Lübeck Institute of Experimental Dermatology and Center for Research on Inflammation of the Skin, University of Lübeck, Lübeck, Germany.
Department of Dermatology and Center for Research on Inflammation of the Skin, University of Lübeck, Lübeck, Germany.
Front Immunol. 2021 Jan 11;11:591971. doi: 10.3389/fimmu.2020.591971. eCollection 2020.
Pemphigus and pemphigoid diseases are autoimmune bullous diseases characterized and caused by autoantibodies targeting adhesion molecules in the skin and/or mucous membranes. Personalized medicine is a new medical model that separates patients into different groups and aims to tailor medical decisions, practices, and interventions based on the individual patient`s predicted response or risk factors. An important milestone in personalized medicine in pemphigus and pemphigoid was achieved by verifying the autoimmune pathogenesis underlying these diseases, as well as by identifying and cloning several pemphigus/pemphigoid autoantigens. The latter has become the basis of the current, molecular-based diagnosis that allows the differentiation of about a dozen pemphigus and pemphigoid entities. The importance of autoantigen-identification in pemphigus/pemphigoid is further highlighted by the emergence of autoantigen-specific B cell depleting strategies. To achieve this goal, the chimeric antigen receptor (CAR) T cell technology, which is used for the treatment of certain hematological malignancies, was adopted, by generating chimeric autoantigen receptor (CAAR) T cells. In addition to these more basic science-driven milestones in personalized medicine in pemphigus and pemphigoid, careful clinical observation and epidemiology are again contributing to personalized medicine. The identification of clearly distinct clinical phenotypes in pemphigoid like the non-inflammatory and gliptin-associated bullous pemphigoid embodies a prominent instance of the latter. We here review these exciting developments in basic, translational, clinical, and epidemiological research in pemphigus and pemphigoid. Overall, we hereby aim to attract more researchers and clinicians to this highly interesting and dynamic field of research.
天疱疮和类天疱疮疾病是自身免疫性大疱性疾病,其特征和病因是自身抗体针对皮肤和/或粘膜中的粘附分子。个性化医学是一种新的医学模式,它将患者分为不同的群体,旨在根据个体患者的预测反应或危险因素来定制医疗决策、实践和干预措施。在天疱疮和类天疱疮的个性化医学中,一个重要的里程碑是验证了这些疾病的自身免疫发病机制,以及鉴定和克隆了几种天疱疮/类天疱疮自身抗原。后者已成为当前基于分子的诊断的基础,该诊断允许区分大约十几种天疱疮和类天疱疮实体。在天疱疮/类天疱疮中,自身抗原鉴定的重要性还通过自身抗原特异性 B 细胞耗竭策略的出现得到进一步强调。为了实现这一目标,采用嵌合抗原受体(CAR)T 细胞技术来治疗某些血液恶性肿瘤,生成嵌合自身抗原受体(CAAR)T 细胞。除了这些更基于基础科学的天疱疮和类天疱疮个性化医学里程碑之外,仔细的临床观察和流行病学也在为个性化医学做出贡献。在类天疱疮中,如非炎症性和Gliptin 相关的大疱性类天疱疮,明确区分了不同的临床表型,这是后者的一个突出实例。我们在此回顾了天疱疮和类天疱疮在基础、转化、临床和流行病学研究中的这些令人兴奋的进展。总的来说,我们旨在吸引更多的研究人员和临床医生关注这一非常有趣和充满活力的研究领域。