{"orpha":79128,"name":"Lymphoid interstitial pneumonia","type":"Disease","group":"Disorder","status":"active","flags":[],"synonyms":["Lymphocytic interstitial pneumonia"],"definition":"A rare idiopathic interstitial pneumonia characterized by a diffuse, dense, polyclonal lymphoid cell infiltration of the pulmonary interstitium and air spaces, with high prevalence in patients with immune dysregulation. Presenting symptoms are non-specific and include dyspnea and cough. The clinical course is highly variable, ranging from spontaneous resolution to progressive, fatal respiratory failure.","note":null,"url":"https://raremap.alftech.space/d/ORPHA-79128/","orphanet_url":"https://www.orpha.net/en/disease/detail/79128","names":{"fr":{"name":"Pneumopathie interstitielle lymphocytaire","definition":"Pneumonie interstitielle idiopathique rare caractérisée par une infiltration diffuse, dense et polyclonale de cellules lymphoïdes dans l'interstitium pulmonaire et l'air alvéolaire, avec une prévalence élevée chez les patients présentant une dysrégulation immunitaire. Les symptômes non spécifiques sont la dyspnée et la toux. L'évolution clinique est très variable et s'étend de la résolution spontanée à l'insuffisance respiratoire progressive et fatale."},"de":{"name":"Pneumonie, interstitielle, lymphoide","definition":"Eine seltene idiopathische interstitielle Pneumonie, die durch eine diffuse, dichte, polyklonale Lymphoidzellinfiltration des pulmonalen Interstitiums und der Lufträume gekennzeichnet ist, mit hoher Prävalenz bei Patienten mit Immundysregulation. Die anfänglichen Symptome sind unspezifisch und umfassen Dyspnoe und Husten. Der klinische Verlauf ist sehr unterschiedlich und reicht von spontaner Rückbildung bis hin zu fortschreitendem, tödlichem Atemversagen."},"es":{"name":"Neumonía linfoide intersticial","definition":"Es una neumonía intersticial idiopática poco frecuente caracterizada por una infiltración difusa y densa del intersticio pulmonar y los espacios aéreos por células linfoides policlonales, con una elevada prevalencia en pacientes con disregulación inmunitaria. Los síntomas de presentación son inespecíficos e incluyen disnea y tos. El curso clínico es muy variable, desde la resolución espontánea hasta la insuficiencia respiratoria progresiva mortal."},"it":{"name":"Polmonite interstiziale linfoide","definition":"Si tratta di una polmonite interstiziale idiopatica rara, caratterizzata da un'infiltrazione diffusa, densa e policlonale di cellule linfoidi nell'interstizio polmonare e negli spazi aerei. La malattia colpisce maggiormente i pazienti con disregolazione immunitaria. I sintomi d'esordio sono aspecifici e comprendono la dispnea e la tosse. Il decorso clinico è molto variabile e comprende la risoluzione spontanea, fino all'insufficienza respiratoria progressiva e letale."},"nl":{"name":"Lymfoïde interstitiële pneumonie","definition":"Een zeldzame idiopathische interstitiële pneumonie, gekarakteriseerd door diffuse, dense, polyklonale lymfoïde celinfiltratie van pulmonaal interstitium en met lucht gevulde ruimtes, en hoge prevalentie bij patiënten met immuundisregulatie. Presenterende symptomen azijn aspecifiek, waaronder dyspneu en hoest. Het klinische verloop is hoogst variabel, gaande van spontane genezing tot progressief, fataal respiratoir falen."},"pt":{"name":"Pneumonia intersticial linfóide"},"pl":{"name":"Limfatyczne śródmiąższowe zapalenie płuc","synonyms":["Limfocytarne śródmiąższowe zapalenie płuc"]},"cs":{"name":"Lymfoidní intersticiální pneumonie","synonyms":["Lymfatická intersticiální pneumonie"]},"uk":{"name":"Лімфоїдна інтерстиціальна пневмонія","synonyms":["Лімфоцитарна інтерстиціальна пневмонія"],"definition":"A rare idiopathic interstitial pneumonia characterized by a diffuse, dense, polyclonal lymphoid cell infiltration of the pulmonary interstitium and air spaces, with high prevalence in patients with immune dysregulation. Presenting symptoms are non-specific and include dyspnea and cough. The clinical course is highly variable, ranging from spontaneous resolution to progressive, fatal respiratory failure."},"zh":{"name":"淋巴样间质性肺炎","synonyms":["淋巴细胞性间质性肺炎"]},"tr":{"name":"Lenfoid interstisyel pnömoni","synonyms":["Lenfositik interstisyel pnömoni"]}},"xrefs":[{"source":"OMIM","code":"247610","qualifier":"E","icd_relation":null,"validated":true},{"source":"ICD-10","code":"J84.1","qualifier":"NTBT","icd_relation":"attributed","validated":true},{"source":"ICD-11","code":"CB03.5","qualifier":"E","icd_relation":"specific","validated":true,"icd11_uri":"1140418798"},{"source":"MONDO","code":"0009537","qualifier":"E","icd_relation":null,"validated":true},{"source":"MeSH","code":"C562489","qualifier":"E","icd_relation":null,"validated":true},{"source":"UMLS","code":"C0264511","qualifier":"E","icd_relation":null,"validated":true},{"source":"MedDRA","code":"10062997","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":["All ages"],"prevalence":[{"type":"Point prevalence","qualification":"Class only","class":"Unknown","value":0.0,"area":"Worldwide","status":"Not yet validated","source":"ORPHANET"}],"phenotypes":{"validation_status":"y","online":"y","validation_date":"2021-09-29","source":"31615921[PMID]_32040879[PMID]_33364263[PMID]","terms":[{"hpo":"HP:0012735","label":"Cough","frequency":"Very frequent (99-80%)","criteria":null},{"hpo":"HP:0030057","label":"Autoimmune antibody positivity","frequency":"Frequent (79-30%)","criteria":null},{"hpo":"HP:0002960","label":"Autoimmunity","frequency":"Frequent (79-30%)","criteria":null},{"hpo":"HP:0001217","label":"Clubbing","frequency":"Frequent (79-30%)","criteria":null},{"hpo":"HP:0030830","label":"Crackles","frequency":"Frequent (79-30%)","criteria":null},{"hpo":"HP:0045051","label":"Decreased DLCO","frequency":"Frequent (79-30%)","criteria":null},{"hpo":"HP:0002094","label":"Dyspnea","frequency":"Frequent (79-30%)","criteria":null},{"hpo":"HP:0000964","label":"Eczematoid dermatitis","frequency":"Frequent (79-30%)","criteria":null},{"hpo":"HP:0001508","label":"Failure to thrive","frequency":"Frequent (79-30%)","criteria":null},{"hpo":"HP:0012378","label":"Fatigue","frequency":"Frequent (79-30%)","criteria":null},{"hpo":"HP:0025179","label":"Ground-glass opacification on pulmonary HRCT","frequency":"Frequent (79-30%)","criteria":null},{"hpo":"HP:0012418","label":"Hypoxemia","frequency":"Frequent (79-30%)","criteria":null},{"hpo":"HP:0005948","label":"Multiple pulmonary cysts","frequency":"Frequent (79-30%)","criteria":null},{"hpo":"HP:0032177","label":"Parenchymal consolidation","frequency":"Frequent (79-30%)","criteria":null},{"hpo":"HP:0002091","label":"Restrictive ventilatory defect","frequency":"Frequent (79-30%)","criteria":null},{"hpo":"HP:0031691","label":"Severe viral infection","frequency":"Frequent (79-30%)","criteria":null},{"hpo":"HP:0030828","label":"Wheezing","frequency":"Frequent (79-30%)","criteria":null},{"hpo":"HP:0003549","label":"Abnormality of connective tissue","frequency":"Occasional (29-5%)","criteria":null},{"hpo":"HP:0002110","label":"Bronchiectasis","frequency":"Occasional (29-5%)","criteria":null},{"hpo":"HP:0025180","label":"Centrilobular ground-glass opacification on pulmonary HRCT","frequency":"Occasional (29-5%)","criteria":null},{"hpo":"HP:0000105","label":"Enlarged kidney","frequency":"Occasional (29-5%)","criteria":null},{"hpo":"HP:0001945","label":"Fever","frequency":"Occasional (29-5%)","criteria":null},{"hpo":"HP:0002240","label":"Hepatomegaly","frequency":"Occasional (29-5%)","criteria":null},{"hpo":"HP:0002721","label":"Immunodeficiency","frequency":"Occasional (29-5%)","criteria":null},{"hpo":"HP:0001097","label":"Keratoconjunctivitis sicca","frequency":"Occasional (29-5%)","criteria":null},{"hpo":"HP:0100721","label":"Mediastinal lymphadenopathy","frequency":"Occasional (29-5%)","criteria":null},{"hpo":"HP:0030950","label":"Pulmonary venous hypertension","frequency":"Occasional (29-5%)","criteria":null},{"hpo":"HP:0030880","label":"Raynaud phenomenon","frequency":"Occasional (29-5%)","criteria":null},{"hpo":"HP:0011947","label":"Respiratory tract infection","frequency":"Occasional (29-5%)","criteria":null},{"hpo":"HP:0001370","label":"Rheumatoid arthritis","frequency":"Occasional (29-5%)","criteria":null},{"hpo":"HP:0000988","label":"Skin rash","frequency":"Occasional (29-5%)","criteria":null},{"hpo":"HP:0025178","label":"Subpleural interstitial thickening","frequency":"Occasional (29-5%)","criteria":null},{"hpo":"HP:0001824","label":"Weight loss","frequency":"Occasional (29-5%)","criteria":null},{"hpo":"HP:0002206","label":"Pulmonary fibrosis","frequency":"Very rare (<4-1%)","criteria":null}]}}