Department of Medical Education, Linkou Chang Gung Memorial Hospital, Taoyuan, Taiwan.
Department of Dermatology, Drug Hypersensitivity Clinical and Research Center, Chang Gung Memorial Hospital, Keelung, Linkou, Taipei, Taiwan.
Clin Rev Allergy Immunol. 2023 Dec;65(3):331-353. doi: 10.1007/s12016-023-08973-1. Epub 2023 Oct 28.
Bullous pemphigoid is one of the most common autoimmune bullous diseases occurring primarily in the elderly. Pathogenic autoantibodies against BP180 and BP230 at the dermal-epidermal junction cause subepidermal blisters, erosions, and intense pruritus, all of which adversely affect the patients' quality of life and may increase their morbidity and mortality. Current systemic treatment options for bullous pemphigoid are limited to corticosteroids and immunosuppressants, which can have substantial side effects on these vulnerable patients that even exceed their therapeutic benefits. Therefore, more precisely, targeting therapies to the pathogenic cells and molecules in bullous pemphigoid is an urgent issue. In this review, we describe the pathophysiology of bullous pemphigoid, focusing on autoantibodies, complements, eosinophils, neutrophils, proteases, and the T helper 2 and 17 axes since they are crucial in promoting proinflammatory environments. We also highlight the emerging therapeutic targets for bullous pemphigoid and their latest discoveries in clinical trials or experimental studies. Further well-designed studies are required to establish the efficacy and safety of these prospective therapeutic options.
大疱性类天疱疮是最常见的自身免疫性大疱病之一,主要发生在老年人中。位于表皮-真皮交界处的致病性自身抗体针对 BP180 和 BP230,导致表皮下水疱、糜烂和剧烈瘙痒,所有这些都会对患者的生活质量产生不利影响,并可能增加他们的发病率和死亡率。目前大疱性类天疱疮的全身性治疗选择仅限于皮质类固醇和免疫抑制剂,这些药物对这些脆弱的患者可能产生显著的副作用,甚至超过了它们的治疗益处。因此,针对大疱性类天疱疮的致病细胞和分子的靶向治疗是一个紧迫的问题。在这篇综述中,我们描述了大疱性类天疱疮的病理生理学,重点介绍了自身抗体、补体、嗜酸性粒细胞、中性粒细胞、蛋白酶以及辅助性 T 细胞 2 和 17 轴,因为它们在促进炎症环境中起着至关重要的作用。我们还强调了大疱性类天疱疮的新兴治疗靶点及其在临床试验或实验研究中的最新发现。需要进一步设计良好的研究来确定这些有前途的治疗选择的疗效和安全性。