Song Zi-Yi, Zhi Xin, Geng Jia-Yi, Sun Xing, Liu Tian, Li Zhan-Guo
Department of Rheumatology and Immunology, Peking University People's Hospital, and Beijing Key Laboratory for Rheumatism Mechanism and Immune Diagnosis (BZ0135), Beijing, China.
Department of Radiology, Peking University People's Hospital, Beijing, China.
ACR Open Rheumatol. 2025 Jul;7(7):e70071. doi: 10.1002/acr2.70071.
Behçet disease (BD) is characterized by recurrent oral and genital ulcers, skin lesions, and vascular involvement. Giant aortic aneurysm, a life-threatening complication of BD, often proves refractory to conventional therapies. Here, we present the case of a 71-year-old man with a 40-year history of BD complicated by a giant aortic aneurysm, which was successfully managed using the interleukin-6 (IL-6) receptor antagonist tocilizumab (TCZ). Despite years of tight treatment with high-dose glucocorticoids and immunosuppressants, there were frequent flares of disease and aortic aneurysm formation. Following the initiation of TCZ therapy, the patient achieved remarkable clinical improvement: the aortic aneurysm diameter decreased significantly from 10.15 × 7.83 cm to 6.22 × 5.12 cm, and disease activity, as measured by the Behçet's Disease Current Activity Form score, dropped from 8 to 1, indicating sustained disease control. This case underscores the potential of IL-6 inhibition as a transformative therapeutic strategy for refractory BD with severe vascular complications, offering new hope for patients with this challenging condition.
白塞病(BD)的特征为复发性口腔和生殖器溃疡、皮肤病变以及血管受累。巨大主动脉瘤是BD的一种危及生命的并发症,往往对传统治疗方法无效。在此,我们报告一例71岁男性患者,有40年BD病史,并发巨大主动脉瘤,使用白细胞介素-6(IL-6)受体拮抗剂托珠单抗(TCZ)成功治疗。尽管多年来一直使用高剂量糖皮质激素和免疫抑制剂进行严格治疗,但疾病仍频繁发作并形成主动脉瘤。开始TCZ治疗后,患者临床症状显著改善:主动脉瘤直径从10.15×7.83厘米显著减小至6.22×5.12厘米,根据白塞病当前活动形式评分衡量的疾病活动度从8降至1,表明疾病得到持续控制。该病例强调了抑制IL-6作为一种变革性治疗策略用于治疗伴有严重血管并发症的难治性BD的潜力,为患有这种具有挑战性疾病的患者带来了新希望。