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Abstract：
Schnitzler syndrome is a rare autoinflammatory disorder characterized by chronic urticarial rash and a monoclonal gammopathy, accompanied by intermittent fever, bone pain, and arthralgia or arthritis. Canakinumab is a fully human monoclonal anti-interleukin-1β (IL-1β) antibody proven to be effective in IL-1 driven autoinflammatory disorders.
We systematically searched PubMed and Embase to include all types of studies on canakinumab treatment in Schnitzler syndrome published until March 16, 2020.
Since 2011, 7 publications have been reported on canakinumab treatment in 34 patients with Schnitzler syndrome. The cumulative follow-up was 253 months, and 5 studies had a follow-up duration of 12 months or more. A complete response during treatment was reported in 58.6% of patients; all other patients had a partial response. Two hundred and seven adverse events were reported in 23 patients. Infection (n = 79) was the most common adverse event. One patient died from sepsis due to atypical mycobacterial infection.
Based on the results of the current systematic review, canakinumab is an effective long-term treatment with a favorable safety profile in patients with Schnitzler syndrome.
We systematically searched PubMed and Embase to include all types of studies on canakinumab treatment in Schnitzler syndrome published until March 16, 2020.
Since 2011, 7 publications have been reported on canakinumab treatment in 34 patients with Schnitzler syndrome. The cumulative follow-up was 253 months, and 5 studies had a follow-up duration of 12 months or more. A complete response during treatment was reported in 58.6% of patients; all other patients had a partial response. Two hundred and seven adverse events were reported in 23 patients. Infection (n = 79) was the most common adverse event. One patient died from sepsis due to atypical mycobacterial infection.
Based on the results of the current systematic review, canakinumab is an effective long-term treatment with a favorable safety profile in patients with Schnitzler syndrome.
摘要：
Schnitzler综合征是一种罕见的自身炎症性疾病，其特征是慢性荨麻疹皮疹和单克隆丙种球蛋白病，伴有间歇性发热,骨痛,关节痛或关节炎。Canakinumab是一种完全的人单克隆抗白细胞介素-1β（IL-1β）抗体，被证明对IL-1驱动的自身炎症性疾病有效。
我们系统地搜索了PubMed和Embase，以包括直到2020年3月16日发表的关于canakinumab治疗Schnitzler综合征的所有类型的研究。
自2011年以来，已有7篇出版物报道了canakinumab在34例Schnitzler综合征患者中的治疗。累计随访253个月,5项研究的随访时间为12个月或更长时间.据报道，58.6%的患者在治疗期间完全缓解；所有其他患者均有部分缓解。23例患者报告了两百零七个不良事件。感染（n=79）是最常见的不良事件。一名患者因非典型分枝杆菌感染死于败血症。
根据当前系统评价的结果，canakinumab是一种有效的长期治疗方法，对Schnitzler综合征患者具有良好的安全性。
我们系统地搜索了PubMed和Embase，以包括直到2020年3月16日发表的关于canakinumab治疗Schnitzler综合征的所有类型的研究。
自2011年以来，已有7篇出版物报道了canakinumab在34例Schnitzler综合征患者中的治疗。累计随访253个月,5项研究的随访时间为12个月或更长时间.据报道，58.6%的患者在治疗期间完全缓解；所有其他患者均有部分缓解。23例患者报告了两百零七个不良事件。感染（n=79）是最常见的不良事件。一名患者因非典型分枝杆菌感染死于败血症。
根据当前系统评价的结果，canakinumab是一种有效的长期治疗方法，对Schnitzler综合征患者具有良好的安全性。
