Sun L X, Liu J J, Hou Y X, Li C R, Li L, Tian X P, Zeng X F, Zheng W J
Department of Rheumatology and Clinical Immunology, Peking Union Medical College Hospital, Peking Union Medical College & Chinese Academy of Medical Sciences, Key Laboratory of Rheumatology & Clinical Immunology, Ministry of Education, National Clinical Research Center for Dermatologic and Immunologic Diseases, Beijing 100730, China.
Department of Rheumatology, the Affiliated Hospital of Inner Mongolia Medical University, Hohhot 010050, China.
Beijing Da Xue Xue Bao Yi Xue Ban. 2020 Dec 18;52(6):1056-1062. doi: 10.19723/j.issn.1671-167X.2020.06.011.
To explore the effectiveness and safety of golimumab in the treatment of severe/refractory cardiovascular Behcet syndrome (BS).
We retrospectively analyzed the clinical data of nine patients diagnosed with severe/refractory cardiovascular BS and treated with golimumab from February 2018 to July 2020 in Peking Union Medical College Hospital. We analyzed levels of erythrocyte sedimentation rate (ESR) and high-sensitivity C-reactive protein (hsCRP), imaging findings, and the doses of glucocorticoids and immunosuppressive agents during the period of combined treatment with golimumab.
Nine patients were enrolled, including 8 males and 1 female, with a mean age and median course of (37.0±8.6) years and 120 (60, 132) months, respectively. Seven patients presented with severe aortic regurgitation combined with other cardiovascular involvement secondary to BS. Two patients presented with large vessel involvement, including multiple aneurysms and vein thrombosis. Prior to golimumab treatment, seven patients were treated with glucocorticoids and multiple immunosuppres-sants [with a median number of 3 (1, 3) types] while still experienced disease progression or elevated inflammation biomarkers during postoperative period. Eight patients with disease progression, uncontrolled inflammation and history of severe postoperative complications required effective and fast control of inflammation during perioperative period. One patient had adverse reaction with tocilizumab and switched to golimumab during perioperative period. The patients were treated with golimumab 50 mg every 4 weeks, along with concomitant treatment of glucocorticoid and immunosuppressants. After a median follow-up of (16.3±5.6) months, all the patients achieved clinical improvement. Vascular lesions were radiologically stable and no vascular progressive or newly-onset of vascular lesions was observed. The eight patients who experienced cardiac or vascular operations showed no evidence of postoperative complications. The ESR and hsCRP levels decreased significantly [16.5 (6.8, 52.5) mm/h . 4 (2, 7) mm/h, and 21.24 (0.93, 32.51) mg/L . 0.58 (0.37, 1.79) mg/L ( < 0.05), respectively]. The dose of prednisone was tapered from 35 (15, 60) mg/d to 10.0 (10.0, 12.5) mg/d. No prominent adverse reactions were observed.
Our study suggests that golimumab is effective in the treatment of severe/refractory cardiovascular BS. Combination immunosuppression therapy with golimumab contributes to control of inflammation, reduction of postoperative complications and tapering the dose of glucocorticoids or immunosuppressants.
探讨戈利木单抗治疗重度/难治性心血管白塞病（BS）的有效性和安全性。
回顾性分析2018年2月至2020年7月在北京协和医院诊断为重度/难治性心血管BS并接受戈利木单抗治疗的9例患者的临床资料。分析了红细胞沉降率（ESR）和高敏C反应蛋白（hsCRP）水平、影像学表现以及戈利木单抗联合治疗期间糖皮质激素和免疫抑制剂的剂量。
纳入9例患者，其中男性8例，女性1例，平均年龄（37.0±8.6）岁，中位病程120（60，132）个月。7例患者出现重度主动脉瓣反流并伴有BS继发的其他心血管受累。2例患者出现大血管受累，包括多发动脉瘤和静脉血栓形成。在接受戈利木单抗治疗前，7例患者接受了糖皮质激素和多种免疫抑制剂治疗[中位种类数为3（1，3）种]，但术后仍出现疾病进展或炎症生物标志物升高。8例疾病进展、炎症未得到控制且有严重术后并发症史的患者需要在围手术期有效快速控制炎症。1例患者对托珠单抗有不良反应，在围手术期改用戈利木单抗。患者每4周接受50 mg戈利木单抗治疗，并同时接受糖皮质激素和免疫抑制剂治疗。中位随访（16.3±5.6）个月后，所有患者均取得临床改善。血管病变在影像学上稳定，未观察到血管病变进展或新发血管病变。8例接受心脏或血管手术的患者未出现术后并发症迹象。ESR和hsCRP水平显著下降[分别为16.5（6.8，52.5）mm/h降至4（2，7）mm/h，以及21.24（0.93，32.51）mg/L降至0.58（0.37，1.79）mg/L（P<0.05）]。泼尼松剂量从35（15，60）mg/d减至10.0（10.0，12.5）mg/d。未观察到明显不良反应。
我们的研究表明，戈利木单抗治疗重度/难治性心血管BS有效。戈利木单抗联合免疫抑制治疗有助于控制炎症、减少术后并发症并减少糖皮质激素或免疫抑制剂的剂量。
