为了评估卡妥单抗在肿瘤坏死因子受体相关周期性综合征（TRAPS）患者中的疗效、安全性和耐受性，对《CLUSTER研究》进行了为期72周的长期、开放标签扩展研究。患者口服卡妥单抗150或300毫克，每4周或每8周一次，根据治疗期间的突发症状可以适当调整剂量（最大剂量：每4周300毫克）。疗效评估包括医师全面评估（PGA）疾病活动、突发症状次数、血清超敏C反应蛋白（CRP）和血清淀粉样A蛋白（SAA）水平。还报告了不良事件。根据累积卡妥单抗剂量（根据体重调整，<36毫克/千克或≥36毫克/千克）对总体人群和结果进行了描述。在进入《CLUSTER研究》最后一阶段（第4期）的53名患者中，有51名完成了治疗。在第4期结束时，超过94％的患者无或轻微疾病活动。大多数患者没有（69.8％）或只有一个突发症状（24.5％），而在基线时，突发症状的中位数为每年9.0次。中位数CRP水平仍保持在<10毫克/升以下。中位数SAA浓度在研究结束时在<36毫克/千克和≥36毫克/千克组别中分别为11.5毫克/升和14.5毫克/升，基本保持不变。未发现任何意外的安全检测结果。在《CLUSTER研究》72周的最后阶段，长期卡妥单抗治疗维持了TRAPS患者的疾病活动控制，突发症状发生率较低，未发现新的安全问题。本文受版权保护。保留所有权利。

To assess efficacy, safety, and tolerability of canakinumab in patients with tumor necrosis factor receptor-associated periodic syndrome (TRAPS) during a 72-week long-term, open-label extension of the CLUSTER study.Patients received open-label canakinumab 150 or 300 mg, either every 4 weeks (q4w) or every 8 weeks, with up-titration permitted following on-treatment flares (maximum dose: 300 mg q4w). Efficacy assessments included physician global assessment (PGA) of disease activity, number of flares, and serum C-reactive protein (CRP) and serum amyloid A protein (SAA) levels. Adverse events were also reported. Results are described for the overall population and according to the cumulative dose of canakinumab adjusted for body weight (<36 mg/kg or ≥36 mg/kg).Of 53 patients entering the final phase (Epoch 4) of CLUSTER, 51 completed the treatment. At the end of Epoch 4, >94% of patients achieved no or minimal disease activity. Most patients had either no (69.8%) or one flare (24.5%), whereas at baseline the median number of flares was 9.0 per year. Median CRP levels remained at <10 mg/L. Median SAA concentrations were largely unchanged with medians of 11.5 mg/L and 14.5 mg/L in the <36 mg/kg and ≥36 mg/kg groups, respectively, at the end of study. No unexpected safety findings were identified.Control of disease activity, with low flare incidence, was maintained with long-term canakinumab treatment in patients with TRAPS during the 72-week final Epoch of the CLUSTER study, with no new safety findings. This article is protected by copyright. All rights reserved.This article is protected by copyright. All rights reserved.