{"orpha":494428,"name":"Idiopathic pleuroparenchymal fibroelastosis","type":"Disease","group":"Disorder","status":"active","flags":[],"synonyms":["IPPFE","Idiopathic pleuropulmonary fibroelastosis"],"definition":"A rare idiopathic interstitial pneumonia characterized by prominent subpleural and parenchymal fibroelastosis and pleural fibrosis, predominantly involving the upper lobes. Signs and symptoms include non-productive cough, dyspnea, and recurrent respiratory infections. Pneumothorax is a frequently reported complication. Pulmonary function test reveals a restrictive pattern and reduced diffusing capacity. Computed tomography shows pleural thickening with signs of fibrosis (traction bronchiectasis, architectural distortion, and loss of volume), and reticulation.","note":null,"url":"https://raremap.alftech.space/d/ORPHA-494428/","orphanet_url":"https://www.orpha.net/en/disease/detail/494428","names":{"fr":{"name":"Fibroélastose pleuroparenchymateuse idiopathique","synonyms":["Fibroélastose pleuropulmonaire idiopathique","IPPFE"],"definition":"Pneumonie interstitielle idiopathique rare caractérisée par une importante fibroélastose sous-pleurale et parenchymateuse et une fibrose pleurale, affectant principalement les lobes supérieurs. Les signes cliniques et symptômes sont une toux non productive, une dyspnée et des infections respiratoires récurrentes. Le pneumothorax est une complication fréquemment rapportée. L'exploration fonctionnelle respiratoire révèle un trouble restrictif et réduction de la capacité de diffusion. La tomodensitométrie montre un épaississement pleural avec des signes de fibrose (bronchectasie de traction, distorsion architecturale et perte de volume) ainsi qu'une réticulation."},"de":{"name":"Pleuroparenchymale Fibroelastose, idiopathische","synonyms":["Fibroelastose, idiopathische pleuropulmonare","IPPFE"],"definition":"Eine seltene idiopathische interstitielle Lungenentzündung, die durch eine ausgeprägte subpleurale und parenchymale Fibroelastose und Pleurafibrose gekennzeichnet ist und vorwiegend die oberen Lungenflügel betrifft. Zu den Anzeichen und Symptomen gehören unproduktiver Husten, Dyspnoe und wiederkehrende Atemwegsinfektionen. Ein Pneumothorax ist eine häufig berichtete Komplikation. Der Lungenfunktionstest zeigt ein restriktives Muster und eine verminderte Diffusionskapazität. Die Computertomographie zeigt eine Pleuraverdickung mit Anzeichen einer Fibrose (Traktionsbronchiektasie, architektonische Verzerrung und Volumenverlust) sowie eine Netzbildung."},"es":{"name":"Fibroelastosis pleuroparenquimatosa idiopática","synonyms":["FEPPI","Fibroelastosis pleuropulmonar idiopática"],"definition":"Es una neumonía intersticial idiopática poco frecuente caracterizada por fibroelastosis subpleural y parenquimatosa importante con fibrosis pleural, que afecta predominantemente a los lóbulos superiores. Los signos y síntomas incluyen tos no productiva, disnea e infecciones respiratorias recurrentes. El neumotórax es una complicación frecuentemente descrita. Los estudios de la función pulmonar revelan un patrón restrictivo y una capacidad de difusión reducida. La tomografía computarizada muestra engrosamiento pleural con signos de fibrosis (bronquiectasias por tracción, distorsión de la arquitectura pulmonar y pérdida de volumen) y reticulación."},"it":{"name":"Fibroelastosi pleuroparenchimale idiopatica","definition":"Si tratta di una polmonite interstiziale idiopatica rara, caratterizzata da marcata fibroelastosi subpleurica e parenchimale, e da fibrosi pleurica, che interessano soprattutto i lobi superiori. I segni clinici comprendono la tosse non produttiva, la dispnea e le infezioni respiratorie ricorrenti. Lo pneumotorace è una complicanza comune. Il test di funzionalità polmonare evidenzia una pneumopatia restrittiva e la riduzione della capacità di diffusione. La tomografia computerizzata mostra un ispessimento della pleura, una fibrosi (bronchiectasie da trazione, distorsione dell'architettura e perdita di volume) e un aspetto reticolato."},"nl":{"name":"Idiopathische pleuroparenchymale fibro-elastose","synonyms":["IPPFE"],"definition":"Een zeldzame idiopathische interstitiële pneumonie, gekarakteriseerd door prominente subpleurale en parenchymale fibro-elastose en pleurale fibrose, vooral met betrokkenheid van bovenste kwabben. Verschijnselen en symptomen zijn onder meer niet-productieve hoest, dyspneu, en recidiverende respiratoire infecties. Klaplong is een vaak gerapporteerde complicatie. Onderzoek van longfunctie toont een restrictief patroon en gereduceerde diffusiecapaciteit. Computertomografie toont verdikking van pleura met verschijnselen van fibrose (bronchiëctasie door tractie, deformiteit in structuur, en verlies van volume), en reticulatie."},"pt":{"name":"Fibroelastose pleuroparenquimatosa idiopática"},"pl":{"name":"Samoistna fibroelastoza opłucnowo-śródmiąższowa","synonyms":["Idiopatyczna fibroelastoza opłucnej i miąższu płuca"]},"cs":{"name":"Idiopatická pleuroparenchymální fibroelastóza","synonyms":["IPPFE"]},"uk":{"name":"Ідіопатичний плевропаренхіматозний фіброеластоз","synonyms":["Ідіопатичний плевропульмональний фіброеластоз","IPPFE"],"definition":"A rare idiopathic interstitial pneumonia characterized by prominent subpleural and parenchymal fibroelastosis and pleural fibrosis, predominantly involving the upper lobes. Signs and symptoms include non-productive cough, dyspnea, and recurrent respiratory infections. Pneumothorax is a frequently reported complication. Pulmonary function test reveals a restrictive pattern and reduced diffusing capacity. Computed tomography shows pleural thickening with signs of fibrosis (traction bronchiectasis, architectural distortion, and loss of volume), and reticulation."},"zh":{"name":"特发性胸膜实质纤维弹性组织增生","synonyms":["Idiopathic pleuropulmonary fibroelastosis"]},"tr":{"name":"İdiyopatik plöroparenkimal fibroelastoz","synonyms":["IPPFE","İdiyopatik plöropulmoner fibroelastoz"]}},"xrefs":[{"source":"ICD-10","code":"J84.1","qualifier":"NTBT","icd_relation":"attributed","validated":true},{"source":"ICD-11","code":"CB03.Y","qualifier":"NTBT","icd_relation":"attributed","validated":true},{"source":"MONDO","code":"0044633","qualifier":"E","icd_relation":null,"validated":true},{"source":"UMLS","code":"C5567467","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","Elderly"],"prevalence":[{"type":"Point prevalence","qualification":"Class only","class":"<1 / 1 000 000","value":0.0,"area":"Worldwide","status":"Validated","source":"ORPHANET_27141432[PMID]_28344924[PMID]"},{"type":"Cases/families","qualification":"Case(s)","class":null,"value":37.0,"area":"Worldwide","status":"Validated","source":"27141432[PMID]"}],"phenotypes":null}