{"orpha":63999,"name":"IgG4-related mediastinitis","type":"Clinical subtype","group":"Subtype of disorder","status":"active","flags":[],"synonyms":["Fibrosing mediastinitis","Mediastinal fibrosis","Sclerosing mediastinitis"],"definition":"A rare systemic autoimmune disease characterized by an aggressive fibroinflammatory process with infiltration of IgG4-positive plasma cells in the mediastinum, potentially resulting in compression and functional impairment of vital mediastinal structures, and associated with elevated serum IgG4. Clinical symptoms are unspecific and include pain or symptoms due to mass effect. The condition may occur together with IgG4-related disease in other parts of the body.","note":null,"url":"https://raremap.alftech.space/d/ORPHA-63999/","orphanet_url":"https://www.orpha.net/en/disease/detail/63999","names":{"fr":{"name":"Médiastinite associée à IgG4","synonyms":["Fibrose médiastinale","Médiastinite fibreuse","Médiastinite sclérosante"],"definition":"Maladie systémique auto-immune rare caractérisée par un processus fibro-inflammatoire agressif avec infiltration de plasmocytes IgG4-positifs dans le médiastin, pouvant causer une compression et une altération fonctionnelle des structures médiastinales vitales, et associée à un taux sérique élevé d'IgG4. Parmi les signes cliniques, qui sont peu spécifiques, figurent des douleurs ou des symptômes dus à un effet de masse. La maladie peut être associée à une maladie liée aux IgG4 affectant d'autres parties du corps."},"de":{"name":"Mediastinitis, IgG4-assoziierte","synonyms":["Mediastinalfibrose","Mediastinitis, fibrosierende","Mediastinitis, sklerosierende"],"definition":"Eine seltene systemische Autoimmunerkrankung, die durch einen aggressiven fibroinflammatorischen Prozess mit Infiltration von IgG4-positiven Plasmazellen im Mediastinum gekennzeichnet ist, was zu einer Kompression und funktionellen Beeinträchtigung lebenswichtiger mediastinaler Strukturen führen kann und mit erhöhtem Serum-IgG4 einhergeht. Die klinischen Symptome sind unspezifisch und umfassen Schmerzen oder Symptome aufgrund der Gewebeverdichtung. Die Erkrankung kann zusammen mit IgG4-assoziierten Erkrankungen in anderen Teilen des Körpers auftreten."},"es":{"name":"Mediastinitis asociada a IgG4","synonyms":["Fibrosis mediastínica","Mediastinitis esclerosante","Mediastinitis fibrosante"],"definition":"Es una enfermedad autoinmune sistémica poco frecuente caracterizada por un proceso fibroinflamatorio agresivo con infiltración de células plasmáticas positivas para IgG4 en el mediastino, que puede resultar en compresión y disfunción de estructuras mediastínicas vitales, y que se asocia con niveles elevados de IgG4 sérica. Los síntomas clínicos son inespecíficos e incluyen dolor o síntomas debidos al efecto de masa. La entidad puede ocurrir junto con una enfermedad relacionada con IgG4 en otras localizaciones anatómicas."},"it":{"name":"Mediastinite correlata alle IgG4","synonyms":["Fibrosi mediastinica","Mediastinite fibrosante","Mediastinite sclerosante"],"definition":"Si tratta di una malattia autoimmune sistemica rara, caratterizzata da un processo fibroinfiammatorio aggressivo con infiltrazione di plasmacellule IgG4-positive nel mediastino, che può causare la compressione e la compromissione funzionale delle strutture vitali del mediastino. Si associa a livelli elevati di IgG4 nel siero. I segni clinici sono aspecifici e comprendono il dolore e i sintomi secondari alla presenza di una massa. La malattia può manifestarsi in concomitanza con la malattia correlata alle IgG4 in altre aree del corpo."},"nl":{"name":"IgG4-gerelateerde mediastinitis","synonyms":["Fibroserende mediastinitis","Mediastinale fibrose","Scleroserende mediastinitis"],"definition":"Een zeldzame systemische auto-immuunziekte, gekarakteriseerd door een agressief fibro-inflammatoir proces met infiltratie van IgG4-positieve plasmacellen in mediastinum, wat potentieel resulteert in compressie en functiestoornis van vitale mediastinale structuren, en geassocieerd met verhoogd IgG4-gehalte in serum. Klinische symptomen zijn aspecifiek en omvatten onder meer pijn of symptomen door massa-effect. De aandoening kan samen voorkomen met IgG4-gerelateerde ziekte in andere delen van het lichaam."},"pt":{"name":"Mediastinite IgG4-relacionada","synonyms":["Fibrose mediastínica","Mediastinite esclerosante","Mediastinite fibrosante"]},"pl":{"name":"Włóknienie śródpiersia","synonyms":["Stwardnienie śródpiersia"]},"cs":{"name":"IgG4 asociovaná mediastinitida","synonyms":["Fibrotizující mediastinitida","Mediastinální fibróza","Sklerotizující mediastinitida"]},"uk":{"name":"Медіастиніт, повязаний з IgG4","synonyms":["Фіброзуючий медіастиніт","Фіброз середостіння","Склерозуючий медіастиніт"],"definition":"A rare systemic autoimmune disease characterized by an aggressive fibroinflammatory process with infiltration of IgG4-positive plasma cells in the mediastinum, potentially resulting in compression and functional impairment of vital mediastinal structures, and associated with elevated serum IgG4. Clinical symptoms are unspecific and include pain or symptoms due to mass effect. The condition may occur together with IgG4-related disease in other parts of the body."},"zh":{"name":"IgG4相关性纵隔炎","synonyms":["硬化性纵隔炎"]},"tr":{"name":"IgG4-ilişkili mediastinit","synonyms":["Mediastinal fibroz","Fibrozan mediastinit","Sklerozan mediastinit"]}},"xrefs":[{"source":"ICD-10","code":"J98.5","qualifier":"NTBT","icd_relation":"inclusion","validated":true},{"source":"ICD-11","code":"CB22.0","qualifier":"E","icd_relation":"specific","validated":true,"icd11_uri":"123840075"},{"source":"MONDO","code":"0018978","qualifier":"E","icd_relation":null,"validated":true},{"source":"MeSH","code":"C536136","qualifier":"E","icd_relation":null,"validated":true},{"source":"UMLS","code":"C0264573","qualifier":"E","icd_relation":null,"validated":true},{"source":"GARD","code":"8337","qualifier":"E","icd_relation":null,"validated":true},{"source":"MedDRA","code":"10027074","qualifier":"E","icd_relation":null,"validated":true}],"associations":[],"incoming_associations":[],"preferential_parent":{"orpha":97955,"name":"Rare respiratory disease"},"parents":[{"orpha":596448,"name":"IgG4-related systemic disease","classifications":[196]},{"orpha":97955,"name":"Rare respiratory disease","classifications":[184]}],"children":[],"genes":[],"genes_via_subtypes":[],"inheritance":["Not applicable"],"onset":[],"prevalence":[],"phenotypes":null}