{"orpha":47612,"name":"Felty syndrome","type":"Disease","group":"Disorder","status":"active","flags":[],"synonyms":["Splenomegaly-neutropenia-rheumatoid arthritis syndrome"],"definition":"Felty syndrome (FS), also known as ''super rheumatoid'' disease, is a severe form of rheumatoid arthritis (RA), characterized by a triad of RA, splenomegaly and neutropenia, resulting in susceptibility to bacterial infections.","note":null,"url":"https://raremap.alftech.space/d/ORPHA-47612/","orphanet_url":"https://www.orpha.net/en/disease/detail/47612","names":{"fr":{"name":"Syndrome de Felty","definition":"Le syndrome de Felty (SF), aussi connu sous le nom de maladie « super » rhumatoïde, est une forme sévère de polyarthrite rhumatoïde (PR), caractérisée par la triade : PR, splénomégalie et neutropénie, ce qui conduit à une susceptibilité à des infections bactériennes."},"de":{"name":"Felty-Syndrom","synonyms":["Splenomegalie-Neutropenie-rheumatoide Arthritis-Syndrom"],"definition":"Das Felty-Syndrom (FS), auch als 'super-rheumatoide' Krankheit bekannt, ist eine schwere Form der rheumatoiden Arthritis (RA; siehe dort) und gekennzeichnet durch die Trias RA, Splenomegalie und Neutropenie mit resultierender Anfälligkeit für bakterielle Infektionen."},"es":{"name":"Síndrome de Felty","synonyms":["Síndrome de esplenomegalia-neutropenia-artritis reumatoide"],"definition":"El síndrome de Felty (SF) es una forma grave de artritis reumatoide (AR; ver este término) que se caracteriza por la tríada de AR, esplenomegalia y neutropenia, y que provoca susceptibilidad a las infecciones bacterianas."},"it":{"name":"Sindrome di Felty","synonyms":["Sindrome splenomegalia-neutropenia-artrite reumatoide"],"definition":"La sidrome di Felty (FS) o malattia super reumatoide è una forma grave di artrite reumatoide (RA), caratterizzata dalla triade costituita da RA, splenomeglia e neutropenia, con conseguente suscettibilità alle infezioni batteriche."},"nl":{"name":"Syndroom van Felty","synonyms":["Splenomegalie - neutropenie - reumatoïde artritis-syndroom"],"definition":"Syndroom van Felty (FS), ook gekend als super-reumatoïde ziekte, is een ernstige vorm van reumatoïde artritis (RA; zie deze term) die wordt gekarakteriseerd door de triade RA, splenomegalie en neutropenie, en resulteert in vatbaarheid voor bacteriële infecties."},"pt":{"name":"Felty syndrome"},"pl":{"name":"Zespół Felty'ego","synonyms":["Zespół splenomegalia-neutropenia-reumatoidalne zapalnie stawów"],"definition":"Zespół Felty’ego (ZF), znany również jako choroba “super reumatoidalna”, jest ciężką postacią reumatoidalnego zapalenia stawów, charakteryzuje się triadą RZS, splenomegalią i neutropenią, dającą w rezultacie skłonność do infekcji bakteryjnych."},"cs":{"name":"Feltyho syndrom","synonyms":["Syndrom zahrnující splenomegalii, neutropenii a revmatoidní artritidu"]},"uk":{"name":"Синдром Фелті","synonyms":["Синдром спленомегалії-нейтропенії-ревматоїдного артриту"],"definition":"Felty syndrome (FS), also known as ''super rheumatoid'' disease, is a severe form of rheumatoid arthritis (RA), characterized by a triad of RA, splenomegaly and neutropenia, resulting in susceptibility to bacterial infections."},"zh":{"name":"Felty综合征","synonyms":["脾大-嗜中性白血球减少症-风湿性关节炎综合征"]},"tr":{"name":"Felty sendromu","synonyms":["Splenomegali-nötropeni-romatoid artrit sendromu"]}},"xrefs":[{"source":"OMIM","code":"134750","qualifier":"E","icd_relation":null,"validated":true},{"source":"ICD-10","code":"M05.0","qualifier":"NTBT","icd_relation":"attributed","validated":true},{"source":"ICD-11","code":"FA20.0","qualifier":"NTBT","icd_relation":"index","validated":true,"icd11_uri":"1118910093"},{"source":"MONDO","code":"0007603","qualifier":"E","icd_relation":null,"validated":true},{"source":"MeSH","code":"D005258","qualifier":"E","icd_relation":null,"validated":true},{"source":"UMLS","code":"C0015773","qualifier":"E","icd_relation":null,"validated":true},{"source":"GARD","code":"8234","qualifier":"E","icd_relation":null,"validated":true},{"source":"MedDRA","code":"10016386","qualifier":"E","icd_relation":null,"validated":true}],"associations":[],"incoming_associations":[],"preferential_parent":{"orpha":98023,"name":"Rare systemic or rheumatologic disease"},"parents":[{"orpha":178996,"name":"Acquired neutropenia","classifications":[195]},{"orpha":182231,"name":"Rare rheumatologic disease","classifications":[196]}],"children":[],"genes":[],"genes_via_subtypes":[],"inheritance":["Unknown"],"onset":["Adult"],"prevalence":[{"type":"Point prevalence","qualification":"Class only","class":"Unknown","value":0.0,"area":"Worldwide","status":"Not yet validated","source":"ORPHANET"}],"phenotypes":{"validation_status":"y","online":"y","validation_date":"2016-06-01","source":null,"terms":[{"hpo":"HP:0001367","label":"Abnormal joint morphology","frequency":"Very frequent (99-80%)","criteria":null},{"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:0002960","label":"Autoimmunity","frequency":"Very frequent (99-80%)","criteria":null},{"hpo":"HP:0001875","label":"Decreased total neutrophil count","frequency":"Very frequent (99-80%)","criteria":null},{"hpo":"HP:0002721","label":"Immunodeficiency","frequency":"Very frequent (99-80%)","criteria":null},{"hpo":"HP:0001376","label":"Limitation of joint mobility","frequency":"Very frequent (99-80%)","criteria":null},{"hpo":"HP:0002797","label":"Osteolysis","frequency":"Very frequent (99-80%)","criteria":null},{"hpo":"HP:0002719","label":"Recurrent infections","frequency":"Very frequent (99-80%)","criteria":null},{"hpo":"HP:0002205","label":"Recurrent respiratory infections","frequency":"Very frequent (99-80%)","criteria":null},{"hpo":"HP:0001482","label":"Subcutaneous nodule","frequency":"Very frequent (99-80%)","criteria":null},{"hpo":"HP:0100769","label":"Synovitis","frequency":"Very frequent (99-80%)","criteria":null},{"hpo":"HP:0004332","label":"Abnormal lymphocyte morphology","frequency":"Frequent (79-30%)","criteria":null},{"hpo":"HP:0001903","label":"Anemia","frequency":"Frequent (79-30%)","criteria":null},{"hpo":"HP:0000389","label":"Chronic otitis media","frequency":"Frequent (79-30%)","criteria":null},{"hpo":"HP:0002716","label":"Lymphadenopathy","frequency":"Frequent (79-30%)","criteria":null},{"hpo":"HP:0100776","label":"Recurrent pharyngitis","frequency":"Frequent (79-30%)","criteria":null},{"hpo":"HP:0006532","label":"Recurrent pneumonia","frequency":"Frequent (79-30%)","criteria":null},{"hpo":"HP:0012384","label":"Rhinitis","frequency":"Frequent (79-30%)","criteria":null},{"hpo":"HP:0000246","label":"Sinusitis","frequency":"Frequent (79-30%)","criteria":null},{"hpo":"HP:0001744","label":"Splenomegaly","frequency":"Frequent (79-30%)","criteria":null},{"hpo":"HP:0001824","label":"Weight loss","frequency":"Frequent (79-30%)","criteria":null},{"hpo":"HP:0005528","label":"Bone marrow hypocellularity","frequency":"Occasional (29-5%)","criteria":null},{"hpo":"HP:0100658","label":"Cellulitis","frequency":"Occasional (29-5%)","criteria":null},{"hpo":"HP:0100534","label":"Episcleritis","frequency":"Occasional (29-5%)","criteria":null},{"hpo":"HP:0007440","label":"Generalized hyperpigmentation","frequency":"Occasional (29-5%)","criteria":null},{"hpo":"HP:0002240","label":"Hepatomegaly","frequency":"Occasional (29-5%)","criteria":null},{"hpo":"HP:0007400","label":"Irregular hyperpigmentation","frequency":"Occasional (29-5%)","criteria":null},{"hpo":"HP:0002665","label":"Lymphoma","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:0002102","label":"Pleuritis","frequency":"Occasional (29-5%)","criteria":null},{"hpo":"HP:0002206","label":"Pulmonary fibrosis","frequency":"Occasional (29-5%)","criteria":null},{"hpo":"HP:0000010","label":"Recurrent urinary tract infections","frequency":"Occasional (29-5%)","criteria":null},{"hpo":"HP:0100806","label":"Sepsis","frequency":"Occasional (29-5%)","criteria":null},{"hpo":"HP:0001873","label":"Thrombocytopenia","frequency":"Occasional (29-5%)","criteria":null}]}}