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Behçet disease (BD) and BD-like clinical phenotypes: NF-kB pathway in mucosal ulcerating diseases.
Scandinavian Journal of Immunology ( IF 3.7 ) Pub Date : 2020-09-05 , DOI: 10.1111/sji.12973
Sandro F Perazzio 1, 2 , Eric J Allenspach 1 , Kari K Eklund 3, 4 , Markku Varjosalo 3, 4, 5 , Michi M Shinohara 6 , Troy R Torgerson 7 , Mikko R J Seppänen 8
Affiliation  

Behçet's disease (BD) is a heterogeneous multi‐organ disorder in search of a unified pathophysiological theory and classification. The disease frequently has overlapping features resembling other disease clusters, such as vasculitides, spondyloarthritides and thrombophilias with similar genetic risk variants, namely HLA‐B*51, ERAP1, IL‐10, IL‐23R. Many of the BD manifestations, such as unprovoked recurrent episodes of inflammation and increased expression of IL‐1, IL‐6 and TNFα, overlap with those of the hereditary monogenic autoinflammatory syndromes, positioning BD at the crossroads between autoimmune and autoinflammatory syndromes. BD‐like disease associates with various inborn errors of immunity, including familial Mediterranean fever, conditions related to dysregulated NF‐κB activation (eg TNFAIP3, NFKB1, OTULIN, RELA, IKBKG) and either constitutional trisomy 8 or acquired trisomy 8 in myelodysplastic syndromes. We review here the recent advances in the immunopathology of BD, BD‐like diseases and the NF‐κB pathway suggesting new elements in the elusive BD etiopathogenesis.

中文翻译:

Behçet疾病(BD)和BD样临床表型:粘膜溃疡性疾病中的NF-kB途径。

白塞病(BD)是一种异质多器官疾病,旨在寻求统一的病理生理学理论和分类。该疾病经常具有与其他疾病群相似的重叠特征,例如具有相似遗传风险变异的血管炎,海绵状关节炎和血友病,即HLA-B * 51ERAP1IL-10IL-23R。BD的许多表现,如无缘无故的复发性炎症发作以及IL-1,IL-6和TNFα的表达增加,都与遗传性单基因自身炎性综合征的表现重叠,从而使BD处于自身免疫和自身炎性综合征之间的十字路口。BD样疾病与各种先天性免疫错误有关,包括家族性地中海热,与NF-κB活化失调有关的疾病(例如TNFAIP3NFKB1OTULINRELAIKBKG))以及骨髓增生异常综合症中的8号三体性或8号三体性。我们在这里回顾了BD,BD样疾病和NF-κB通路的免疫病理学的最新进展,这提示了难以捉摸的BD发病机制中的新元素。
更新日期:2020-10-30
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