{"orpha":31740,"name":"Hypersensitivity pneumonitis","type":"Disease","group":"Disorder","status":"active","flags":[],"synonyms":["Extrinsic allergic alveolitis"],"definition":"A rare, immunologically mediated, interstitial lung disease characterized by respiratory symptoms (cough, dyspnea) due to sensitization and subsequent hypersensitivity to environmental antigens. The disorder may be accompanied by systemic manifestations (weight loss, fatigue).","note":null,"url":"https://raremap.alftech.space/d/ORPHA-31740/","orphanet_url":"https://www.orpha.net/en/disease/detail/31740","names":{"fr":{"name":"Alvéolite allergique extrinsèque","synonyms":["AAE","Pneumopathie d'hypersensibilité"],"definition":"Maladie pulmonaire interstitielle rare, à médiation immunitaire, caractérisée par des symptômes respiratoires (toux, dyspnée) dus à une sensibilisation et à une hypersensibilité consécutive à des antigènes environnementaux. La maladie peut s'accompagner de manifestations systémiques (perte de poids, fatigue)."},"de":{"name":"Exogen-allergische Alveolitis","synonyms":["Hypersensitivitätspneumonie"],"definition":"Eine seltene, immunologisch vermittelte, interstitielle Lungenerkrankung, die durch respiratorische Symptome (Husten, Dyspnoe) infolge einer Sensibilisierung und anschließenden Überempfindlichkeit gegenüber Umweltantigenen gekennzeichnet ist. Die Erkrankung kann von systemischen Manifestationen (Gewichtsverlust, Müdigkeit) begleitet sein."},"es":{"name":"Neumonitis por hipersensibilidad","synonyms":["Alveolitis alérgica extrínseca"],"definition":"Es una enfermedad pulmonar intersticial inmunomediada poco frecuente caracterizada por síntomas respiratorios (tos, disnea) debida a la sensibilización y la subsiguiente hipersensibilidad a antígenos ambientales. El trastorno puede estar acompañado de manifestaciones sistémicas (pérdida de peso, cansancio)."},"it":{"name":"Polmonite da ipersensibilità","synonyms":["Alveolite allergica estrinseca","HP"]},"nl":{"name":"Hypersensitiviteitspneumonitis","synonyms":["Extrinsieke allergische alveolitis","Overgevoeligheidspneumonitis"],"definition":"Een zeldzame, immunologisch gemedieerde, interstitiële longziekte, gekenmerkt door respiratoire symptomen (hoest, dyspneu) als gevolg van sensibilisatie en daaropvolgend hypersensitiviteit voor antigenen uit de omgeving. De aandoening kan gepaard gaan met systemische manifestaties (gewichtsverlies, vermoeidheid)."},"pt":{"name":"Pneumonite de hipersensibilidade","synonyms":["Alveolite alérgica extrínseca"],"definition":"Doença pulmonar intersticial rara, imunologicamente mediada, caracterizada por sintomas respiratórios (tosse, dispneia) devido à sensibilização e subsequente hipersensibilidade a antigénios ambientais. A patologia pode ser acompanhada de manifestações sistémicas (perda de peso, fadiga)."},"pl":{"name":"Alergiczne zapalenie pęcherzyków płucnych","synonyms":["Extrinsic allergic alveolitis","HP"]},"cs":{"name":"Hypersenzitivní pneumonitida","synonyms":["HP"]},"uk":{"name":"Гіперчутливий пневмоніт","synonyms":["Зовнішній алергічний альвеоліт"],"definition":"A rare, immunologically mediated, interstitial lung disease characterized by respiratory symptoms (cough, dyspnea) due to sensitization and subsequent hypersensitivity to environmental antigens. The disorder may be accompanied by systemic manifestations (weight loss, fatigue)."},"zh":{"name":"过敏性肺炎","synonyms":["外源性过敏性肺泡炎"]},"tr":{"name":"Hipersensitif pnömoni","synonyms":["Ekstrinsik alerjik alveolit"]}},"xrefs":[{"source":"OMIM","code":"145300","qualifier":"BTNT","icd_relation":null,"validated":true},{"source":"ICD-10","code":"J67.8","qualifier":"NTBT","icd_relation":"attributed","validated":false},{"source":"ICD-11","code":"CA70","qualifier":"E","icd_relation":"specific","validated":true,"icd11_uri":"589867913"},{"source":"MONDO","code":"0017853","qualifier":"E","icd_relation":null,"validated":true},{"source":"MeSH","code":"D000542","qualifier":"E","icd_relation":null,"validated":true},{"source":"UMLS","code":"C0002390","qualifier":"E","icd_relation":null,"validated":true},{"source":"GARD","code":"12","qualifier":"E","icd_relation":null,"validated":true},{"source":"MedDRA","code":"10081988","qualifier":"E","icd_relation":null,"validated":true}],"associations":[],"incoming_associations":[{"type":"Referred to","orpha":99906,"name":"OBSOLETE: Farmer's lung disease","status":"inactive"},{"type":"Referred to","orpha":99907,"name":"OBSOLETE: House allergic alveolitis","status":"inactive"},{"type":"Referred to","orpha":99908,"name":"OBSOLETE: Pigeon-breeder lung disease","status":"inactive"},{"type":"Referred to","orpha":99909,"name":"OBSOLETE: Occupational allergic alveolitis","status":"inactive"}],"preferential_parent":{"orpha":97955,"name":"Rare respiratory disease"},"parents":[{"orpha":264984,"name":"Exposure-related interstitial lung disease","classifications":[184,233]},{"orpha":98052,"name":"Rare allergic respiratory disease","classifications":[212]}],"children":[{"orpha":686465,"name":"Fibrotic hypersensitivity pneumonitis","type":"Clinical subtype"},{"orpha":686462,"name":"Non-fibrotic hypersensitivity pneumonitis","type":"Clinical subtype"}],"genes":[],"genes_via_subtypes":[{"symbol":"MUC5B","name":"mucin 5B, oligomeric mucus/gel-forming","gene_type":"gene with protein product","association":"Major susceptibility factor in","status":"Assessed","source":"28648751[PMID]","hgnc":"7516","ensembl":"ENSG00000117983","omim":"600770","loci":["11p15.5"],"via":{"orpha":686465,"name":"Fibrotic hypersensitivity pneumonitis"}}],"inheritance":["Not applicable"],"onset":["All ages"],"prevalence":[{"type":"Point prevalence","qualification":"Class only","class":"Unknown","value":0.0,"area":"Worldwide","status":"Validated","source":"ORPHANET"}],"phenotypes":null}