{"orpha":85435,"name":"Rheumatoid factor-positive polyarticular juvenile idiopathic arthritis","type":"Disease","group":"Disorder","status":"active","flags":[],"synonyms":["Juvenile idiopathic rheumatoid factor-positive polyarthritis","Juvenile polyarthritis with rheumatoid factor","Rheumatoid factor-positive polyarticular JIA"],"definition":"A rare form of juvenile idiopathic arthritis characterized by distal and symmetrical polyarthritis (more than 5 joints) with presence of rheumatoid factor and possible evolution towards the appearance of erosions and joint destruction.","note":null,"url":"https://raremap.alftech.space/d/ORPHA-85435/","orphanet_url":"https://www.orpha.net/en/disease/detail/85435","names":{"fr":{"name":"Arthrite juvénile idiopathique polyarticulaire avec facteur rhumatoïde","synonyms":["AJI polyarticulaire avec facteur rhumatoïde","Arthrite juvénile polyarticulaire avec facteur rhumatoïde","Polyarthrite juvénile avec facteur rhumatoïde","Polyarthrite juvénile facteur rhumatoïde positif"],"definition":"Forme rare d'arthrite juvénile idiopathique caractérisée par une polyarthrite distale et symétrique (affectant plus de cinq articulations) avec présence du facteur rhumatoïde et une évolution possible vers l'apparition d'érosions et de destruction articulaire."},"de":{"name":"Arthritis, idiopathische juvenile polyartikuläre, Rheumafaktor-positive","synonyms":["Juvenile Polyarthritis mit Rheumafaktor","Polyarthritis, juvenile, Rheumafaktor-positive","Rheumafaktor-positive polyartikuläre JIA"],"definition":"Eine seltene Form der juvenilen idiopathischen Arthritis, die durch eine distale und symmetrische Polyarthritis (mehr als 5 Gelenke) mit Vorhandensein von Rheumafaktoren und einer möglichen Entwicklung hin zu Erosionen und Gelenkzerstörung gekennzeichnet ist."},"es":{"name":"Artritis idiopática juvenil poliarticular factor reumatoide positivo","synonyms":["AIJ poliarticular factor reumatoide positivo","Poliartritis idiopática juvenil factor reumatoide positivo","Poliartritis juvenil con factor reumatoide"],"definition":"Es una forma de artritis idiopática juvenil poco frecuente caracterizada por poliartritis distal y simétrica (afectando a más de 5 articulaciones) con presencia de factor reumatoide y posible evolución hacia la aparición de erosiones y destrucción articular."},"it":{"name":"Artrite idiopatica giovanile poliarticolare con fattore reumatoide positivo","definition":"Si tratta di una forma rara di artrite idiopatica giovanile, caratterizzata da poliartrite distale simmetrica (che interessa più di 5 articolazioni) con fattore reumatoide positivo e possibile evoluzione verso la comparsa di erosioni e la distruzione delle articolazioni."},"nl":{"name":"Reumafactor-positieve polyarticulaire juveniele idiopathische artritis","synonyms":["Juveniele idiopathische reumafactor-positieve polyartritis","Juveniele polyartritis met reumafactor","Reumafactor-positieve polyarticulaire JIA"],"definition":"Een zeldzame vorm van juveniele idiopathische artritis, gekarakteriseerd door distale en symmetrische polyartritis (meer dan 5 gewrichten) met aanwezigheid van reumafactor en mogelijke evolutie naar erosie en destructie van gewricht."},"pt":{"name":"Poliartrite com fator reumatóide"},"pl":{"name":"Wielostawowe młodzieńcze idiopatyczne zapalenie stawów z dodatnim czynnikiem reumatoidalnym","synonyms":["Zapalenie wielostawowe z czynnikiem reumatoidalnym","JIA z dodatnim czynnikiem reumatoidalnym","Młodzieńcze zapalenie stawów z dodatnim czynnikiem reumatoidalnym"]},"cs":{"name":"Juvenilní revmatoidní faktor-pozitivní polyartritida","synonyms":["Polyartritida s revmatoidním faktorem"]},"uk":{"name":"Ревматоїдний фактор-позитивний поліартикулярний ювенільний ідіопатичний артрит","synonyms":["Ювенільний ідіопатичний ревматоїдний фактор-позитивний поліартрит","Ювенільний поліартрит з ревматоїдним фактором","Ревматоїдний фактор-позитивний поліартикулярний ЮРА"],"definition":"A rare form of juvenile idiopathic arthritis characterized by distal and symmetrical polyarthritis (more than 5 joints) with presence of rheumatoid factor and possible evolution towards the appearance of erosions and joint destruction."},"zh":{"name":"类风湿因子阳性的多关节型幼年特发性关节炎","synonyms":["类风湿因子阳性的多关节型幼年特发性关节炎"]},"tr":{"name":"Romatoid faktör-pozitif poliartiküler jüvenil idiyopatik artrit","synonyms":["Romatoid faktör-pozitif poliartiküler JIA","Romatoid faktörlü jüvenil poliartrit","Jüvenil idiyopatik romatoid faktör pozitif poliartrit"]}},"xrefs":[{"source":"ICD-10","code":"M08.0","qualifier":"NTBT","icd_relation":"inclusion","validated":true},{"source":"ICD-11","code":"FA24.1","qualifier":"NTBT","icd_relation":"index","validated":true,"icd11_uri":"1335443987"},{"source":"MONDO","code":"0019435","qualifier":"E","icd_relation":null,"validated":true},{"source":"UMLS","code":"C4048747","qualifier":"E","icd_relation":null,"validated":true}],"associations":[],"incoming_associations":[],"preferential_parent":{"orpha":98023,"name":"Rare systemic or rheumatologic disease"},"parents":[{"orpha":404580,"name":"Polyarticular juvenile idiopathic arthritis","classifications":[184,196,231,233]}],"children":[],"genes":[],"genes_via_subtypes":[],"inheritance":["Unknown"],"onset":["Childhood"],"prevalence":[{"type":"Point prevalence","qualification":"Value and class","class":"1-9 / 100 000","value":4.2,"area":"Europe","status":"Not yet validated","source":"ORPHANET"}],"phenotypes":{"validation_status":"y","online":"y","validation_date":"2021-05-25","source":"32119492[PMID]_29582130[PMID]_27785924[PMID]_29426059[PMID]","terms":[{"hpo":"HP:0002829","label":"Arthralgia","frequency":"Very frequent (99-80%)","criteria":null},{"hpo":"HP:0001369","label":"Arthritis","frequency":"Very frequent (99-80%)","criteria":null},{"hpo":"HP:0005764","label":"Polyarticular arthritis","frequency":"Very frequent (99-80%)","criteria":null},{"hpo":"HP:0002923","label":"Rheumatoid factor positive","frequency":"Very frequent (99-80%)","criteria":null},{"hpo":"HP:0011117","label":"Abnormal circulating interleukin concentration","frequency":"Frequent (79-30%)","criteria":null},{"hpo":"HP:0005930","label":"Abnormality of epiphysis morphology","frequency":"Frequent (79-30%)","criteria":null},{"hpo":"HP:0002813","label":"Abnormality of limb bone morphology","frequency":"Frequent (79-30%)","criteria":null},{"hpo":"HP:0011227","label":"Elevated circulating C-reactive protein concentration","frequency":"Frequent (79-30%)","criteria":null},{"hpo":"HP:0003565","label":"Elevated erythrocyte sedimentation rate","frequency":"Frequent (79-30%)","criteria":null},{"hpo":"HP:0006252","label":"Interphalangeal joint erosions","frequency":"Frequent (79-30%)","criteria":null},{"hpo":"HP:0001386","label":"Joint swelling","frequency":"Frequent (79-30%)","criteria":null},{"hpo":"HP:0001376","label":"Limitation of joint mobility","frequency":"Frequent (79-30%)","criteria":null},{"hpo":"HP:0005187","label":"Progressive joint destruction","frequency":"Frequent (79-30%)","criteria":null},{"hpo":"HP:0040311","label":"Symetrical distal arthritis","frequency":"Frequent (79-30%)","criteria":null},{"hpo":"HP:0100769","label":"Synovitis","frequency":"Frequent (79-30%)","criteria":null},{"hpo":"HP:0100555","label":"Asymmetric growth","frequency":"Occasional (29-5%)","criteria":null},{"hpo":"HP:0000938","label":"Osteopenia","frequency":"Occasional (29-5%)","criteria":null},{"hpo":"HP:0010588","label":"Premature epimetaphyseal fusion","frequency":"Occasional (29-5%)","criteria":null},{"hpo":"HP:0004349","label":"Reduced bone mineral density","frequency":"Occasional (29-5%)","criteria":null}]}}