生物制剂治疗以心脏瓣膜病变为主要表现的白塞病8例  

Biological agents in the treatment of Beh?et’s disease with cardiac valve involvement

作　　者：丁艳霞 李超然[1] 刘金晶 史婧 李璐 赵岩 曾小峰 张奉春 许尚栋 郑文洁[1] DING Yan-xia;LI Chao-ran;LIU Jin-jing;SHI Jing;LI Lu;ZHAO Yan;ZENG Xiao-feng;ZHANG Feng-chun;XU Shang-dong;ZHENG Wen-jie(Department of Rheumatology,Peking Union Medical College Hospital,Chinese Academy of Medical sciences & Peking Union Medical College,Key Laboratory of Rheumatology and Clinical Immunology, Ministry of Education, Beijing 100730,China;Department of Rheumatology,The First Affiliated Hospital of Zhengzhou University,Zhengzhou 450052,China;Department of Cardiac Surgery, Beijing Anzhen Hospital, Capital Medical University, Beijing 100029,China)

机构地区：[1]中国医学科学院北京协和医学院北京协和医院风湿免疫科风湿免疫病学教育部重点实验室,北京100730 [2]郑州大学第一附属医院风湿免疫科,郑州450052 [3]首都医科大学附属北京安贞医院心脏外科,北京100029

出　　处：《中华临床免疫和变态反应杂志》2019年第2期108-112,共5页Chinese Journal of Allergy & Clinical Immunology

基　　金：国家自然科学基金(81571598,81871299)~~

摘　　要：目的探讨生物制剂治疗以心脏瓣膜病变为主要表现的白塞病患者的疗效。方法收集北京协和医院2016年6月至2018年9月以心脏瓣膜病变为主要表现并应用生物制剂治疗的8例患者的临床资料,回顾性分析其临床表现、超声心动图资料、手术情况及应用生物制剂后的疗效。结果 8例均为男性,平均年龄(39.9±7.7)岁,平均病程(11.8±6.0)年。瓣膜病变均以主动脉瓣受累为主,超声心动图均表现为重度主动脉瓣关闭不全。8例均在围手术期应用了生物制剂,包括英夫利昔单抗、阿达木单抗和托珠单抗。8例患者共行18例次手术,其中6例手术≥2次,先后出现瓣周漏10例次,均为单纯主动脉瓣置换术后,且术前未用激素/免疫抑制剂治疗者8例次;经围手术期积极治疗(激素联合免疫抑制剂及生物制剂),改用Carbrol术、Bentall术及瓣周漏封堵后,随访平均13(6~24)个月无明显术后并发症。应用生物制剂后,8例患者激素用量显著下降[从(41.3±15.3)mg降至(14.7±3.9)] mg(P<0.05)。结论白塞病瓣膜病变诊治困难,应加强激素和免疫抑制剂治疗,注意改良手术方式,对疗效欠佳或需要尽快手术的患者加用生物制剂能快速控制炎症,尽快创造手术时机,并减少术后并发症的发生。Objective To evaluate the efficacy of biological agents in the treatment of Beh et s disease (BD) with cardiac valve involvement. Methods We retrospectively reviewed the clinical data of 8 BD patients with cardiac valve lesions that were admitted to Peking Union Medical College Hospital(PUMCH) and treated with biological agents between June 2016 to September 2018. Results All patients were male, with an average age of (39.9±7.7)years old and an average disease duration of (11.8±6.0)years. Aortic valve was the most commonly affected, and echocardiography findings showed severe aortic regurgitation in all patients. Biological agents , including infliximab, adalimumab, or tocilizumab were administrated in all patients during the perioperative period. Eight patients underwent 18 operations, and six of them underwent at least two surgeries. Ten simple aortic valve replacement (AVR) surgeries all resulted in severe perivalvular leakage (PVL), and eight of them did not receive preoperative immunosuppressive therapy. Patients underwent Carbrol, Bentall, or aortic root replacement surgeries with perioperative immunosuppressive therapy had no complications during an average of 13(6-24) months follow-up. The corticosteroid dose was tapered [from(41.3±15.3 )mg to (14.7±3.9) mg, P < 0.05] in all patients after the application of biological agents. Conclusions Cardiac valve involvement in BD requires a timely diagnosis and management. Immunosuppressive therapy and modified surgical approaches may be essential for improving the outcome of these patients, and appropriate biological agents could be taken into consideration for purpose of rapid control of preoperative inflammation and less postoperative complications.

关 键 词：白塞病 心脏瓣膜受累 生物制剂 

分 类 号：R597.9[医药卫生—内科学]