PDF(Pubmed)
Abstract：
Generalized pustular psoriasis (GPP) is a rare, chronic, and severe skin disorder characterized by the eruption of non-infectious pustules on an erythematous background often associated with systemic symptoms. It may appear in association with plaque psoriasis or occur in previously healthy individuals. It differs from psoriasis vulgaris in clinical presentation, immunopathogenesis, histology, and therapeutic strategies. Overexpression of interleukin 36 (IL-36) or a loss-of-function mutation of IL-36 receptor antagonist (IL-36RA) are thought to play a pivotal role in the pathogenesis of this disease. There are currently no globally approved guidelines for the treatment of GPP, and the therapies used so far, with variable results, have given unsatisfactory results. Spesolimab, a selective humanized antibody against the IL-36 receptor that blocks its activation, is the first biologic drug approved in Europe in December 2022 for the treatment of GPP flares. It represents a promising therapy, demonstrating efficacy in reducing disease severity and improving patient outcomes. In our review, we have analyzed the latest advancements and findings regarding the efficacy and safety of spesolimab in the context of GPP management.
摘要：
广义脓疱型牛皮癣(3GPP)是一种罕见的，慢性，和严重的皮肤病，其特征是在红斑背景下非感染性脓疱的爆发，通常与全身症状有关。它可能与斑块状银屑病相关或发生在先前健康的个体中。它在临床表现上与寻常型银屑病不同，免疫发病机制，组织学,和治疗策略。白介素36（IL-36）的过表达或IL-36受体拮抗剂（IL-36RA）的功能丧失突变被认为在该疾病的发病机理中起关键作用。目前还没有全球批准的关于GPP治疗的指南，以及到目前为止使用的疗法，具有可变的结果，给出了不满意的结果。Spesolimab,一种针对IL-36受体的选择性人源化抗体，可以阻断其激活，是2022年12月在欧洲批准的第一种生物药物，用于治疗3GPP耀斑。它代表了一种有希望的疗法，证明在降低疾病严重程度和改善患者预后方面的功效。在我们的审查中,我们分析了关于spesolimab在3GPP管理中的疗效和安全性的最新进展和发现.
