司库奇尤单抗治疗白塞综合征黏膜受累的临床疗效与安全性

Efficacy and safety of secukinumab in the treatment of mucosal involvement in Beh et syndrome

目的探讨司库奇尤单抗(SEC)治疗黏膜型白塞综合征(BS)的疗效和安全性。方法回顾性分析2020年10月至2022年7月在北京协和医院接受SEC治疗的7例BS黏膜受累患者的临床资料。结果共纳入男性3例,女性4例;平均年龄(34.3±6.2)岁,中位病程6(3,10)年。临床表现为复发性口腔溃疡(n=7),外阴溃疡(n=6),关节炎(n=4),皮肤病变(n=4),同时并发脊柱关节炎2例和SAPHO综合征1例,入组时均存在活动性口腔、外阴溃疡,既往应用传统治疗方案和/或生物制剂治疗反应不佳或不耐受。5例患者应用SEC皮下注射150 mg/周,连续5次后,逐渐减量为每2~4周一次;另2例接受150 mg/2周。随访7(2,11.5)个月后,4例患者达到完全缓解(CR)或部分缓解(PR);3例患者无效,其中2例出现消化道糜烂、溃疡。结论司库奇尤单抗治疗BS黏膜受累的疗效尚存争议,需进一步探讨。

Objective To evaluate the effectiveness and safety of secukinumab in Beh et syndrome(BS)patients with refractory mucosal involvement.Methods We retrospectively analyzed the clinical data of 7 BS patients with refractory mucosal involvement,who had been treated with secukinumab from October 2020 to July 2022 in Peking Union Medical College Hospital.Results Seven participants(3 men and 4 women)with a mean age of(34.3±6.2)years were enrolled.The median duration of the disease was 6(range:3-10)years.They presented with refractory oral ulcers(n=7),genital ulcers(n=6),arthritis and skin lesions(n=4).Two patient had concurrent spondyloarthritis,and one had SAPHO(synovitis,acne,pustulosis,hyperostosis,and osteitis)syndrome.All the patients responded poorly to or were intolerant to immunosuppressants and biologics prior to secukinumab therapy.In 5 of these patients,secukinumab was administered subcutaneously at 150 mg per week in the first month,and then 150 mg every 2-4 weeks.In the other 2 patients,secukinumab was administered at 150 mg per two weeks during the entire follow-up.After a median follow-up of 7 months,4 patients achieved complete response or partial response,while 3 patients had no response to secukinumab.Two patients experienced newly developed gastrointestinal erosions and ulcers during follow-up.Conclusions The effectiveness of secukinumab to mucosal manifestations of Beh et syndrome is questionable,further large-scale study is warranted.