超声造影评估托法替布成功治疗难治性大动脉炎1例并文献复习  

Successful remission of refractory Takayasu arteritis with tofacitinib evaluated by contrast-enhanced ultrasound:a case report and review of literature

作　　者：杨金水[1] 李民[1] 张江林[1] 万月华 朱剑[1] 邓小虎[1] 赵玉荣 王秀茹 张洁[1] Yang Jin-Shui;Li Min;Zhang Jiang-Lin;Wan Yue-Hua;Zhu Jian;Deng Xiao-Hu;Zhao Yu-Rong;Wang Xiu-Ru;Zhang Jie(Department of Rheumatology,the First Medical Centre of Chinese PLA General Hospital,Beijing 100853,China)

机构地区：[1]解放军总医院第一医学中心风湿免疫科,北京100853

出　　处：《解放军医学杂志》2022年第1期58-64,共7页Medical Journal of Chinese People's Liberation Army

摘　　要：目的报道1例应用托法替布成功治疗的难治性大动脉炎(TAK)患者并复习文献,以提高对该病的认识。方法回顾性分析1例应用托法替布治疗并使用超声造影连续评价的TAK患者的病例资料,通过检索中国知网、万方数据知识服务平台、PubMed数据库,综合文献结果,总结TAK的治疗及随访经验。结果本例为28岁女性,病史10年,临床表现为发热、颈痛和关节疼痛,超声发现颈动脉管壁增厚、管腔狭窄,实验室检测发现C反应蛋白和红细胞沉降率升高,接受激素治疗后病情可减轻。激素减量过程中反复复发,先后联合使用多种抗风湿药物及托珠单抗等,因疗效不佳或药物不良反应未能规律应用,动脉炎症逐渐加重,出现左侧锁骨下动脉瘤,疾病治疗困难。随后调整治疗方案为托法替布(初始10 mg/d,后逐渐减量至7.5 mg/d)联合糖皮质激素(甲泼尼龙14 mg/d,后逐渐减量至8 mg/d),患者临床症状缓解,未发现严重的不良反应。超声造影提示动脉壁厚度降低(左颈总动脉壁厚度由用药前的8.9 mm降至1.2 mm,左锁骨下动脉壁厚度由7.4 mm降至0.8 mm)、造影强度减弱(总评分由5分降至1分)、动脉瘤消失。检索中国知网、万方数据知识服务平台、PubMed数据库(截至2021年3月),共报道10例应用托法替布治疗的TAK患者,其中男1例,女9例;7例病情减轻,激素剂量均有减少,仅1例出现不良反应。结论 Janus激酶(JAK)抑制剂有望成为治疗TAK新的有效药物;血管超声造影能够实时检测血管壁厚度及增厚管壁造影增强情况,是评估血管炎症的有效工具。Objective To report a case of refractory Takayasu arteritis (TAK) treated with tofacitinib (TOF) and evaluated by contrast-enhanced ultrasound,and perform a literature review to better understand this disorder.Methods The data of a case of TAK treated with TOF were analyzed retrospectively,the treatment and follow-up experience summarized by searching the database (CNKI,Wanfang Data,PubMed) and the literature results analyzed comprehensively.Results The patient,a 28-year-old female with a 10-year history of TAK,presented with fever,neck pain and joints pain.Ultrasonography revealed intimal thickening and stenosis of the carotid artery.Laboratory tests demonstrated an elevated erythrocyte sedimentation rate (ESR) and a C-reactive protein (CRP) level.The symptoms were improved after glucocorticoid (GC) treatment.However,it relapsed during the course of GC tapering,even with the combination of disease modifying anti-rheumatic drugs (DMARDs) and tocilizumab.The DMARDs and tocilizumab were not used continuously due to poor response and adverse drug reactions.As disease progressing,left subclavian aneurysm appeared.The treatment of this patient is a big challenge.After adjusting the treatment with tofacitinib (10 mg/d,then adjust to 7.5 mg/d) and GC (methylprednisolone 14 mg/d,then reduce to 8 mg/d),the patient was successfully relieved without any serious adverse events.Contrast-enhanced ultrasound showed that the thickness of arterial wall decreased (the thickness of left common carotid artery decreased from 8.9 mm to 1.2 mm,and the thickness of left subclavian artery decreased from 7.4 mm to 0.8 mm),the contrast intensity decreased (the total score decreased from 5 to 1),and the aneurysm disappeared.By March 2021 (searching CNKI,Wanfang Data and PubMed),a total of 10 cases of TAK patients treated with TOF.Nine patients were women.Seven patients improved and GC reduced.Only one patient had adverse reactions.Conclusions Janus kinase inhibitor is expected to be a new drug for the treatment of TAK.Contrast-enhanced ultr

关 键 词：大动脉炎 超声造影 托法替布 

分 类 号：R593.2[医药卫生—内科学]