全身性脓疱性牛皮癣:免疫机制,遗传学和新兴治疗方法。

IF 13.1 1区 医学 Q1 IMMUNOLOGY
Trends in Immunology Pub Date : 2025-01-01 Epub Date: 2024-12-27 DOI:10.1016/j.it.2024.12.001
Chih-Chun Lee, Yu-Huei Huang, Ching-Chi Chi, Wen-Hung Chung, Chun-Bing Chen
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引用次数: 0

摘要

摘要广泛性脓疱性牛皮癣(GPP)是一种罕见的人类自身炎性疾病,具有危及生命的全身性影响。角化细胞衍生的白细胞介素(IL)-36信号已被确定为受影响个体皮肤免疫反应的关键介质。对沿IL-36轴和下游核转录因子κB (NF-κB)信号传导的各种突变的识别,使GPP在遗传、免疫和组织病理学上具有独特性,并可适应免疫调节,最近IL-36拮抗剂的成功体现了这一点。这篇综述涵盖了GPP的遗传和免疫学基础的最新发现,这些发现为提高这种具有临床挑战性和衰弱性疾病的护理质量提供了肥沃的土壤。
本文章由计算机程序翻译,如有差异,请以英文原文为准。
Generalized pustular psoriasis: immunological mechanisms, genetics, and emerging therapeutics.

Generalized pustular psoriasis (GPP) is a rare human autoinflammatory disorder with life-threatening systemic effects. Keratinocyte-derived interleukin (IL)-36 signaling has been identified as a key mediator of immune response in the skin of affected individuals. Recognition of various mutations along the IL-36 axis and the downstream nuclear transcription factor κB (NF-κB) signaling have established GPP as genetically, immunologically, and histopathologically distinct and amenable to immunomodulation, which is epitomized by the recent success of IL-36 antagonism. This review covers recent discoveries of the genetic and immunological underpinnings of GPP, which have proved fertile ground for improving the quality of care of this clinically challenging and debilitating condition.

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来源期刊
Trends in Immunology
Trends in Immunology 医学-免疫学
CiteScore
25.10
自引率
0.60%
发文量
130
审稿时长
6-12 weeks
期刊介绍: Trends in Immunology serves as a vital platform for tracking advancements across various areas of immunology, offering concise reviews and hypothesis-driven viewpoints in each issue. With additional sections providing comprehensive coverage, the journal offers a holistic view of immunology. This broad perspective makes it an invaluable resource for researchers, educators, and students, facilitating the connection between basic and clinical immunology. Recognized as one of the top monthly review journals in its field, Trends in Immunology is highly regarded by the scientific community.
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