{"orpha":99930,"name":"Secondary pulmonary hemosiderosis","type":"Disease","group":"Disorder","status":"active","flags":[],"synonyms":[],"definition":"Secondary pulmonary hemosiderosis is a respiratory disease due to the deposition of hemosiderin-laden macrophages in lungs as a result of repeated alveolar hemorrhage secondary to another disease, especially dysimmunitary disorders (i.e. Heiner syndrome, autoimmune diseases), thrombotic disorders and cardiovascular disorders such as mitral stenosis. It manifests as a triad of hemoptysis, anemia and diffuse parenchymal infiltrates on chest radiography","note":null,"url":"https://raremap.alftech.space/d/ORPHA-99930/","orphanet_url":"https://www.orpha.net/en/disease/detail/99930","names":{"fr":{"name":"Hémosidérose pulmonaire secondaire","definition":"L'hémosidérose pulmonaire secondaire est une maladie respiratoire due au dépôt d'hémosidérine dans les macrophages pulmonaires secondaire à une hémorragie alvéolaire répétée en relation à une maladie sous-jacente, notamment des troubles immunitaires (i.e. le syndrome de Heiner , et des maladies auto-immunes), des troubles thrombotiques et cardiovasculaires (i.e. sténose mitrale). Elle se manifeste par une triade d'hémoptysie, une anémie et une infiltration parenchymateuse diffuse révélées par une radiographie du thorax."},"de":{"name":"Lungen-Hämosiderose, sekundäre","definition":"Die Sekundäre Lungen-Hämosiderose ist eine Atemwegserkrankung durch Ablagerung hämosiderinbeladener Makrophagen in den Lungen nach wiederholten Einblutungen in die Alveolen. Diese Einblutungen sind durch andere Krankheiten verursacht, besonders Immunitätsstörungen (Heiner-Syndrom (s. dort), Autoimmun-Krankheiten), thrombotische Krankheiten und kardiovaskuläre Erkrankungen, z.B. Mitralstenose. Die Krankheit wird mit der Symptomentrias Bluthusten, Anämie und diffuse parenchymatöse Lungeninfiltrate im Röntgenthorax manifest."},"es":{"name":"Hemosiderosis pulmonar secundaria","definition":"La hemosiderosis pulmonar secundaria es una enfermedad respiratoria debida a un depósito de macrófagos cargados de hemosiderina en los pulmones como resultado de una repetida hemorragia alveolar secundaria a otra enfermedad, especialmente enfermedades disinmunitarias (p.e. síndrome de Heiner (consulte este término), enfermedades autoinmunes), trastornos trombóticos y trastornos cardiovasculares como la estenosis mitral. Se manifiesta como una triada de hemoptisis, anemia e infiltrados parenquimales difusos en la radiografía torácica."},"it":{"name":"Emosiderosi polmonare secondaria","definition":"L'emosiderosi polmonare secondaria è una malattia respiratoria da deposito di macrofagi carichi di emosiderina nei polmoni, successivamente ad un'emorragia alveolare ricorrente correlata ad un'altra malattia, in particolare le patologie immunitarie (come la sindrome di Heiner [si veda questo termine], le malattie autoimmuni), le trombosi e i disturbi cardiovascolari, come la stenosi mitralica. Si manifesta con la triade costituita da emottisi, anemia, infiltrati parenchimali diffusi visibili sulle radiografie del torace."},"nl":{"name":"Secundaire pulmonale hemosiderose","definition":"Een respiratoire aandoening, te wijten aan depositie van met hemosiderine gestapelde macrofagen in long als gevolg van herhaaldelijke alveolaire hemorragie secundair aan een andere ziekte, vooral immuunstoornissen (zoals syndroom van Heiner, auto-immuunziekten), trombotische aandoeningen, en cardiovasculaire aandoeningen zoals mitralisklepstenose. Secundaire pulmonale hemosiderose manifesteert zich als een triade van hemoptoë, anemie, en diffuse parenchymale infiltraten bij radiografie van thorax."},"pt":{"name":"Hemossiderose pulmonar, secundária"},"pl":{"name":"Wtórna hemosyderoza płuc"},"cs":{"name":"Sekundární plicní hemosideróza"},"uk":{"name":"Вторинний гемосидероз легень","definition":"Secondary pulmonary hemosiderosis is a respiratory disease due to the deposition of hemosiderin-laden macrophages in lungs as a result of repeated alveolar hemorrhage secondary to another disease, especially dysimmunitary disorders (i.e. Heiner syndrome (see this term), autoimmune diseases), thrombotic disorders and cardiovascular disorders such as mitral stenosis. It manifests as a triad of hemoptysis, anemia and diffuse parenchymal infiltrates on chest radiography"},"zh":{"name":"继发性肺含铁血黄素沉积","definition":"继发性肺含铁血黄素沉着症是由于继发于其他疾病的反复肺泡出血导致肺内负载含铁血黄素的巨噬细胞沉积所致的呼吸系统疾病，尤其是免疫功能紊乱(即海纳综合征（见该词条）、自身免疫性疾病)、血栓性疾病和二尖瓣狭窄等心血管疾病。胸部X线表现为咯血、贫血和弥漫性实质浸润三联征。"},"tr":{"name":"Sekonder pulmoner hemosideroz"}},"xrefs":[{"source":"ICD-10","code":"E83.1+","qualifier":"NTBT","icd_relation":"attributed","validated":true},{"source":"ICD-10","code":"J99.8*","qualifier":"NTBT","icd_relation":"attributed","validated":true},{"source":"ICD-11","code":"CB05.5","qualifier":"E","icd_relation":"specific","validated":true,"icd11_uri":"878618614"},{"source":"MONDO","code":"0020553","qualifier":"E","icd_relation":null,"validated":true},{"source":"UMLS","code":"C4274326","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":[],"onset":[],"prevalence":[],"phenotypes":null}