{"orpha":264691,"name":"Isolated pulmonary capillaritis","type":"Disease","group":"Disorder","status":"active","flags":[],"synonyms":[],"definition":"Isolated pauciimmune pulmonary capillaritis is a small vessel vasculitis restricted to the lungs that may induce diffuse alveolar hemorrhage with dyspnea, anemia, chest pain, hemoptysis, bilateral and diffuse alveolar infiltrates at chest X-rays, without any underlying systemic disease. ANCA are frequently positive but could be negative.","note":null,"url":"https://raremap.alftech.space/d/ORPHA-264691/","orphanet_url":"https://www.orpha.net/en/disease/detail/264691","names":{"fr":{"name":"Capillarite pulmonaire isolée","definition":"Vascularite des petits vaisseaux limitée aux poumons. Elle peut entraîner une hémorragie alvéolaire diffuse accompagnée de dyspnée, d'anémie, de douleurs thoraciques et d'hémoptysie. La radiographie thoracique met en évidence des infiltrats alvéolaires bilatéraux et diffus en l'absence de maladie systémique sous-jacente. Les ANCA sont souvent positifs, mais peuvent également être négatifs."},"de":{"name":"Lungen-Kapillaritis, isolierte","definition":"Die Isolierte pauciimmune Lungen-Kapillaritis ist eine auf die Lunge beschränkte Vaskulitis der kleinen Gefäße. Mögliche Folgen sind diffuse Einblutungen in die Alveolen mit Dyspnoe, Anämie, Thoraxschmerzen und Hämoptyse. Im Röntgenbild des Thorax zeigen sich diffuse alveoläre Infiltrate. Eine primär zugrundeliegende systemische Krankheit besteht nicht. ANCA sind häufig positiv, können aber auch negativ sein."},"es":{"name":"Capilaritis pulmonar aislada","definition":"La capilaritis pulmonar pauciinmunitaria aislada es una vasculitis de vasos pequeños restringida a los pulmones que puede inducir una hemorragia alveolar difusa con disnea, anemia, dolor torácico, hemoptisis, infiltrados alveolares bilaterales y difusos en las radiografías torácicas, sin ninguna enfermedad sistémica subyacente. El análisis de ANCA es frecuentemente positivo pero puede ser negativo."},"it":{"name":"Capillarite polmonare isolata","definition":"La capillarite polmonare pauci-immune isolata è una vasculite dei piccoli vasi limitata ai polmoni, che può provocare una diffusa emorragia alveolare con dispnea, anemia, dolore toracico, emottisi, infiltrati alveolari diffusi e bilaterali visibili dalla radiografia del torace, in assenza di una malattia sistemica di base. Gli ANCA sono spesso positivi, ma possono anche essere negativi."},"nl":{"name":"Geïsoleerde pulmonale capillaritis","definition":"Een vasculitis van kleine bloedvaten beperkt tot long, die kan leiden tot diffuse alveolaire hemorragie met dyspneu, anemie, pijn op borst, hemoptoë, en bilaterale en diffuse alveolaire infiltraten op röntgenopname van thorax, zonder enige onderliggende systemische ziekte. Analyse van anti-neutrofiele cytoplasmatische antilichamen (ANCA) is vaak positief, maar kan ook negatief zijn."},"pt":{"name":"Capilarite pulmonar isolada"},"pl":{"name":"Izolowane zapalenie naczyń włosowatych w płucach"},"cs":{"name":"Izolovaná plicní kapilaritida"},"uk":{"name":"Ізольований пульмонарний капілярит","definition":"Isolated pauciimmune pulmonary capillaritis is a small vessel vasculitis restricted to the lungs that may induce diffuse alveolar hemorrhage with dyspnea, anemia, chest pain, hemoptysis, bilateral and diffuse alveolar infiltrates at chest X-rays, without any underlying systemic disease. ANCA are frequently positive but could be negative."},"zh":{"name":"孤立性肺毛细血管炎","definition":"孤立性低免疫性肺毛细血管炎是一种局限于肺部的小血管炎，可引起弥漫性肺泡出血，伴有呼吸困难、贫血、胸痛、咯血和双侧胸部X线片上的弥漫性肺泡浸润，没有任何潜在的全身性疾病。ANCA通常是阳性的，但也可能是阴性的。"},"tr":{"name":"İzole pulmoner kapillerit"}},"xrefs":[{"source":"ICD-10","code":"J84.0","qualifier":"NTBT","icd_relation":"attributed","validated":true},{"source":"ICD-11","code":"CB04.4","qualifier":"NTBT","icd_relation":"index","validated":true,"icd11_uri":"1607123556"},{"source":"MONDO","code":"0017018","qualifier":"E","icd_relation":null,"validated":true},{"source":"UMLS","code":"C3873357","qualifier":"E","icd_relation":null,"validated":true}],"associations":[],"incoming_associations":[],"preferential_parent":{"orpha":97955,"name":"Rare respiratory disease"},"parents":[{"orpha":264683,"name":"Primary interstitial lung disease specific to childhood due to alveolar vascular disorder","classifications":[184,233]}],"children":[],"genes":[],"genes_via_subtypes":[],"inheritance":["Not applicable"],"onset":["Childhood"],"prevalence":[{"type":"Point prevalence","qualification":"Class only","class":"Unknown","value":0.0,"area":"Worldwide","status":"Not yet validated","source":"ORPHANET"}],"phenotypes":null}