典型文献
Schnitzler syndrome and Schnitzler-like syndromes
文献摘要：
Schnitzler syndrome is a rare disease of adult-onset with main features including chronic urticarial rash, recurrent fever, arthralgia or arthritis, monoclonal gammopathy of undetermined significance (MGUS), and marked systemic inflammation. Schnitzler syndrome is often underdiagnosed. Patients with Schnitzler syndrome may present to dermatologists and allergists for urticaria, hematologists for MGUS, or rheumatologists for arthritis. It is important to recognize Schnitzler syndrome for its remarkable response to interleukin (IL)-1 blockade. Besides, many cases of Schnitzler-like syndromes do not meet the diagnostic criteria of classical Schnitzler syndrome but display excellent response to IL-1 inhibitors. The overly produced IL-1 is the result of a somatic mosaic gain of function mutation of
NLRP3 (nucleotide-binding oligomerization domain [NOD]-like receptor [NLR] family pyrin domain containing 3) gene in some patients with Schnitzler-like syndromes. Inflammasome activation is evident in patients with classical Schnitzler syndrome although no
NLRP3 gene mutation is identified. Collectively, Schnitzler syndrome and Schnitzler-like syndromes represent a spectrum of IL-1 mediated adult-onset autoinflammatory diseases.
文献关键词：
Schnitzler syndrome;Schnitzler syndrome-like conditions;Interleukin-1;Chronic urticarial rash
中图分类号：
作者姓名：
Chu Cong-Qiu
作者机构：
Division of Arthritis and Rheumatic Diseases, Oregon Health & Science University and Rheumatology Section, VA Portland Health Care System, Portland, OR 97239, USA
文献出处：
引用格式：
[1]Chu Cong-Qiu-.Schnitzler syndrome and Schnitzler-like syndromes)[J].中华医学杂志（英文版）,2022(10):1190-1202
A类：
Schnitzler,urticarial,allergists,hematologists,rheumatologists,Inflammasome,no
B类：
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AB值：
0.464043
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