Objective: To explore the efficacy and safety of anti-tumor necrosis factor alpha (TNFα) monoclonal antibodies (mAbs) for severe/refractory vasculo-Behcet's disease (BD). Method: The clinical data of severe/refractory vasculo-BD patients treated with anti-TNFα mAbs were retrospectively analyzed. Response of anti TNFα mAbs was analyzed. The dosage changes of glucocorticoid, the level of erythrocyte sedimentation rate (ESR) and hypersensitive C-reactive protein (hsCRP) before and after treatment were recorded, as well as side effects. Result: Sixteen patients were enrolled. Arterial lesions were reported in 12 patients, including 9 with arterial aneurysm, 6 with arterial dilation, 2 with stenosis and 2 with occlusion. Seven patients presented venous thrombosis, including lower extremity veins (n=6), cerebral venous sinus (n=2) and inferior vena cava system (n=2). Two cases had both arterial and venous involvement. Before the application of TNFα mAbs, all 16 patients failed to response to prednisone or its equivalent dose of 40 (7.5-90) mg/d in combination with cyclophosphamide, methotrexate, thalidomide or azathioprine for median 4 (0-156) months. After a mean duration of treatment for (17.1±6.5) months, 15 patients achieved complete remission and 1 patient achieved partial remission. Three patients received surgery without any postoperative complications. After using anti TNFα mAbs, the dosage of prednisone [5(0-12.5)mg/d vs. 40(7.5-90)mg/d, P<0.01], ESR [(7.3±4.6) mm/1h vs. (33.5±26.7) mm/1h, P<0.01] and hsCRP [1.9(0.2-11.4) mg/L vs. 24.3(0.4-113.9) mg/L, P<0.01] were significantly decreased. Side effects were observed in 2 patients. One developed pulmonary infection 12 months after adalimumab with conventional treatment. Another patient had allergy to infliximab then switched to adalimumab. Conclusion: In combination with corticosteroids and immunosuppressants, anti-TNF α mAbs are effective and well-tolerated in severe/refractory vasculo-BD, with a favorable steroid -sparing effect and rare postoperative complications.
目的： 初步探讨抗肿瘤坏死因子α（TNFα）单抗治疗重症/难治性血管白塞病的疗效与安全性。 方法： 回顾性分析16例应用抗TNFα单抗治疗的重症/难治性血管白塞病患者的临床资料及抗TNFα单抗的疗效，比较联合用药前后糖皮质激素剂量、红细胞沉降率（ESR）和超敏C反应蛋白（CRP）水平，记录不良反应。 结果： 16例血管白塞病患者男性14例，女性2例，年龄（35.8±13.7）岁，病程（10.5±5.8）年。白塞病起病至出现血管病变（7.5±5.3）年，16例患者中动脉系统受累12例，表现为动脉瘤（9例）、动脉扩张（6例)、动脉狭窄（2例）和动脉闭塞（2例)；静脉血栓7例，受累部位包括下肢静脉（6例)、下腔静脉（2例）和颅内静脉窦（2例)；动静脉系统均有受累者2例。应用抗TNFα单抗前，16例患者经泼尼松或等效剂量糖皮质激素40（7.5~90）mg/d联合环磷酰胺、甲氨蝶呤、沙利度胺、硫唑嘌呤等免疫抑制剂中位治疗4（0~156）个月后疗效不佳。联合应用英夫利西单抗3~5 mg/kg或阿达木单抗40mg/次，平均疗程（17.1±6.5）个月后，15例患者达到完全缓解，1例患者达到部分缓解。3例患者行外科手术治疗，均未发生术后并发症。治疗后，泼尼松剂量[5（0~12.5）mg/d比40（7.5~90）mg/d，P<0.01]、ESR[（7.3±4.6）mm/1h比（33.5±26.7）mm/1h，P<0.01]、超敏CRP[1.9（0.2~11.4）mg/L比24.3（0.4~113.9）mg/L，P<0.01]较治疗前显著下降。2例患者出现不良反应，1例应用阿达木单抗联合传统治疗12个月后出现肺部感染，停用阿达木单抗并给予抗感染治疗后好转；1例首次应用英夫利西单抗过程中，出现过敏反应，之后换用阿达木单抗后无过敏反应发生。 结论： 对重症/难治性血管白塞病，应用抗TNFα单抗与糖皮质激素和免疫抑制剂联合治疗，有效且耐受性好，有利于减少糖皮质激素用量及术后并发症。.
Keywords: Anti-tumor necrosis factor α monoclonal antibodies; Behcet syndrome; Efficacy; Safety; Vascular involvement.