Schnitzler's syndrome: lessons from 281 cases

被引：84
作者：
de Koning, Heleen D. [1 ,2 ,3 ]
机构：
[1] Radboud Univ Nijmegen, Med Ctr, Dept Dermatol, Nijmegen, Netherlands
[2] RIMLS, Nijmegen, Netherlands
[3] Nijmegen Ctr Immunodeficiency & Autoinflammat, Nijmegen, Netherlands
来源：
CLINICAL AND TRANSLATIONAL ALLERGY | 2014年 / 4卷
关键词：
Schnitzler's syndrome; Interleukin-1; beta; Autoinflammation; Chronic urticaria; Paraprotein; Monoclonal gammopathy;
D O I：
10.1186/2045-7022-4-41
中图分类号：
R392 [医学免疫学];
学科分类号：
100102 ;
摘要：
Schnitzler's syndrome is an autoinflammatory disorder characterized by the association of a monoclonal IgM (or IgG) gammopathy, a chronic urticarial rash, and signs and symptoms of systemic inflammation, including fever, arthralgias and bone pain. It was first described in 1972. This review summarizes the clinical features, efficacy of therapies, and follow-up data of the 281 cases that have been reported to date. Also, the results of skin histology, bone imaging, laboratory investigations, and studies of the pathogenesis will be discussed, including the pivotal role of interleukin-1 beta in this disorder.
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页数：15
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