Portsmouth Hospitals NHS Trust, National Amyloidosis Centre, UCL Division of Medicine and Royal Free London NHS Foundation Trust, Portsmouth, Hampshire, UK.
Clin Exp Dermatol. 2020 Dec;45(8):967-973. doi: 10.1111/ced.14251. Epub 2020 Sep 3.
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 Part 1 of this review, we took an overview of SAIDS and described the common features; in this article, we take a more in-depth look at the better recognized or more dermatologically relevant conditions.
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的疾病,可以是遗传性的或获得性的。它们几乎都是非常罕见的,容易被忽视;通常,患者在诊断前会看过多个专科,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,显著提高生活质量,并防止发生不可逆的并发症,如 AA 淀粉样变性。在这篇综述的第 1 部分中,我们对 SAIDS 进行了概述,并描述了常见特征;在本文中,我们更深入地探讨了一些更为公认或更与皮肤科相关的疾病。