{"orpha":420259,"name":"Secondary pulmonary alveolar proteinosis","type":"Disease","group":"Disorder","status":"active","flags":[],"synonyms":["Secondary PAP"],"definition":"A rare, acquired, interstitial lung disease, characterized by alveolar surfactant accumulation, cough, progressive dyspnea and respiratory insufficiency. The disease may be secondary to hematological disorder, toxic inhalation, and infection or may occur within the setting of immunosuppression after transplantation.","note":null,"url":"https://raremap.alftech.space/d/ORPHA-420259/","orphanet_url":"https://www.orpha.net/en/disease/detail/420259","names":{"fr":{"name":"Protéinose alvéolaire pulmonaire secondaire","synonyms":["PAP secondaire"],"definition":"Pneumopathie interstitielle rare acquise caractérisée par une accumulation de surfactant alvéolaire, une toux, une dyspnée progressive et une insuffisance respiratoire. La maladie peut se développer après une maladie hématologique, une inhalation toxique ou une infection, mais elle peut aussi survenir sur un terrain d'immunosuppression après une transplantation."},"de":{"name":"Pulmonale Alveolarproteinose, sekundäre","synonyms":["Sekundäre PAP"],"definition":"Eine seltene, erworbene interstitielle Lungenerkrankung, die durch alveoläre Surfactant-Ansammlung, Husten, fortschreitende Dyspnoe und respiratorische Insuffizienz gekennzeichnet ist. Die Erkrankung kann sekundär zu hämatologischen Störungen, toxischer Inhalation und Infektionen auftreten oder im Rahmen einer Immunsuppression nach einer Transplantation."},"es":{"name":"Proteinosis alveolar pulmonar secundaria","synonyms":["PAP secundaria"],"definition":"Es una enfermedad pulmonar intersticial adquirida y poco frecuente caracterizada por un acúmulo de surfactante en los alveolos, tos, disnea progresiva e insuficiencia respiratoria. La enfermedad puede ser secundaria a un trastorno hematológico, inhalación tóxica e infección, o puede ocurrir en situaciones de inmunosupresión a consecuencia de un trasplante."},"it":{"name":"Proteinosi alveolare polmonare secondaria","synonyms":["PAP secondaria"],"definition":"È una pneumopatia interstiziale acquisita rara, caratterizzata dall'accumulo di surfattante negli alveoli, tosse, dispnea progressiva e insufficienza respiratoria. La malattia può essere secondaria a una emopatia, all'inalazione di sostanze tossiche o a un'infezione, oppure può insorgere nel quadro dell'immunosoppressione dopo un trapianto."},"nl":{"name":"Secundaire pulmonale alveolaire proteïnose","synonyms":["Secundaire PAP"],"definition":"Een zeldzame, verworven, interstitiële longziekte gekarakteriseerd door accumulatie van alveolair surfactant, hoest, progressieve dyspneu en respiratoire insufficiëntie. De ziekte kan secundair zijn aan een hematologische stoornis, inademing van een giftige stof, en infectie, of kan voorkomen in de context van immuunsuppressie na transplantatie."},"pt":{"name":"Proteinose pulmonar alveolar secundária"},"pl":{"name":"Wtórna proteinoza pęcherzyków płucnych","synonyms":["Wtórna PAP"]},"cs":{"name":"Sekundární plicní alveolární proteinóza","synonyms":["Sekundární PAP"]},"uk":{"name":"Вторинний альвеолярний протеїноз легень","synonyms":["Вторинний ПАП"],"definition":"A rare, acquired, interstitial lung disease, characterized by alveolar surfactant accumulation, cough, progressive dyspnea and respiratory insufficiency. The disease may be secondary to hematological disorder, toxic inhalation, and infection or may occur within the setting of immunosuppression after transplantation."},"zh":{"name":"继发性肺泡蛋白沉着症","synonyms":["继发型PAP"],"definition":"是一种罕见的获得性间质性肺疾病，其特征为肺泡表面活性剂积聚，咳嗽，进行性呼吸困难和呼吸功能不全。该疾病可能继发于血液系统疾病，吸入毒性物质和感染，也可发生在移植后免疫抑制的状态下发生。"},"tr":{"name":"Sekonder pulmoner alveolar proteinoz","synonyms":["Sekonder PAP"]}},"xrefs":[{"source":"ICD-10","code":"J84.0","qualifier":"NTBT","icd_relation":"attributed","validated":true},{"source":"ICD-11","code":"CB04.31","qualifier":"NTBT","icd_relation":"index","validated":true,"icd11_uri":"1480338606"},{"source":"MONDO","code":"0018483","qualifier":"E","icd_relation":null,"validated":true},{"source":"UMLS","code":"C3873302","qualifier":"E","icd_relation":null,"validated":true}],"associations":[],"incoming_associations":[],"preferential_parent":{"orpha":97955,"name":"Rare respiratory disease"},"parents":[{"orpha":264944,"name":"Secondary interstitial lung disease in childhood and adulthood","classifications":[184,233]}],"children":[],"genes":[],"genes_via_subtypes":[],"inheritance":["Not applicable"],"onset":[],"prevalence":[],"phenotypes":null}