Emmi L, Brugnolo F, Marchione T
Istituto di Allergologia e Immunologia Clinica, Università degli Studi di Firenze.
Ann Ital Med Int. 1997 Jan-Mar;12(1):20-5.
Behçet's disease is a relapsing-remitting systemic vasculitis characterized by oral and genital ulcers, uveitis and thrombophlebitis which can involve many organs. Although its pathogenesis is not fully understood, a possible pathogenetic model can be proposed on the basis of recent studies. Genetic factors, in particular, have been investigated and the role of the genes encoding tumor necrosis factor, transporter in antigen processing proteins and MIC (MHC class I chain related) has been emphasized. In addition, a possible polarization of T lymphocytes towards the Th1 phenotype in Behçet's disease has been suggested by recent observations in experimental uveoretinitis and by preliminary data in humans. Neutrophils may also play a role in the pathogenesis of this disease, as they are attracted by macrophage-released cytokines at the site of the lesions, and thus contribute to tissue damage and self-maintenance of inflammation. New strategies for the treatment of Behçet's disease are being devised. In particular, immunosuppressive drugs used in association or in sequence may be administered to patients with particular clinical features or very severe disease.
白塞病是一种复发缓解型全身性血管炎，其特征为口腔溃疡、生殖器溃疡、葡萄膜炎和血栓性静脉炎，可累及多个器官。尽管其发病机制尚未完全明确，但基于近期研究可提出一种可能的发病模型。尤其是遗传因素已得到研究，编码肿瘤坏死因子、抗原加工转运蛋白和MIC（MHC I类链相关分子）的基因的作用已得到强调。此外，实验性葡萄膜炎的近期观察结果及人类的初步数据表明，白塞病中T淋巴细胞可能向Th1表型极化。中性粒细胞在该病发病机制中也可能起作用，因为它们在病变部位被巨噬细胞释放的细胞因子吸引，从而导致组织损伤并维持炎症。正在设计治疗白塞病的新策略。特别是，对于具有特定临床特征或病情非常严重的患者，可联合或序贯使用免疫抑制药物。
