自身免疫性大疱性疾病:发病机制和临床管理。

IF 6.3 Q1 BIOCHEMISTRY & MOLECULAR BIOLOGY
Xun Feng, Huaping Zheng, Mi Wang, Yiyi Wang, Xingli Zhou, Xiwen Zhang, Jishu Li, Yue Xiao, Mintong Wei, Xiaoguang Li, Takashi Hashimoto, Jingyi Li, Wei Li
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引用次数: 0

摘要

自身免疫性大疱性疾病(aibd)是一种异质性免疫介导的疾病,其特征是危及生命的皮肤和粘膜起泡。这篇综述综合了目前对AIBD发病机制、临床表型、诊断方法和治疗策略的理解,强调了最近的进展和转化机会。aibd的核心是自身抗体介导的表皮或基底膜区结构蛋白的破坏,特别是在桥粒体和半桥粒体连接处。主要亚型,包括天疱疮、副肿瘤天疱疮、类天疱疮和iga相关疾病,通过其靶抗原、临床表现和免疫病理特征来区分。诊断工作流程依赖于直接免疫荧光和血清学分析,但由于特征重叠,亚型分化仍然具有挑战性。传统的治疗方法,如全身皮质类固醇和免疫抑制剂,有改善的结果,但受到毒性的限制。最近的突破突出了靶向干预,包括利妥昔单抗的b细胞消耗,杜匹单抗的细胞因子调节,以及炎症途径的JAK抑制剂。嵌合自身抗体受体t细胞(CAART)治疗等创新策略通过消除自身反应性B细胞进一步解决难治性病例。此外,鉴于aibd与恶性肿瘤、自身免疫性合并症的关联,该综述强调了炎症驱动机制的新兴作用以及多学科护理的必要性。尽管取得了进展,但在早期诊断、个性化治疗优化和了解抗原特异性免疫反应方面仍然存在挑战。未来的方向包括改进诊断生物标志物,探索新的靶点,以及开发精准医学方法。
本文章由计算机程序翻译,如有差异,请以英文原文为准。
Autoimmune bullous diseases: pathogenesis and clinical management.

Autoimmune bullous diseases (AIBDs) represent a heterogeneous group of immune-mediated disorders characterized by life-threatening blistering of the skin and mucous membranes. This Review synthesizes current understanding of AIBD pathogenesis, clinical phenotypes, diagnostic approaches, and therapeutic strategies, emphasizing recent advancements and translational opportunities. At the core of AIBDs is autoantibody-mediated disruption of structural proteins in the epidermis or basement membrane zone, particularly at desmosomal and hemidesmosomal junctions. Key subtypes, including pemphigus, paraneoplastic pemphigus, pemphigoid, and IgA-related diseases, are distinguished by their target antigens, clinical manifestations, and immunopathological profiles. Diagnostic workflows rely on direct immunofluorescence, and serological assays, yet subtype differentiation remains challenging due to overlapping features. Traditional therapies, such as systemic corticosteroids and immunosuppressants, have improved outcomes but are limited by toxicity. Recent breakthroughs highlight targeted interventions, including B-cell depletion with rituximab, cytokine modulation via dupilumab, and JAK inhibitors for inflammatory pathways. Innovative strategies like chimeric autoantibody receptor T-cell (CAART) therapy further address refractory cases by eliminating autoreactive B cells. Additionally, the Review underscores the emerging role of inflammation-driven mechanisms and the necessity of multidisciplinary care, given AIBDs' associations with malignancies, autoimmune comorbidities. Despite progress, challenges persist in early diagnosis, personalized therapy optimization, and understanding antigen-specific immune responses. Future directions include refining diagnostic biomarkers, exploring novel targets, and developing precision medicine approaches.

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CiteScore
6.30
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