"pl":{"name":"plamica Henocha i Schönleina","synonyms":["Czerwienica reumatyczna","plamica rzekomoanafilaktyczna","Zapalenie naczyń związane z immunoglobuliną A"]},"cs":{"name":"Imunoglobulin A vaskulitida","synonyms":["Anafylaktoidní purpura","Henochova-Schönleinova purpura","IgA vaskulitida","Revmatická purpura","Revmatoidní purpura","Henoch-Schönleinova purpura"]},"uk":{"name":"Васкуліт, асоційований з імуноглобуліном А","synonyms":["Анафілактоїдна пурпура","Пурпура Геноха-Шенлейна","Васкуліт IgA","Ревматична пурпура","Ревматоїдна пурпура"],"definition":"A rare, small-vessel vasculitis characterized by skin purpura, arthritis, abdominal and/or renal involvement, IgA tissue deposits (arterioles, capillaries, and venules) and circulating IgA immune complexes."},"zh":{"name":"IgA血管炎","synonyms":["Henoch-Schönlein紫癜"]},"tr":{"name":"İmmünoglobulin A vasküliti","synonyms":["IgA vasküliti","Purpura romatika","Romatoid purpura","Anafilaktoid purpura","Henoch-Schönlein purpurası"]}},"xrefs":[{"source":"ICD-10","code":"D69.0","qualifier":"NTBT","icd_relation":"inclusion","validated":true},{"source":"ICD-11","code":"4A44.92","qualifier":"E","icd_relation":"specific","validated":true,"icd11_uri":"1629105375"},{"source":"MONDO","code":"0019167","qualifier":"E","icd_relation":null,"validated":true},{"source":"MeSH","code":"D011695","qualifier":"E","icd_relation":null,"validated":true},{"source":"UMLS","code":"C0034152","qualifier":"E","icd_relation":null,"validated":true},{"source":"GARD","code":"8204","qualifier":"E","icd_relation":null,"validated":true},{"source":"MedDRA","code":"10082960","qualifier":"E","icd_relation":null,"validated":true}],"associations":[],"incoming_associations":[],"preferential_parent":{"orpha":98023,"name":"Rare systemic or rheumatologic disease"},"parents":[{"orpha":156149,"name":"Immune complex mediated vasculitis","classifications":[196,198]},{"orpha":280369,"name":"Rare pediatric vasculitis","classifications":[231]},{"orpha":264709,"name":"Secondary interstitial lung disease specific to childhood associated with a systemic vasculitis","classifications":[184,233]},{"orpha":567560,"name":"Systemic vasculitis associated with glomerulopathy","classifications":[188,233]}],"children":[],"genes":[],"genes_via_subtypes":[],"inheritance":["Not applicable"],"onset":["Childhood"],"prevalence":[{"type":"Annual incidence","qualification":"Value and class","class":"6-9 / 10 000","value":55.9,"area":"Korea, Republic of","status":"Validated","source":"36263029[PMID]"},{"type":"Annual incidence","qualification":"Value and class","class":"1-5 / 10 000","value":30.0,"area":"France","status":"Validated","source":"36263029[PMID]"},{"type":"Annual incidence","qualification":"Value and class","class":"1-9 / 100 000","value":6.79,"area":"Croatia","status":"Validated","source":"36263029[PMID]"},{"type":"Annual incidence","qualification":"Value and class","class":"1-5 / 10 000","value":13.0,"area":"United Kingdom","status":"Validated","source":"36263029[PMID]"},{"type":"Annual incidence","qualification":"Value and class","class":"1-9 / 100 000","value":6.1,"area":"Netherlands","status":"Validated","source":"36263029[PMID]"},{"type":"Annual incidence","qualification":"Value and class","class":"1-5 / 10 000","value":17.55,"area":"Sweden","status":"Validated","source":"36263029[PMID]"},{"type":"Annual incidence","qualification":"Value and class","class":"1-9 / 100 000","value":12.9,"area":"Taiwan, Province of China","status":"Validated","source":"36263029[PMID]"},{"type":"Point prevalence","qualification":"Class only","class":"Unknown","value":0.0,"area":"Worldwide","status":"Validated","source":"36263029[PMID]_ORPHANET"}],"phenotypes":{"validation_status":"y","online":"y","validation_date":"2016-06-01","source":null,"terms":[{"hpo":"HP:0002027","label":"Abdominal pain","frequency":"Very frequent (99-80%)","criteria":null},{"hpo":"HP:0002829","label":"Arthralgia","frequency":"Very frequent (99-80%)","criteria":null},{"hpo":"HP:0000978","label":"Bruising susceptibility","frequency":"Very frequent (99-80%)","criteria":null},{"hpo":"HP:0005244","label":"Gastrointestinal infarctions","frequency":"Very frequent (99-80%)","criteria":null},{"hpo":"HP:0000790","label":"Hematuria","frequency":"Very frequent (99-80%)","criteria":null},{"hpo":"HP:0002017","label":"Nausea and vomiting","frequency":"Very frequent (99-80%)","criteria":null},{"hpo":"HP:0000979","label":"Purpura","frequency":"Very frequent (99-80%)","criteria":null},{"hpo":"HP:0200039","label":"Pustule","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:0002039","label":"Anorexia","frequency":"Frequent (79-30%)","criteria":null},{"hpo":"HP:0001369","label":"Arthritis","frequency":"Frequent (79-30%)","criteria":null},{"hpo":"HP:0010783","label":"Erythema","frequency":"Frequent (79-30%)","criteria":null},{"hpo":"HP:0001945","label":"Fever","frequency":"Frequent (79-30%)","criteria":null},{"hpo":"HP:0002383","label":"Infectious encephalitis","frequency":"Frequent (79-30%)","criteria":null},{"hpo":"HP:0002076","label":"Migraine","frequency":"Frequent (79-30%)","criteria":null},{"hpo":"HP:0003326","label":"Myalgia","frequency":"Frequent (79-30%)","criteria":null},{"hpo":"HP:0100796","label":"Orchitis","frequency":"Frequent (79-30%)","criteria":null},{"hpo":"HP:0200042","label":"Skin ulcer","frequency":"Frequent (79-30%)","criteria":null},{"hpo":"HP:0011276","label":"Vascular skin abnormality","frequency":"Frequent (79-30%)","criteria":null},{"hpo":"HP:0100665","label":"Angioedema","frequency":"Occasional (29-5%)","criteria":null},{"hpo":"HP:0000969","label":"Edema","frequency":"Occasional (29-5%)","criteria":null},{"hpo":"HP:0100534","label":"Episcleritis","frequency":"Occasional (29-5%)","criteria":null},{"hpo":"HP:0002239","label":"Gastrointestinal hemorrhage","frequency":"Occasional (29-5%)","criteria":null},{"hpo":"HP:0100820","label":"Glomerulopathy","frequency":"Occasional (29-5%)","criteria":null},{"hpo":"HP:0004374","label":"Hemiplegia/hemiparesis","frequency":"Occasional (29-5%)","criteria":null},{"hpo":"HP:0012733","label":"Macule","frequency":"Occasional (29-5%)","criteria":null},{"hpo":"HP:0001324","label":"Muscle weakness","frequency":"Occasional (29-5%)","criteria":null},{"hpo":"HP:0000648","label":"Optic atrophy","frequency":"Occasional (29-5%)","criteria":null},{"hpo":"HP:0000093","label":"Proteinuria","frequency":"Occasional (29-5%)","criteria":null},{"hpo":"HP:0000083","label":"Renal insufficiency","frequency":"Occasional (29-5%)","criteria":null},{"hpo":"HP:0002091","label":"Restrictive ventilatory defect","frequency":"Occasional (29-5%)","criteria":null},{"hpo":"HP:0001250","label":"Seizure","frequency":"Occasional (29-5%)","criteria":null},{"hpo":"HP:0001025","label":"Urticaria","frequency":"Occasional (29-5%)","criteria":null}]}}