Objective

To describe the outcome and tolerance in patients treated with anti-TNFα in severe and refractory major vessel disease in Behçet's disease (BD).

Methods

A multicenter study evaluating 18 refractory BD patients with major vessel involvement [pulmonary artery (n = 4), aorta (n = 4) or peripheral artery aneurysm (n = 1) and/or pulmonary artery (n = 7), inferior vena cava (n = 5), or intra-cardiac (n = 3) thrombosis or Budd Chiari Syndrome (n = 2)] treated with anti-TNFα agents.

Results

Vascular remission was achieved in 16 (89%) patients. The 9 months risk of relapse was significantly higher with conventional immunosuppressants used prior anti-TNFα agents as compared to anti-TNFα therapy [OR = 8.7 (1.42–62.6), p = 0.03]. The median daily dose of corticosteroids significantly decreased at 12 months. Side effects included infection (n = 4) and pulmonary edema (n = 1).

Conclusion

TNFα-antagonists are safe and might be associated with a decreased risk of relapse at 9 months compared to conventional immunosuppressants in BD patients with major vessels disease.

中文翻译：

---

抗TNFα在Behcet病重症和难治性大血管受累中的功效：对18位患者的多中心观察研究

客观的

描述在严重和难治性白血管病（BD）中使用抗TNFα治疗的患者的结局和耐受性。

方法

一项多中心研究评估了18例难治性BD患者，其主要血管受累[肺动脉（n = 4），主动脉（n = 4）或周围动脉瘤（n = 1）和/或肺动脉（n = 7），下腔静脉（n = 5），或用抗TNFα药物治疗的心内（n = 3）血栓形成或Budd Chiari综合征（n = 2）]。

结果

16名（89％）患者实现了血管缓解。与抗TNFα治疗相比，使用先前抗TNFα药物的常规免疫抑制剂的9个月复发风险显着更高[OR = 8.7（1.42-62.6），p = 0.03]。皮质类固醇的每日中位数剂量在12个月时显着下降。副作用包括感染（n = 4）和肺水肿（n = 1）。

结论

与传统的免疫抑制剂相比，对于患有大血管疾病的BD患者，TNFα拮抗剂是安全的，并且可能与9个月时复发风险降低有关。