Department of general internal medicine, University Hospitals Leuven, Herestraat 49, 3000 Leuven, Belgium; Laboratory of clinical infectious and inflammatory disease, department of Microbiology, Immunology and Transplantation, KU Leuven, Herestraat 49, 3000 Leuven, Belgium.
Department of general internal medicine, University Hospitals Leuven, Herestraat 49, 3000 Leuven, Belgium; Allergy and Clinical Immunology Research Group, department of Microbiology, Immunology and Transplantation, KU Leuven, Herestraat 49, 3000 Leuven, Belgium.
Semin Arthritis Rheum. 2020 Aug;50(4):636-642. doi: 10.1016/j.semarthrit.2020.05.002. Epub 2020 May 25.
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.
Schnitzler 综合征是一种罕见的自身炎症性疾病,其特征为慢性荨麻疹样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 是一种完全人源化的抗白细胞介素-1β(IL-1β)单克隆抗体,已被证明可有效治疗由 IL-1 驱动的自身炎症性疾病。
我们系统地检索了 PubMed 和 Embase,纳入了截至 2020 年 3 月 16 日发表的 Schnitzler 综合征患者使用 Canakinumab 治疗的所有类型研究。
自 2011 年以来,已有 7 篇关于 Schnitzler 综合征患者使用 Canakinumab 治疗的文献报道了 34 例患者。累积随访时间为 253 个月,5 项研究的随访时间为 12 个月或更长。治疗期间报告完全缓解的患者占 58.6%;其余所有患者均有部分缓解。23 名患者报告了 207 次不良事件。感染(n=79)是最常见的不良事件。1 例患者因非典型分枝杆菌感染导致脓毒症死亡。
基于目前的系统评价结果,Canakinumab 是 Schnitzler 综合征患者的一种有效长期治疗方法,具有良好的安全性。