{"orpha":2902,"name":"Idiopathic chronic eosinophilic pneumonia","type":"Disease","group":"Disorder","status":"active","flags":[],"synonyms":["Chronic eosinophilic pneumonia"],"definition":"A rare, severe, interstitial lung disease characterized by insidious onset with subacute or chronic non-specific respiratory manifestations (dyspnea, cough, wheezing) often associated with systemic manifestations (fatigue, malaise, weight loss) and a history of asthma (up to half of patients). Eosinophilia is present in most cases, usually in excess of 1000 cells/mm3.","note":null,"url":"https://raremap.alftech.space/d/ORPHA-2902/","orphanet_url":"https://www.orpha.net/en/disease/detail/2902","names":{"fr":{"name":"Pneumopathie chronique idiopathique à éosinophiles","synonyms":["Maladie de Carrington","Pneumonie éosinophile chronique"],"definition":"La pneumonie chronique idiopathique à éosinophiles se caractérise par l'association de symptômes respiratoires et généraux subaigus ou chroniques, une éosinophilie alvéolaire et/ou plasmatique et des infiltrats pulmonaires de topographie périphérique en imagerie."},"de":{"name":"Pneumopathie, eosinophile idiopathische, chronische","synonyms":["Carrington-Krankheit"],"definition":"Eine schwere interstitielle Lungenerkrankung, die durch einen schleichenden Beginn mit subakuten oder chronischen unspezifischen Atemwegsmanifestationen (Dyspnoe, Husten, Keuchen) gekennzeichnet ist, die häufig mit systemischen Manifestationen (Müdigkeit, Unwohlsein, Gewichtsverlust) und einer Asthmaanamnese (bei bis zur Hälfte der Patienten) einhergehen. In den meisten Fällen liegt eine Eosinophilie vor, in der Regel mit mehr als 1000 Zellen/mm3."},"es":{"name":"Neumonía eosinofílica crónica idiopática","synonyms":["NECI","Síndrome de Carrington"],"definition":"Es una enfermedad pulmonar intersticial grave y poco frecuente caracterizada por un inicio insidioso con manifestaciones respiratorias subagudas o crónicas inespecíficas (disnea, tos, sibilancias) a menudo asociadas a manifestaciones sistémicas (cansancio, malestar, pérdida de peso) y a antecedentes de asma (hasta el 50% de los pacientes). La eosinofilia está presente en la mayoría de los casos, normalmente por encima de 1000 células/mm3."},"it":{"name":"Polmonite cronica idiopatica con eosinofili","synonyms":["Sindrome di Carrington"],"definition":"La polmonite cronica idiopatica con eosinofili (ICEP) è una pneumopatia interstiziale grave molto rara. L'esordio è insidioso, caratterizzato dalla presenza di segni clinici respiratori subacuti o cronici e aspecifici (dispnea, tosse e affanno), spesso associati a segni sistemici (affaticamento, malessere e perdita di peso)."},"nl":{"name":"Idiopathische chronische eosinofiele pneumonie","synonyms":["Chronische eosinofiele pneumonie"],"definition":"Een zeldzame, ernstige, interstitiële longziekte, gekarakteriseerd door sluipende aanvang met subacute of chronische aspecifieke respiratoire manifestaties (dyspneu, hoest, piepende ademhaling), vaak geassocieerd met systemische manifestaties (vermoeidheid, malaise, gewichtsverlies) en een voorgeschiedenis met astma (tot de helft van de patiënten). Eosinofilie is aanwezig in de meeste gevallen, meestal met meer dan 1000 cellen/mm3."},"pt":{"name":"Pneumopatia eosinofílica crónica idiopática","synonyms":["Síndrome Carrington"]},"pl":{"name":"Idiopatyczne przewlekłe eozynofilowe zapalenie płuc","synonyms":["Przewlekłe eozynofilowe zapalenie płuc"]},"cs":{"name":"Idiopatická chronická eozinofilní pneumonie","synonyms":["Chronická eozinofilní pneumonie"]},"uk":{"name":"Ідіопатична хронічна еозинофільна пневмонія","synonyms":["Хронічна еозинофільна пневмонія"],"definition":"A rare, severe, interstitial lung disease characterized by insidious onset with subacute or chronic non-specific respiratory manifestations (dyspnea, cough, wheezing) often associated with systemic manifestations (fatigue, malaise, weight loss) and a history of asthma (up to half of patients). Eosinophilia is present in most cases, usually in excess of 1000 cells/mm3."},"zh":{"name":"特发性慢性嗜酸细胞性肺炎","synonyms":["慢性嗜酸粒细胞性肺炎"]},"tr":{"name":"İdiyopatik kronik eozinofilik pnömoni","synonyms":["Kronik eozinofilik pnömoni"]}},"xrefs":[{"source":"ICD-10","code":"J82","qualifier":"NTBT","icd_relation":"attributed","validated":true},{"source":"ICD-11","code":"CB02.11","qualifier":"E","icd_relation":"specific","validated":true,"icd11_uri":"958353326"},{"source":"MONDO","code":"0017363","qualifier":"E","icd_relation":null,"validated":true},{"source":"UMLS","code":"C2930941","qualifier":"E","icd_relation":null,"validated":true},{"source":"GARD","code":"1130","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]},{"orpha":98052,"name":"Rare allergic respiratory disease","classifications":[212]}],"children":[],"genes":[],"genes_via_subtypes":[],"inheritance":["Not applicable"],"onset":["All ages"],"prevalence":[{"type":"Point prevalence","qualification":"Class only","class":"Unknown","value":0.0,"area":"Worldwide","status":"Not yet validated","source":"ORPHANET"},{"type":"Annual incidence","qualification":"Value and class","class":"1-9 / 1 000 000","value":0.23,"area":"Iceland","status":"Validated","source":"17277407[PMID]"}],"phenotypes":{"validation_status":"y","online":"y","validation_date":"2021-08-26","source":"29735405[PMID]_33898133[PMID]","terms":[{"hpo":"HP:0031983","label":"Abnormal pulmonary thoracic imaging finding","frequency":"Very frequent (99-80%)","criteria":null},{"hpo":"HP:0032177","label":"Parenchymal consolidation","frequency":"Very frequent (99-80%)","criteria":null},{"hpo":"HP:0002099","label":"Asthma","frequency":"Frequent (79-30%)","criteria":null},{"hpo":"HP:0001047","label":"Atopic dermatitis","frequency":"Frequent (79-30%)","criteria":null},{"hpo":"HP:0002960","label":"Autoimmunity","frequency":"Frequent (79-30%)","criteria":null},{"hpo":"HP:0030830","label":"Crackles","frequency":"Frequent (79-30%)","criteria":null},{"hpo":"HP:0002094","label":"Dyspnea","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:0032061","label":"Hypereosinophilia","frequency":"Frequent (79-30%)","criteria":null},{"hpo":"HP:0006516","label":"Hypersensitivity pneumonitis","frequency":"Frequent (79-30%)","criteria":null},{"hpo":"HP:0012418","label":"Hypoxemia","frequency":"Frequent (79-30%)","criteria":null},{"hpo":"HP:0003212","label":"Increased circulating IgE concentration","frequency":"Frequent (79-30%)","criteria":null},{"hpo":"HP:0001974","label":"Increased total leukocyte count","frequency":"Frequent (79-30%)","criteria":null},{"hpo":"HP:0031246","label":"Nonproductive cough","frequency":"Frequent (79-30%)","criteria":null},{"hpo":"HP:0002091","label":"Restrictive ventilatory defect","frequency":"Frequent (79-30%)","criteria":null},{"hpo":"HP:0032017","label":"Sputum eosinophilia","frequency":"Frequent (79-30%)","criteria":null},{"hpo":"HP:0030828","label":"Wheezing","frequency":"Frequent (79-30%)","criteria":null},{"hpo":"HP:0002829","label":"Arthralgia","frequency":"Occasional (29-5%)","criteria":null},{"hpo":"HP:0025406","label":"Asthenia","frequency":"Occasional (29-5%)","criteria":null},{"hpo":"HP:0001945","label":"Fever","frequency":"Occasional (29-5%)","criteria":null},{"hpo":"HP:0030166","label":"Night sweats","frequency":"Occasional (29-5%)","criteria":null},{"hpo":"HP:0001824","label":"Weight loss","frequency":"Occasional (29-5%)","criteria":null},{"hpo":"HP:0011024","label":"Abnormality of the gastrointestinal tract","frequency":"Very rare (<4-1%)","criteria":null},{"hpo":"HP:0100750","label":"Atelectasis","frequency":"Very rare (<4-1%)","criteria":null},{"hpo":"HP:0011354","label":"Generalized abnormality of skin","frequency":"Very rare (<4-1%)","criteria":null},{"hpo":"HP:0002202","label":"Pleural effusion","frequency":"Very rare (<4-1%)","criteria":null}]}}