Clinical Department of General Internal Medicine and The Research Department of Microbiology and Immunology, Laboratory for Clinical Infectious and Immunological Disorders, University Hospital Leuven, Leuven, Belgium.
Semin Arthritis Rheum. 2013 Feb;42(4):413-6. doi: 10.1016/j.semarthrit.2012.06.003. Epub 2012 Aug 15.
Daily injections of anakinra, an interleukin-1-receptor antagonist, have been reported to control effectively the symptoms and signs of Schnitzler syndrome, a rare acquired autoinflammatory disorder, presenting in adulthood by intermittent fever, urticarial rash, and paraproteinemia, usually IgM. Canakinumab, a fully human interleukin-1β monoclonal antibody, approved for the cryoporin-associated periodic syndrome, may offer a practical advantage because its half-life of ∼28 days may allow less frequent dosing. The present trial was designed to test canakinumab in patients with Schnitzler syndrome.
A patient with Schnitzler syndrome was treated with canakinumab, 150 mg subcutaneously injection every 8 weeks for 6 consecutive months. Injections were resumed in case of a flare following discontinuation.
Canakinumab induced a swift and sustained clinical response, with disappearance of fever and arthralgias, near abolishment of fatigue and rash, and substantial reduction of C-reactive protein levels. Interruption of canakinumab after four 8-weekly injections led to a flare 10 weeks after the last administration, which was countered as soon as canakinumab injections were resumed. The patient remained in complete remission. Canakinumab was well tolerated. No injection site reactions, other adverse events, or laboratory abnormalities were observed.
Canakinumab has potential for the treatment of Schnitzler syndrome (ClinicalTrials.gov.number, NCT01245127).
白介素-1 受体拮抗剂阿那白滞素的每日注射已被报道能有效控制 Schnitzler 综合征（一种罕见的获得性自身炎症性疾病）的症状和体征，该病在成年期以间歇性发热、荨麻疹样皮疹和单克隆丙种球蛋白病（通常为 IgM）为特征。Canakinumab 是一种全人源白介素-1β 单克隆抗体，已被批准用于 cryoporin 相关周期性综合征，因其半衰期约为 28 天，可能具有更方便的给药间隔，因此可能具有实际优势。本试验旨在研究 canakinumab 治疗 Schnitzler 综合征的效果。
一名 Schnitzler 综合征患者接受 canakinumab 治疗，剂量为 150mg，皮下注射，每 8 周一次，连续 6 个月。停药后如出现病情复发，则再次开始注射。
Canakinumab 诱导了迅速而持续的临床反应，发热和关节痛消失，疲劳和皮疹几乎完全消退，C 反应蛋白水平显著降低。在连续 4 次 8 周注射后中断治疗，在末次给药后 10 周出现病情复发，一旦再次开始注射 canakinumab，病情就得到了控制。患者完全缓解，且对治疗的耐受性良好。未观察到注射部位反应、其他不良事件或实验室异常。
Canakinumab 可能对 Schnitzler 综合征具有治疗作用（ClinicalTrials.gov 编号：NCT01245127）。
