{"orpha":85436,"name":"Psoriasis-related juvenile idiopathic arthritis","type":"Disease","group":"Disorder","status":"active","flags":[],"synonyms":["Juvenile psoriatic arthritis","Psoriasis-related JIA"],"definition":"A rare pediatric inflammatory rheumatic disease characterized by the presence of arthritis accompanied by either psoriasis or at least two of the following supporting features; presence of nail pitting, onycholysis, dactylitis, or a family history of psoriasis in a first degree relative. Patients are younger than 16 years of age and the disease lasts longer than 6 weeks.","note":null,"url":"https://raremap.alftech.space/d/ORPHA-85436/","orphanet_url":"https://www.orpha.net/en/disease/detail/85436","names":{"fr":{"name":"Arthrite juvénile idiopathique associée au psoriasis","synonyms":["Arthrite psoriasique juvénile","AJI associée au psoriasis"],"definition":"Maladie rhumatologique inflammatoire rare en pédiatrie caractérisée par la présence d'une arthrite accompagnée soit de psoriasis, soit d'au moins deux des caractéristiques suivantes : présence de piqueté unguéal, onycholyse, dactylite, ou antécédents familiaux de psoriasis chez un parent du premier degré. Les patients sont âgés de moins de 16 ans et la maladie persiste plus de six semaines."},"de":{"name":"Arthritis, idiopathische juvenile, Psoriasis-assoziierte","synonyms":["Juvenile Psoriasis-Arthritis","Psoriasis-assoziierte JIA"],"definition":"Eine seltene entzündlich-rheumatische Erkrankung bei Kindern, die durch das Vorhandensein von Arthritis gekennzeichnet ist, die entweder von Psoriasis oder mindestens zwei der folgenden Begleitmerkmale begleitet wird: Vorhandensein von Nagelflecken, Onycholyse, Daktylitis oder eine Familienanamnese von Psoriasis bei einem Verwandten ersten Grades. Die Patienten sind jünger als 16 Jahre und die Krankheit dauert länger als 6 Wochen."},"es":{"name":"Artritis idiopática juvenil asociada a psoriasis","synonyms":["AIJ asociada a psoriaris","Artritis psoriásica juvenil"],"definition":"Es una enfermedad reumática inflamatoria pediátrica poco frecuente caracterizada por la presencia de artritis acompañada de psoriasis o de, al menos, dos de las siguientes características:presencia de punteado en las uñas, onicólisis, dactilitis o antecedentes familiares de psoriasis en un pariente de primer grado. Los pacientes son menores de 16 años y la enfermedad tiene una duración de más de 6 semanas."},"it":{"name":"Artrite idiopatica giovanile correlata alla psoriasi","definition":"L'artrite idiopatica giovanile correlata alla psoriasi è una malattia reumatica infiammatoria pediatrica rara, caratterizzata dall'artrite associata alla psoriasi o ad almeno due dei seguenti segni clinici: fossette ungueali, onicolisi, dattilite, oppure un'anamnesi familiare positiva per la psoriasi in un consanguineo di primo grado. La malattia colpisce i giovani di età inferiore ai 16 anni e dura per più di sei settimane."},"nl":{"name":"Psoriasis-gerelateerde juveniele idiopathische artritis","synonyms":["Juveniele psoriatische artritis","Psoriasis-gerelateerde JIA"],"definition":"Een zeldzame pediatrische inflammatoire reumatische aandoening, gekarakteriseerd door aanwezigheid van artritis vergezeld van ofwel psoriasis ofwel minstens twee van de volgende kenmerken: aanwezigheid van putjes in nagels, onycholyse, dactylitis, of een familiegeschiedenis van psoriasis bij een eerstegraadsverwant. Patiënten zijn jonger dan 16 jaar en de ziekte duurt langer dan 6 weken."},"pt":{"name":"Artrite idiopática juvenil psoríase-relacionada","synonyms":["Artrite psoriática juvenil"]},"pl":{"name":"Młodzieńcze zapalenie stawów związane z łuszczycą","synonyms":["JIA związane z łuszczycą","Młodzieńcze łuszczycowe zapalenie stawów"]},"cs":{"name":"Juvenilní psoriatická artritida","synonyms":["Juvenile psoriatic arthritis","Psoriasis-related JIA"]},"uk":{"name":"Псоріаз-асоційований ювенільний ідіопатичний артрит","synonyms":["Ювенільний псоріатичний артрит","ЮРА, повязаний з псоріазом"],"definition":"A rare pediatric inflammatory rheumatic disease characterized by the presence of arthritis accompanied by either psoriasis or at least two of the following supporting features; presence of nail pitting, onycholysis, dactylitis, or a family history of psoriasis in a first degree relative. Patients are younger than 16 years of age and the disease lasts longer than 6 weeks."},"zh":{"name":"银屑病相关幼年特发性关节炎","synonyms":["青少年银屑病性关节炎"]},"tr":{"name":"Psöriazise bağlı jüvenil idiyopatik artrit","synonyms":["Sedef hastalığı-ilişkili JIA","Jüvenil psoriatik artrit"]}},"xrefs":[{"source":"ICD-10","code":"L40.5+","qualifier":"NTBT","icd_relation":"attributed","validated":true},{"source":"ICD-10","code":"M09.0*","qualifier":"NTBT","icd_relation":"attributed","validated":true},{"source":"ICD-11","code":"FA24.2","qualifier":"E","icd_relation":"specific","validated":true,"icd11_uri":"1473955563"},{"source":"MONDO","code":"0019436","qualifier":"E","icd_relation":null,"validated":true},{"source":"UMLS","code":"C3714758","qualifier":"E","icd_relation":null,"validated":true},{"source":"GARD","code":"10970","qualifier":"E","icd_relation":null,"validated":true},{"source":"MedDRA","code":"10076674","qualifier":"E","icd_relation":null,"validated":true}],"associations":[],"incoming_associations":[],"preferential_parent":{"orpha":98023,"name":"Rare systemic or rheumatologic disease"},"parents":[{"orpha":92,"name":"Juvenile idiopathic arthritis","classifications":[184,196,231,233]}],"children":[],"genes":[],"genes_via_subtypes":[],"inheritance":["Unknown"],"onset":["Adolescent","Childhood"],"prevalence":[{"type":"Point prevalence","qualification":"Class only","class":"Unknown","value":0.0,"area":"Worldwide","status":"Validated","source":"24210707[PMID]"}],"phenotypes":{"validation_status":"y","online":"y","validation_date":"2019-06-06","source":"29101009[PMID]_17162381[PMID]_28148698[PMID]_28222745[PMID]_28752373[PMID]","terms":[{"hpo":"HP:0011118","label":"Abnormality of tumor necrosis factor secretion","frequency":"Very frequent (99-80%)","criteria":null},{"hpo":"HP:0003493","label":"Antinuclear antibody positivity","frequency":"Frequent (79-30%)","criteria":null},{"hpo":"HP:0002829","label":"Arthralgia","frequency":"Frequent (79-30%)","criteria":null},{"hpo":"HP:0001369","label":"Arthritis","frequency":"Frequent (79-30%)","criteria":null},{"hpo":"HP:0002960","label":"Autoimmunity","frequency":"Frequent (79-30%)","criteria":null},{"hpo":"HP:0100686","label":"Enthesitis","frequency":"Frequent (79-30%)","criteria":null},{"hpo":"HP:0031090","label":"Finger dactylitis","frequency":"Frequent (79-30%)","criteria":null},{"hpo":"HP:0001803","label":"Nail pits","frequency":"Frequent (79-30%)","criteria":null},{"hpo":"HP:0040313","label":"Oligoarthritis","frequency":"Frequent (79-30%)","criteria":null},{"hpo":"HP:0005764","label":"Polyarticular arthritis","frequency":"Frequent (79-30%)","criteria":null},{"hpo":"HP:0000989","label":"Pruritus","frequency":"Frequent (79-30%)","criteria":null},{"hpo":"HP:0003765","label":"Psoriasiform dermatitis","frequency":"Frequent (79-30%)","criteria":null},{"hpo":"HP:0031091","label":"Toe dactylitis","frequency":"Frequent (79-30%)","criteria":null},{"hpo":"HP:0002815","label":"Abnormality of the knee","frequency":"Occasional (29-5%)","criteria":null},{"hpo":"HP:0003043","label":"Abnormality of the shoulder","frequency":"Occasional (29-5%)","criteria":null},{"hpo":"HP:0003019","label":"Abnormality of the wrist","frequency":"Occasional (29-5%)","criteria":null},{"hpo":"HP:0012122","label":"Anterior uveitis","frequency":"Occasional (29-5%)","criteria":null},{"hpo":"HP:0005197","label":"Generalized morning stiffness","frequency":"Occasional (29-5%)","criteria":null},{"hpo":"HP:0001376","label":"Limitation of joint mobility","frequency":"Occasional (29-5%)","criteria":null},{"hpo":"HP:0025300","label":"Malar rash","frequency":"Occasional (29-5%)","criteria":null},{"hpo":"HP:0025526","label":"Psoriasiform lesion","frequency":"Occasional (29-5%)","criteria":null},{"hpo":"HP:0007663","label":"Reduced visual acuity","frequency":"Occasional (29-5%)","criteria":null},{"hpo":"HP:0000988","label":"Skin rash","frequency":"Occasional (29-5%)","criteria":null},{"hpo":"HP:0000554","label":"Uveitis","frequency":"Occasional (29-5%)","criteria":null},{"hpo":"HP:0010754","label":"Abnormality of the temporomandibular joint","frequency":"Very rare (<4-1%)","criteria":null},{"hpo":"HP:0001094","label":"Iridocyclitis","frequency":"Very rare (<4-1%)","criteria":null},{"hpo":"HP:0001101","label":"Iritis","frequency":"Very rare (<4-1%)","criteria":null},{"hpo":"HP:0001806","label":"Onycholysis","frequency":"Very rare (<4-1%)","criteria":null},{"hpo":"HP:0012317","label":"Sacroiliac arthritis","frequency":"Very rare (<4-1%)","criteria":null}]}}