{"orpha":91364,"name":"Non-specific interstitial pneumonia","type":"Disease","group":"Disorder","status":"active","flags":[],"synonyms":["NSIP","Non-specific idiopathic interstitial pneumonia"],"definition":"A rare idiopathic interstitial pneumonia characterized by temporally uniform alveolar and interstitial mononuclear cell inflammation (cellular type) and/or fibrosis of the alveolar walls (fibrotic type) with preserved alveolar architecture. Other types of interstitial lung disease must be excluded. Symptoms are non-specific and include dyspnea, cough, and often constitutional symptoms such as fever and fatigue. Pulmonary function test reveals a restrictive pattern. Computed tomography shows predominantly lower lobe subpleural reticular changes, traction bronchiectasis, and ground-glass opacities. The cellular type of the disease is less common but carries a better prognosis.","note":null,"url":"https://raremap.alftech.space/d/ORPHA-91364/","orphanet_url":"https://www.orpha.net/en/disease/detail/91364","names":{"fr":{"name":"Pneumonie interstitielle non spécifique","synonyms":["NSIP","Pneumonie interstitielle idiopathique non spécifique"],"definition":"Pneumonie interstitielle idiopathique rare caractérisée par une inflammation des cellules mononucléaires alvéolaires et interstitielles (type cellulaire) et/ou une fibrose des parois alvéolaires (type fibrotique) uniformes, avec une architecture alvéolaire préservée. D'autres types de maladies pulmonaires interstitielles doivent être écartées. Les symptômes sont non spécifiques et comprennent la dyspnée, la toux et souvent des symptômes constitutionnels tels que la fièvre et la fatigue. Le test de la fonction pulmonaire révèle un trouble restrictif. La tomodensitométrie révèle principalement des changements réticulaires sous-pleuraux du lobe inférieur, des bronchectasies par traction et des opacités en verre dépoli. Le type cellulaire de la maladie est moins fréquent mais son pronostic est meilleur."},"de":{"name":"Pneumonie, interstitielle, unspezifische","synonyms":["NSIP","Pneumonie, interstitielle, idiopathische, unspezifische"],"definition":"Eine idiopathische interstitielle Pneumonie, die durch eine zeitlich einheitliche Entzündung der alveolären und interstitiellen mononukleären Zellen (zellulärer Typ) und/oder eine Fibrose der Alveolarwände (fibrotischer Typ) bei erhaltener Alveolararchitektur gekennzeichnet ist. Andere Arten der interstitiellen Lungenerkrankung müssen ausgeschlossen werden. Die Symptome sind unspezifisch und umfassen Dyspnoe, Husten und häufig konstitutionelle Symptome wie Fieber und Müdigkeit. Der Lungenfunktionstest zeigt ein restriktives Muster. Die Computertomographie zeigt vorwiegend subpleurale retikuläre Veränderungen im Unterlappen, Traktionsbronchiektasien und Milchglastrübungen. Die zelluläre Form der Erkrankung ist weniger häufig, hat aber eine bessere Prognose."},"es":{"name":"Neumonía intersticial no específica","synonyms":["NSIP","Neumonía intersticial no específica idiopática"],"definition":"Es una neumonía intersticial idiopática poco frecuente caracterizada por inflamación (de tipo celular) temporal y uniforme de las células mononucleares intersticiales y alveolares y/o fibrosis de las paredes alveolares (tipo fibrótico) con arquitectura alveolar conservada. Deben excluirse otros tipos de enfermedad pulmonar intersticial. Los síntomas son inespecíficos e incluyen disnea, tos y, a menudo, síntomas constitucionales como fiebre y cansancio. Los estudios de la función pulmonar revelan un patrón restrictivo. La tomografía computarizada muestra alteraciones reticulares subpleurales, predominantemente del lóbulo inferior, bronquiectasias por tracción y opacidades en vidrio esmerilado. El tipo celular de la enfermedad es menos común pero conlleva un mejor pronóstico."},"it":{"name":"Polmonite interstiziale aspecifica","synonyms":["NSIP","Polmonite interstiziale idiopatica non specifica"],"definition":"Si tratta di una polmonite interstiziale idiopatica rara, caratterizzata dall'infiammazione contemporanea delle cellule mononucleate alveolari e interstiziali (tipo cellulare) e/o dalla fibrosi delle pareti degli alveoli (tipo fibrotico), con architettura alveolare conservata. È necessario escludere gli altri tipi di pneumopatia interstiziale. I sintomi sono aspecifici e comprendono la dispnea, la tosse e, spesso, i sintomi costituzionali come la febbre e l'affaticamento. Il test di funzionalità polmonare evidenzia la pneumopatia restrittiva. La tomografia computerizzata mostra per lo più alterazioni reticolari subpleuriche nel lobo inferiore, bronchiectasie da trazione e opacità a vetro smerigliato. Il tipo cellulare della malattia è meno comune e si associa ad una prognosi maggiormente favorevole."},"nl":{"name":"Niet-specifieke interstitiële pneumonie","synonyms":["NSIP","Niet-specifieke idiopathische interstitiële pneumonie"],"definition":"Een zeldzame idiopathische interstitiële pneumonie, gekarakteriseerd door tijdelijke uniforme inflammatie van alveolaire en interstitiële mononucleaire cellen (cellulair type) en/of fibrose van alveolaire wanden (fibrotisch type) met behoud van alveolaire structuur. Andere types van interstitiële longziekte dienen uitgesloten te worden. Symptomen azijn aspecifiek en omvatten onder meer dyspneu, hoest, en vaak constitutionele symptomen zoals koorts en vermoeidheid. Onderzoek van longfunctie toont een restrictief patroon. Computertomografie toont voornamelijk in onderste kwabben subpleurale reticulaire veranderingen, bronchiëctasie door tractie, en matglasopaciteiten. Het cellulaire type van de ziekte komt minder vaak voor maar heeft een betere prognose."},"pt":{"name":"Pneumonia intersticial não-específica"},"pl":{"name":"Niespecyficzne śródmiąższowe zapalenie płuc","synonyms":["Niespecyficzne idiopatyczne śródmiąższowe zapalenie płuc","NSIP"]},"cs":{"name":"Nespecifická intersticiální pneumonie","synonyms":["NSIP","Nespecifická idiopatická intersticiální pneumonie"]},"uk":{"name":"Неспецифічна інтерстиціальна пневмонія","synonyms":["Неспецифічна ідіопатична інтерстиціальна пневмонія","NSIP"],"definition":"A rare idiopathic interstitial pneumonia characterized by temporally uniform alveolar and interstitial mononuclear cell inflammation (cellular type) and/or fibrosis of the alveolar walls (fibrotic type) with preserved alveolar architecture. Other types of interstitial lung disease must be excluded. Symptoms are non-specific and include dyspnea, cough, and often constitutional symptoms such as fever and fatigue. Pulmonary function test reveals a restrictive pattern. Computed tomography shows predominantly lower lobe subpleural reticular changes, traction bronchiectasis, and ground-glass opacities. The cellular type of the disease is less common but carries a better prognosis."},"zh":{"name":"非特异性间质性肺炎","synonyms":["非特异性特发性间质性肺炎"]},"tr":{"name":"Spesifik olmayan interstisyel pnömoni","synonyms":["NSIP","Spesifik olmayan idiyopatik interstisyel pnömoni"]}},"xrefs":[{"source":"ICD-10","code":"J84.1","qualifier":"NTBT","icd_relation":"attributed","validated":true},{"source":"ICD-11","code":"CB03.Y","qualifier":"NTBT","icd_relation":"index","validated":true,"icd11_uri":"1319771917"},{"source":"MONDO","code":"0019622","qualifier":"E","icd_relation":null,"validated":true},{"source":"UMLS","code":"C1290344","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":[],"onset":[],"prevalence":[],"phenotypes":null}