Department of Rheumatology and Immunology, Peking University People's Hospital.
Beijing Key Laboratory for Rheumatism Mechanism and Immune Diagnosis, Beijing, China.
Rheumatology (Oxford). 2022 Jul 6;61(7):2923-2930. doi: 10.1093/rheumatology/keab840.
The aim of this observational cohort study was to assess the effectiveness and safety of the IL-6-receptor inhibitor tocilizumab (TCZ) in Behçet's syndrome (BS) with refractory arterial involvement.
Ten patients admitted to the Rheumatology and Immunology Department of Peking University People's Hospital between January 2014 and December 2019 were enrolled. The enrolled patients met the BS international criteria and exhibited severe arterial impairments. Refractory arterio-BS was diagnosed based on objective vascular symptoms unexplainable by other known illnesses, and resistance to traditional immunosuppressants and glucocorticoids after 12 weeks. Patients received 8 mg/kg TCZ infusions every 4 weeks for ≥24 weeks, with simultaneous continuation of immunosuppressants and glucocorticoids. Clinical and imaging data were assessed before and after TCZ treatment.
The enrolled patients were men aged 44.3 (10.5) years; the median disease duration was 186.5 (45.7) months, and the average age of arterial impairment onset was 38.7 (12.9) years. The following trends were observed: improvement and maintenance of symptoms after the 26.8 (7.2)-month follow-up, n = 9; complete remission, n = 6; partial response, n = 3; immunosuppressant dose reduction, n = 4; radiologic improvement of arterial lesions, n = 4; and TCZ discontinuation owing to enlarged abdominal aortic aneurysm relapse, n = 1. The average daily glucocorticoid dose reduced from 54.5 (20.6) to 8.3 (3.6) mg/d (P < 0.001), while the median ESR and CRP values reduced from 50 (2-82) mm/h and 32.9 (2.1-62.3) mg/dl to 4 (1-10) mm/h and 2.9 (0.2-12.1) mg/dl, respectively (P < 0.001). No TCZ-associated side effects were noted.
TCZ proved to be safe and effective for refractory arterial lesions in BS, with a steroid- and immunosuppressant-sparing benefit.
本观察性队列研究旨在评估白细胞介素 6 受体抑制剂托珠单抗（TCZ）治疗难治性贝赫切特综合征（BS）伴动脉受累的疗效和安全性。
2014 年 1 月至 2019 年 12 月，10 例患者在北京大学人民医院风湿免疫科住院。纳入患者符合 BS 国际标准，且存在严重动脉损伤。难治性动脉 BS 基于客观血管症状诊断，即无法用其他已知疾病解释，并在 12 周后对传统免疫抑制剂和糖皮质激素耐药。患者接受 8mg/kg TCZ 每 4 周 1 次，共≥24 周，同时继续使用免疫抑制剂和糖皮质激素。在 TCZ 治疗前后评估临床和影像学数据。
纳入患者为男性，年龄 44.3（10.5）岁；中位病程 186.5（45.7）个月，动脉损伤发病平均年龄 38.7（12.9）岁。随访 26.8（7.2）个月后，9 例患者症状改善和维持；6 例患者完全缓解；3 例患者部分缓解；4 例患者减少免疫抑制剂剂量；4 例患者动脉病变影像学改善；1 例患者因腹主动脉瘤复发性增大而停用 TCZ。平均每日糖皮质激素剂量从 54.5（20.6）mg 降至 8.3（3.6）mg/d（P<0.001），平均红细胞沉降率和 C 反应蛋白值分别从 50（2-82）mm/h 和 32.9（2.1-62.3）mg/dl 降至 4（1-10）mm/h 和 2.9（0.2-12.1）mg/dl（P<0.001）。未观察到 TCZ 相关副作用。
TCZ 对 BS 难治性动脉病变安全有效，具有减少激素和免疫抑制剂的作用。
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