Pemphigus
Pemphigus
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DOI：
10.1038/nrdp.2017.26
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发表时间：
2017-05-11
影响因子：
81.5
通讯作者：
Amagai, Masayuki
中科院分区：
文献类型：
--
作者：
Kasperkiewicz, Michael;Ellebrecht, Christoph T.;Amagai, Masayuki
天疱疮是一组igg介导的层状鳞状上皮自身免疫性疾病，如皮肤和口腔粘膜，其中棘层溶解（细胞粘连丧失）引起水泡和糜烂。天疱疮有三个主要亚型：寻常型天疱疮，叶状天疱疮和副肿瘤天疱疮。IgG自身抗体的特点是针对桥粒1和桥粒3产生，这是在桥粒中发现的细胞-细胞粘附分子。水疱形成的部位可以用抗桥粒蛋白自身抗体谱和桥粒蛋白同种异构体的组织特异性表达模式在生理学上解释。T细胞和B细胞在天疱疮小鼠模型和患者中的病理生理作用已经被表征，揭示了自身免疫机制的见解。诊断依据临床表现，并经组织学和免疫化学检查证实。目前的一线治疗是全身性皮质类固醇和辅助治疗，包括免疫抑制剂、静脉注射免疫球蛋白和血浆置换。利妥昔单抗是一种针对CD20(+) B细胞的单克隆抗体，是一种很有前景的治疗选择，可能很快成为一线治疗方法。天疱疮是最典型的人类自身免疫性疾病之一，为基础和临床研究提供了理想的范例，特别是对自身免疫性疾病抗原特异性免疫抑制治疗的发展。
Pemphigus is a group of IgG-mediated autoimmune diseases of stratified squamous epithelia, such as the skin and oral mucosa, in which acantholysis (the loss of cell adhesion) causes blisters and erosions. Pemphigus has three major subtypes: pemphigus vulgaris, pemphigus foliaceus and paraneoplastic pemphigus. IgG autoantibodies are characteristically raised against desmoglein 1 and desmoglein 3, which are cell-cell adhesion molecules found in desmosomes. The sites of blister formation can be physiologically explained by the anti-desmoglein autoantibody profile and tissue-specific expression pattern of desmoglein isoforms. The pathophysiological roles of T cells and B cells have been characterized in mouse models of pemphigus and patients, revealing insights into the mechanisms of autoimmunity. Diagnosis is based on clinical manifestations and confirmed with histological and immunochemical testing. The current first-line treatment is systemic corticosteroids and adjuvant therapies, including immunosuppressive agents, intravenous immunoglobulin and plasmapheresis. Rituximab, a monoclonal antibody against CD20(+) B cells, is a promising therapeutic option that may soon become first-line therapy. Pemphigus is one of the best-characterized human autoimmune diseases and provides an ideal paradigm for both basic and clinical research, especially towards the development of antigen-specific immune suppression treatments for autoimmune diseases.