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难治性荨麻疹及诊断施尼茨勒综合征的重要性。

Refractory urticaria and the importance of diagnosing Schnitzler's syndrome.

作者信息

Nham Tina, Saleh Carol, Chu Derek, Vakaljan Stephanie L, Ohayon Jason A, Siegal Deborah M

机构信息

Department of Medicine, McMaster University, Hamilton, Ontario, Canada.

Population Health Research Institute, McMaster University, Hamilton, Ontario, Canada.

出版信息

BMJ Case Rep. 2019 Apr 29;12(4):e228546. doi: 10.1136/bcr-2018-228546.

DOI:10.1136/bcr-2018-228546
PMID:31036740
原文链接:https://pmc.ncbi.nlm.nih.gov/articles/PMC6505991/
Abstract

A 52-year-old man presented with chronic urticaria that was refractory to standard chronic spontaneous urticaria (CSU) treatment. Over time, he developed systemic symptoms including fatigue, weight loss, arthralgia and bone pain. His laboratory investigations also became significant for microcytic anaemia, neutrophilia and elevated C reactive protein, erythrocyte sedimentation rate and IgE levels, in addition to an IgM monoclonal protein. He achieved only partial remission with typical medications for CSU including omalizumab, cyclosporine and cetirizine. After 6 years, his worsening symptoms and abnormal investigations led to a rare diagnosis of Schnitzler's syndrome and a trial of the interleukin 1 receptor antagonist, anakinra, which caused a rapid and complete resolution of his symptoms.

摘要

一名52岁男性患有慢性荨麻疹,对标准的慢性自发性荨麻疹(CSU)治疗无效。随着时间的推移,他出现了全身症状,包括疲劳、体重减轻、关节痛和骨痛。他的实验室检查还显示有小细胞贫血、中性粒细胞增多以及C反应蛋白、红细胞沉降率和IgE水平升高,此外还有IgM单克隆蛋白。他使用包括奥马珠单抗、环孢素和西替利嗪在内的CSU典型药物仅获得部分缓解。6年后,他不断恶化的症状和异常检查结果导致了罕见的施尼茨勒综合征诊断,并试用了白细胞介素1受体拮抗剂阿那白滞素,该药物使他的症状迅速完全缓解。

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J Allergy Clin Immunol Pract. 2017 Sep-Oct;5(5):1314-1318. doi: 10.1016/j.jaip.2016.11.037. Epub 2017 Mar 14.
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Efficacy and safety of canakinumab in Schnitzler syndrome: A multicenter randomized placebo-controlled study.卡那奴单抗治疗 Schnitzler 综合征的疗效和安全性:一项多中心、随机、安慰剂对照研究。
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Comorbidity and pathogenic links of chronic spontaneous urticaria and systemic lupus erythematosus--a systematic review.慢性自发性荨麻疹与系统性红斑狼疮的共病情况及致病关联——一项系统综述
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The role of interleukin-1 beta in the pathophysiology of Schnitzler's syndrome.白细胞介素-1β在施尼茨勒综合征病理生理学中的作用。
Arthritis Res Ther. 2015 Jul 22;17(1):187. doi: 10.1186/s13075-015-0696-0.
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Schnitzler's syndrome: lessons from 281 cases.施尼茨勒综合征:来自281例病例的经验教训。
Clin Transl Allergy. 2014 Dec 5;4:41. doi: 10.1186/2045-7022-4-41. eCollection 2014.
6
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