{"orpha":79126,"name":"Acute interstitial pneumonia","type":"Disease","group":"Disorder","status":"active","flags":[],"synonyms":["Acute interstitial pneumonitis","Hamman-Rich syndrome"],"definition":"A rare, rapidly progressive, and histologically distinct, form of idiopathic interstitial pneumonia.","note":null,"url":"https://raremap.alftech.space/d/ORPHA-79126/","orphanet_url":"https://www.orpha.net/en/disease/detail/79126","names":{"fr":{"name":"Pneumopathie interstitielle aiguë","synonyms":["Pneumonite interstitielle aiguë","Syndrome de Hamman-Rich"],"definition":"Forme rare et rapidement progressive de pneumonie interstitielle idiopathique présentant des caractéristiques histologiques particulières."},"de":{"name":"Pneumonie, interstitielle, akute","synonyms":["Hamman-Rich-Syndrom"],"definition":"Eine seltene, schnell fortschreitende und histologisch distinkte Form der idiopathischen interstitiellen Pneumonie."},"es":{"name":"Neumonía intersticial aguda","synonyms":["Neumonitis intersticial aguda","Síndrome de Hamman-Rich"],"definition":"Es una forma poco frecuente de neumonía intersticial idiopática rápidamente progresiva e histológicamente diferenciada."},"it":{"name":"Polmonite acuta interstiziale","synonyms":["Sindrome di Hamman-Rich"]},"nl":{"name":"Acute interstitiële pneumonie","synonyms":["Acute interstitiële pneumonitis","Syndroom van Hamman-Rich"],"definition":"Een zeldzame, snel progressieve en histologisch aparte vorm van idiopathische interstitiële pneumonie."},"pt":{"name":"Pneumonia intersticial aguda","synonyms":["Pneumonite intersticial aguda","Síndrome de Hamman-Rich"],"definition":"Forma rara, rapidamente progressiva e histologicamente distinta de pneumonia intersticial idiopática."},"pl":{"name":"Ostre śródmiąższowe zapalenie płuc","synonyms":["Ostre śródmiąższowe zapalenie płuc","Zespół Hammana i Richa"]},"cs":{"name":"Akutní intersticiální pneumonie","synonyms":["Akutní intersticiální pneumonitida","Hammanův-Richův syndrom","Hamman-Richův syndrom"]},"uk":{"name":"Гостра інтерстиціальна пневмонія","synonyms":["Гострий інтерстиціальний пневмоніт","Синдром Хаммана-Річа"],"definition":"A rare, rapidly progressive, and histologically distinct, form of idiopathic interstitial pneumonia."},"zh":{"name":"急性间质性肺炎","synonyms":["急性间质性肺炎"]},"tr":{"name":"Akut interstisyel pnömoni","synonyms":["Hamman-Rich sendromu","Akut interstisyel pnömoni"]}},"xrefs":[{"source":"OMIM","code":"178500","qualifier":"NTBT","icd_relation":null,"validated":true},{"source":"ICD-10","code":"J84.1","qualifier":"NTBT","icd_relation":"inclusion","validated":true},{"source":"ICD-11","code":"CB03.0","qualifier":"E","icd_relation":"specific","validated":true,"icd11_uri":"2116884221"},{"source":"MONDO","code":"0019203","qualifier":"E","icd_relation":null,"validated":true},{"source":"UMLS","code":"C0085786","qualifier":"E","icd_relation":null,"validated":true},{"source":"GARD","code":"12835","qualifier":"E","icd_relation":null,"validated":true},{"source":"MedDRA","code":"10066728","qualifier":"E","icd_relation":null,"validated":true}],"associations":[],"incoming_associations":[],"preferential_parent":{"orpha":97955,"name":"Rare respiratory disease"},"parents":[{"orpha":98300,"name":"Idiopathic interstitial pneumonia","classifications":[184,233]}],"children":[],"genes":[],"genes_via_subtypes":[],"inheritance":["Unknown"],"onset":["Adult"],"prevalence":[{"type":"Point prevalence","qualification":"Value and class","class":"1-9 / 100 000","value":3.8,"area":"Europe","status":"Validated","source":"ORPHANET"}],"phenotypes":{"validation_status":"y","online":"y","validation_date":"2020-02-12","source":"31333914[PMID]_29159034[PMID]_19095855[PMID]","terms":[{"hpo":"HP:0002110","label":"Bronchiectasis","frequency":"Very frequent (99-80%)","criteria":null},{"hpo":"HP:0002094","label":"Dyspnea","frequency":"Very frequent (99-80%)","criteria":null},{"hpo":"HP:0025179","label":"Ground-glass opacification on pulmonary HRCT","frequency":"Very frequent (99-80%)","criteria":null},{"hpo":"HP:0012418","label":"Hypoxemia","frequency":"Very frequent (99-80%)","criteria":null},{"hpo":"HP:0030879","label":"Interlobular septal thickening on pulmonary HRCT","frequency":"Very frequent 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(29-5%)","criteria":null},{"hpo":"HP:0003259","label":"Elevated circulating creatinine concentration","frequency":"Occasional (29-5%)","criteria":null},{"hpo":"HP:0003565","label":"Elevated erythrocyte sedimentation rate","frequency":"Occasional (29-5%)","criteria":null},{"hpo":"HP:0002716","label":"Lymphadenopathy","frequency":"Occasional (29-5%)","criteria":null},{"hpo":"HP:0003326","label":"Myalgia","frequency":"Occasional (29-5%)","criteria":null},{"hpo":"HP:0001698","label":"Pericardial effusion","frequency":"Occasional (29-5%)","criteria":null},{"hpo":"HP:0012398","label":"Peripheral edema","frequency":"Occasional (29-5%)","criteria":null},{"hpo":"HP:0002206","label":"Pulmonary fibrosis","frequency":"Occasional (29-5%)","criteria":null},{"hpo":"HP:0031851","label":"Reduced hematocrit","frequency":"Occasional (29-5%)","criteria":null},{"hpo":"HP:0031631","label":"Subpleural honeycombing","frequency":"Occasional (29-5%)","criteria":null}]}}