The systemic autoinflammatory disorders for dermatologists. Part 2: disease examples

被引:6
作者
Oldham, J. [1 ,2 ]
Lachmann, H. J. [1 ,2 ]
机构
[1] Portsmouth Hosp NHS Trust, Natl Amyloidosis Ctr, UCL Div Med, Portsmouth, Hants, England
[2] Royal Free London NHS Fdn Trust, Portsmouth, Hants, England
关键词
FAMILIAL MEDITERRANEAN FEVER; INTERLEUKIN-36-RECEPTOR ANTAGONIST DEFICIENCY; SCHNITZLER-SYNDROME; AA AMYLOIDOSIS; CANAKINUMAB; ANAKINRA; SAFETY; INFLAMMATION; RILONACEPT; MANAGEMENT;
D O I
10.1111/ced.14251
中图分类号
R75 [皮肤病学与性病学];
学科分类号
100206 ;
摘要
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.
引用
收藏
页码:967 / 973
页数:7
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