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天疱疮和类天疱疮在认识与治疗方面的最新进展

Recent advances in the understanding and treatment of pemphigus and pemphigoid.

作者信息

Yamagami Jun

机构信息

Department of Dermatology, Keio University School of Medicine, 35 Shinanomachi, Shinjuku-ku, Tokyo, 160-8582, Japan.

出版信息

F1000Res. 2018 Aug 30;7. doi: 10.12688/f1000research.14474.1. eCollection 2018.

Abstract

Pemphigus and pemphigoid are characterized as autoimmune blistering diseases in which immunoglobulin G autoantibodies cause blisters and erosions of the skin or mucosa or both. Recently, understanding of the pathophysiology of pemphigus and pemphigoid has been furthered by genetic analyses, characterization of autoantibodies and autoreactive B cells, and elucidation of cell-cell adhesion between keratinocytes. For the management of pemphigus and pemphigoid, the administration of systemic corticosteroids still represents the standard treatment strategy; however, evidence of the efficacy of therapies not involving corticosteroids, such as those employing anti-CD20 antibodies, is increasing. The goal should be to develop antigen-specific immune suppression-based treatments.

摘要

天疱疮和类天疱疮是自身免疫性水疱性疾病,其中免疫球蛋白G自身抗体可导致皮肤或黏膜或两者出现水疱和糜烂。近来,通过基因分析、自身抗体和自身反应性B细胞的特征鉴定以及角质形成细胞间细胞黏附的阐明,对天疱疮和类天疱疮的病理生理学有了更深入的了解。对于天疱疮和类天疱疮的治疗,全身使用糖皮质激素仍然是标准的治疗策略;然而,不涉及糖皮质激素的疗法(如使用抗CD20抗体的疗法)的疗效证据正在增加。目标应该是开发基于抗原特异性免疫抑制的治疗方法。

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