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皮肤科医生须知的系统性自身炎症性疾病。第 1 部分:概述。

The systemic autoinflammatory disorders for dermatologists. Part 1: overview.

机构信息

Portsmouth Hospitals NHS Trust, National Amyloidosis Centre, UCL Division of Medicine and Royal Free London NHS Foundation Trust, Portsmouth, Hampshire, UK.

出版信息

Clin Exp Dermatol. 2020 Dec;45(8):962-966. doi: 10.1111/ced.14250. Epub 2020 Sep 9.

Abstract

The systemic autoinflammatory disorders (SAIDs) or periodic fever syndromes are disorders of innate immunity, which can be inherited or acquired. They are almost all very rare and easily overlooked; typically, patients will have seen multiple specialities prior to diagnosis, so a high level of clinical suspicion is key. It is important to note that these are 'high-value' diagnoses as the majority of these syndromes can be very effectively controlled, dramatically improving quality of life and providing protection against the development of irreversible complications such as AA amyloidosis. In this article, we take an overview of SAIDs and look at the common features; in Part 2, we take a more in-depth look at the better recognized or more dermatologically relevant conditions.

摘要

系统性自身炎症性疾病(SAIDs)或周期性发热综合征是先天免疫紊乱,可遗传或获得。它们几乎都非常罕见,容易被忽视;通常,患者在确诊前已经看过多个专科,因此高度的临床怀疑是关键。值得注意的是,这些都是“高价值”的诊断,因为这些综合征中的大多数都可以得到非常有效的控制,极大地提高生活质量,并防止发生不可逆转的并发症,如 AA 淀粉样变性。在本文中,我们对 SAIDs 进行了概述,并探讨了其常见特征;在第二部分中,我们更深入地探讨了一些更被认可或更与皮肤科相关的疾病。

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