de Koning Heleen D
Department of Dermatology, Radboud University Medical Center, Nijmegen, The Netherlands ; Radboud Institute for Molecular Life Sciences (RIMLS), Nijmegen, The Netherlands ; Nijmegen Center for Immunodeficiency and Autoinflammation, Nijmegen, The Netherlands.
Clin Transl Allergy. 2014 Dec 5;4:41. doi: 10.1186/2045-7022-4-41. eCollection 2014.
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.
施尼茨勒综合征是一种自身炎症性疾病，其特征为单克隆IgM（或IgG）丙种球蛋白病、慢性荨麻疹样皮疹以及全身炎症的体征和症状，包括发热、关节痛和骨痛。该病于1972年首次被描述。本综述总结了迄今为止已报道的281例病例的临床特征、治疗效果及随访数据。此外，还将讨论皮肤组织学、骨成像、实验室检查结果以及发病机制的研究，包括白细胞介素-1β在该疾病中的关键作用。
