{"orpha":1302,"name":"Cryptogenic organizing pneumonia","type":"Disease","group":"Disorder","status":"active","flags":[],"synonyms":["BOOP","Bronchiolitis obliterans organizing pneumonia","COP"],"definition":"A rare form of idiopathic interstitial pneumonia characterized pathologically by organizing pneumonia (OP) that presents with nonspecific flu-like symptoms, as well as cough and dyspnea, and where no etiological agent is found.","note":null,"url":"https://raremap.alftech.space/d/ORPHA-1302/","orphanet_url":"https://www.orpha.net/en/disease/detail/1302","names":{"fr":{"name":"Pneumopathie organisée cryptogénique","synonyms":["BOOP","Bronchiolite oblitérante avec organisation pneumonique","POC"],"definition":"Forme rare de pneumopathie interstitielle idiopathique, caractérisée sur le plan pathologique par une pneumopathie organisée (PO) qui se manifeste par des signes non spécifiques semblables à ceux de la grippe, une toux et une dyspnée, en l'absence d'agent étiologique identifié."},"de":{"name":"Pneumonie, kryptogene organisierende","synonyms":["BOOP","Bronchiolitis obliterans mit organisierender Pneumonie","COP"],"definition":"Eine seltene Form der idiopathischen interstitiellen Pneumonie, die pathologisch durch eine organisierende Pneumonie (OP) gekennzeichnet ist, die mit unspezifischen grippeähnlichen Symptomen sowie Husten und Dyspnoe einhergeht und bei der kein ätiologischer Erreger gefunden wird."},"es":{"name":"Neumonía con organización criptogénica","synonyms":["BONO","Bronquiolitis obliterante con neumonía organizada","NOC"],"definition":"Es una forma poco frecuente de neumonía intersticial idiopática caracterizada patológicamente por neumonía organizativa (NO) que cursa con síntomas gripales inespecíficos, tales como tos y disnea, y para la que no se encuentra ningún agente etiológico."},"it":{"name":"Polmonite criptogenica organizzata","synonyms":["Bronchiolite obliterante - polmonite organizzata"],"definition":"La polmonite criptogenica organizzata (COP) è una forma di polmonite interstiziale idiopatica; dal punto di vista patologico è caratterizzata da polmonite organizzata (OP), che esordisce con sintomi influenzali aspecifici, tosse e dispnea; l'eziologia non è nota."},"nl":{"name":"Cryptogene organiserende pneumonie","synonyms":["BOOP","Bronchiolitis obliterans organiserende pneumonie","COP"],"definition":"Een zeldzame vorm van idiopathische interstitiële pneumonie, pathologisch gekenmerkt door organiserende pneumonie (OP) die zich presenteert met aspecifieke griepachtige symptomen, alsook hoest en dyspneu, en waarvan de etiologie niet gekend is."},"pt":{"name":"Pneumopatia criptogénica organizada","synonyms":["BOOP","Bronquiolite obliterante-pneumonia organizada","COP"],"definition":"Forma rara de pneumonia intersticial idiopática cuja anatomopatologia é caracterizada por pneumonia em organização (PO) com sintomas inespecíficos semelhantes aos da gripe, além de tosse e dispneia, sem agente etiológico identificado."},"pl":{"name":"Kryptogenne organizujące się zapalenie płuc","synonyms":["Organizujące się zapalenie płuc z zarostowym zapaleniem oskrzelików"]},"cs":{"name":"Kryptogenní organizující se pneumonie","synonyms":["Bronchiolitis obliterans s organizující se pneumonií"]},"uk":{"name":"Криптогенна організуюча пневмонія","synonyms":["BOOP","Облітеруючий бронхіоліт, що організовує пневмонію","COP"],"definition":"A rare form of idiopathic interstitial pneumonia characterized pathologically by organizing pneumonia (OP) that presents with nonspecific flu-like symptoms, as well as cough and dyspnea, and where no etiological agent is found."},"zh":{"name":"隐源性机化性肺炎","synonyms":["闭塞性细支气管炎机化性肺炎"]},"tr":{"name":"Kriptojenik organize pnömoni","synonyms":["COP","BOOP","Bronşiyolitis obliterans organize pnömoni (boop)"]}},"xrefs":[{"source":"ICD-10","code":"J84.1","qualifier":"NTBT","icd_relation":"attributed","validated":true},{"source":"ICD-11","code":"CB03.2","qualifier":"E","icd_relation":"specific","validated":true,"icd11_uri":"1937662238"},{"source":"MONDO","code":"0015264","qualifier":"E","icd_relation":null,"validated":true},{"source":"MeSH","code":"D018549","qualifier":"E","icd_relation":null,"validated":true},{"source":"UMLS","code":"C0242770","qualifier":"E","icd_relation":null,"validated":true},{"source":"GARD","code":"1620","qualifier":"E","icd_relation":null,"validated":true},{"source":"MedDRA","code":"10011496","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":["Not applicable"],"onset":["Adolescent","Adult","Childhood","Elderly"],"prevalence":[{"type":"Point prevalence","qualification":"Class only","class":"Unknown","value":0.0,"area":"Worldwide","status":"Not yet validated","source":"ORPHANET"},{"type":"Point prevalence","qualification":"Value and class","class":"1-9 / 1 000 000","value":0.81,"area":"Spain","status":"Validated","source":"15127977[PMID]"},{"type":"Point prevalence","qualification":"Value and class","class":"1-9 / 1 000 000","value":0.92,"area":"Greece","status":"Validated","source":"19345567[PMID]"},{"type":"Annual incidence","qualification":"Value and class","class":"1-9 / 100 000","value":1.1,"area":"Iceland","status":"Validated","source":"18204109[PMID]"}],"phenotypes":{"validation_status":"y","online":"y","validation_date":"2019-11-06","source":"29939651[PMID]_30322441[PMID]_31159568[PMID]","terms":[{"hpo":"HP:0012735","label":"Cough","frequency":"Very frequent (99-80%)","criteria":null},{"hpo":"HP:0032177","label":"Parenchymal consolidation","frequency":"Very frequent (99-80%)","criteria":null},{"hpo":"HP:0002039","label":"Anorexia","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:0003565","label":"Elevated erythrocyte sedimentation rate","frequency":"Frequent (79-30%)","criteria":null},{"hpo":"HP:0012378","label":"Fatigue","frequency":"Frequent (79-30%)","criteria":null},{"hpo":"HP:0001945","label":"Fever","frequency":"Frequent (79-30%)","criteria":null},{"hpo":"HP:0025179","label":"Ground-glass opacification on pulmonary HRCT","frequency":"Frequent (79-30%)","criteria":null},{"hpo":"HP:0001974","label":"Increased total leukocyte count","frequency":"Frequent (79-30%)","criteria":null},{"hpo":"HP:0011897","label":"Neutrophilia","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:0032016","label":"Abnormal sputum","frequency":"Occasional (29-5%)","criteria":null},{"hpo":"HP:0031994","label":"Bronchial breath sound","frequency":"Occasional (29-5%)","criteria":null},{"hpo":"HP:0100749","label":"Chest pain","frequency":"Occasional (29-5%)","criteria":null},{"hpo":"HP:0000961","label":"Cyanosis","frequency":"Occasional (29-5%)","criteria":null},{"hpo":"HP:0011227","label":"Elevated circulating C-reactive protein concentration","frequency":"Occasional (29-5%)","criteria":null},{"hpo":"HP:0002105","label":"Hemoptysis","frequency":"Occasional (29-5%)","criteria":null},{"hpo":"HP:0001824","label":"Weight loss","frequency":"Occasional (29-5%)","criteria":null},{"hpo":"HP:0030828","label":"Wheezing","frequency":"Occasional (29-5%)","criteria":null},{"hpo":"HP:0002829","label":"Arthralgia","frequency":"Very rare (<4-1%)","criteria":null},{"hpo":"HP:0012418","label":"Hypoxemia","frequency":"Very rare (<4-1%)","criteria":null},{"hpo":"HP:0030166","label":"Night sweats","frequency":"Very rare (<4-1%)","criteria":null},{"hpo":"HP:0025421","label":"Pneumomediastinum","frequency":"Very rare (<4-1%)","criteria":null},{"hpo":"HP:0002107","label":"Pneumothorax","frequency":"Very rare (<4-1%)","criteria":null},{"hpo":"HP:0002098","label":"Respiratory distress","frequency":"Very rare (<4-1%)","criteria":null}]}}