{"orpha":724,"name":"Idiopathic acute eosinophilic pneumonia","type":"Disease","group":"Disorder","status":"active","flags":[],"synonyms":["IAEP"],"definition":"Idiopathic acute eosinophilic pneumonia (IAEP) is an eosinophilic pneumonia of undetermined etiology that is characterized by acute febrile hypoxic respiratory failure associated with diffuse radiographic infiltrates and pulmonary eosinophilia, but without concurring allergy or infection.","note":null,"url":"https://raremap.alftech.space/d/ORPHA-724/","orphanet_url":"https://www.orpha.net/en/disease/detail/724","names":{"fr":{"name":"Pneumonie à éosinophiles idiopathique aiguë","synonyms":["IAEP","Syndrome de Loeffler","Syndrome de Loffler"],"definition":"La pneumonie à éosinophiles idiopathique aigüe (PEIA) est une pneumonie éosinophilique d'étiologie indéterminée, caractérisée par une insuffisance respiratoire hypoxique fébrile aigüe associée à des infiltrats diffus à la radiographie et à une éosinophilie pulmonaire, mais sans allergie ou infection concomitante."},"de":{"name":"Pneumonie, eosinophile idiopathische, akute Form","synonyms":["IAEP"],"definition":"Die Idiopathische akute eosinophile Pneumonie (IAEP) ist eine eosinophile Pneumonie mit unbekannter Ursache. Charakteristisch sind: Akute febrile hypoxische Atemstörung, diffuse Infiltrate im Röntgenbild, pulmonale Eosinophilie, keine gleichzeitige Allergie oder Infektion."},"es":{"name":"Neumonía eosinofílica idiopática aguda","synonyms":["IAEP"],"definition":"La neumonía eosinofílica idiopática aguda (IAEP) es una neumonía eosinofílica de etiología indeterminada que se caracteriza por insuficiencia respiratoria hipóxica febril aguda asociada a infiltrados radiográficos difusos y eosinofilia pulmonar pero sin que concurra alergia o infección."},"it":{"name":"Polmonite eosinofila acuta idiopatica","synonyms":["IAEP"],"definition":"La polmonite eosinofila acuta idiopatica (IAEP) è una polmonite eosinofila ad eziologia non nota, caratterizzata da insufficienza respiratoria ipossica febbrile acuta, associata ad infiltrati radiografici diffusi ed eosinofilia polmonare, in assenza di allergia o di infezione concomitante."},"nl":{"name":"Idiopathische acute eosinofiele pneumonie","synonyms":["IAEP"],"definition":"Idiopathische acute eosinofiele pneumonie (IAEP) is een eosinofiele pneumonie met onbepaalde etiologie die wordt gekarakteriseerd door acute febriele hypoxische respiratoire insufficiëntie, diffuse infiltraten op een röntgenfoto en pulmonale eosinofilie, zonder gelijktijdige allergie of infectie."},"pt":{"name":"Pneumonia eosinofílica aguda idiopática","definition":"A pneumonia eosinofílica aguda idiopática (PEAI) é uma pneumonia eosinofílica de etiologia indeterminada, caracterizada por insuficiência respiratória hipóxica febril aguda associada a infiltrados radiográficos difusos e eosinofilia pulmonar, mas sem alergia ou infeção concomitante."},"pl":{"name":"Idiopatyczne ostre eozynofilowe zapalenie płuc","synonyms":["IAEP"],"definition":"Idiopatyczne ostre eozynofilowe zapalenie płuc (Idiopathic acute eosinophilic pneumonia – IAEP) to eozynofilowe zapalenie płuc o niezidentyfikowanej etiologii, które charakteryzuje się ostrą gorączkową niewydolnością oddechową związaną z rozlanymi naciekami widocznymi w obrazie radiologicznym oraz eozynofilią płucną, ale bez współistniejącej alergii lub infekcji."},"cs":{"name":"Idiopatická akutní eozinofilní pneumonie","synonyms":["IAEP","Loefflerův syndrom","Löfflerův syndrom"]},"uk":{"name":"Ідіопатична гостра еозинофільна пневмонія","synonyms":["IAEP"],"definition":"Idiopathic acute eosinophilic pneumonia (IAEP) is an eosinophilic pneumonia of undetermined etiology that is characterized by acute febrile hypoxic respiratory failure associated with diffuse radiographic infiltrates and pulmonary eosinophilia, but without concurring allergy or infection."},"zh":{"name":"特发性急性嗜酸粒细胞肺炎","synonyms":["IAEP"]},"tr":{"name":"İdiyopatik akut eozinofilik pnömoni","synonyms":["IAEP"]}},"xrefs":[{"source":"ICD-10","code":"J82","qualifier":"NTBT","icd_relation":"inclusion","validated":true},{"source":"ICD-11","code":"CB02.10","qualifier":"E","icd_relation":"specific","validated":true,"icd11_uri":"1455309767"},{"source":"MONDO","code":"0019122","qualifier":"E","icd_relation":null,"validated":true},{"source":"UMLS","code":"C4518469","qualifier":"E","icd_relation":null,"validated":true},{"source":"GARD","code":"519","qualifier":"E","icd_relation":null,"validated":true}],"associations":[],"incoming_associations":[],"preferential_parent":{"orpha":97955,"name":"Rare respiratory disease"},"parents":[{"orpha":182101,"name":"Idiopathic eosinophilic pneumonia","classifications":[184,233]}],"children":[],"genes":[],"genes_via_subtypes":[],"inheritance":["Unknown"],"onset":["All ages"],"prevalence":[{"type":"Cases/families","qualification":"Case(s)","class":null,"value":100.0,"area":"Worldwide","status":"Validated","source":"12403693[PMID]"},{"type":"Point prevalence","qualification":"Class only","class":"<1 / 1 000 000","value":0.0,"area":"Worldwide","status":"Validated","source":"ORPHANET"}],"phenotypes":{"validation_status":"y","online":"y","validation_date":"2016-06-01","source":null,"terms":[{"hpo":"HP:0100749","label":"Chest pain","frequency":"Very frequent (99-80%)","criteria":null},{"hpo":"HP:0001945","label":"Fever","frequency":"Very frequent (99-80%)","criteria":null},{"hpo":"HP:0002113","label":"Pulmonary infiltrates","frequency":"Very frequent (99-80%)","criteria":null},{"hpo":"HP:0002093","label":"Respiratory insufficiency","frequency":"Very frequent (99-80%)","criteria":null},{"hpo":"HP:0002091","label":"Restrictive ventilatory defect","frequency":"Very frequent (99-80%)","criteria":null},{"hpo":"HP:0002027","label":"Abdominal pain","frequency":"Frequent (79-30%)","criteria":null},{"hpo":"HP:0002793","label":"Abnormal pattern of respiration","frequency":"Frequent (79-30%)","criteria":null},{"hpo":"HP:0001879","label":"Abnormality of eosinophils","frequency":"Frequent (79-30%)","criteria":null},{"hpo":"HP:0002103","label":"Abnormality of the pleura","frequency":"Frequent (79-30%)","criteria":null},{"hpo":"HP:0012735","label":"Cough","frequency":"Frequent (79-30%)","criteria":null},{"hpo":"HP:0003326","label":"Myalgia","frequency":"Frequent (79-30%)","criteria":null}]}}