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大疱性类天疱疮的新兴治疗方法。

Emerging treatments for bullous pemphigoid.

机构信息

Dermatology Department, Hospital de Santa Maria, Centro Hospitalar Universitário Lisboa Norte (CHULN), Lisbon, Portugal.

Faculdade de Medicina da Universidade de Lisboa, Dermatology Universitary Clinic, Lisbon, Portugal.

出版信息

J Dermatolog Treat. 2022 Mar;33(2):649-661. doi: 10.1080/09546634.2020.1782325. Epub 2021 Jul 2.

Abstract

Bullous pemphigoid (BP) is the most common autoimmune blistering disease. It is characterized by an immune response against the hemidesmosomal anchoring proteins BP180 and BP230. BP mainly affects elderly patients, with an increasing incidence over the past two decades. High potency topical and/or systemic corticosteroids as well as immunosuppressants are the current mainstay of treatment. However, long-term systemic immunosuppression may result in significant morbidity and mortality. Recent advances in the understanding of the pathogenesis of BP have enabled the investigation of newer therapies that specifically work against a variety of pro-inflammatory mediators associated with BP. These new treatments hold promise to be highly efficient and safer alternatives and are expected to be shortly available for the treatment of BP. This review discusses current evidence on the use of novel targeted therapeutic approaches in the treatment of BP.

摘要

大疱性类天疱疮(BP)是最常见的自身免疫性水疱病。它的特征是针对半桥粒锚定蛋白 BP180 和 BP230 的免疫反应。BP 主要影响老年患者,在过去二十年中发病率不断增加。强效外用和/或全身性皮质类固醇和免疫抑制剂是目前的主要治疗方法。然而,长期的全身免疫抑制可能导致显著的发病率和死亡率。对 BP 发病机制的理解的最新进展使得可以研究针对与 BP 相关的各种促炎介质的新型治疗方法。这些新的治疗方法有望成为高效且更安全的替代方法,并有望很快用于治疗 BP。本文综述了新型靶向治疗方法在 BP 治疗中的应用的现有证据。

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