Objectives Treatment response may be variable across organ manifestations of Behçet’s syndrome (BS). We aimed to determine the frequency of de novo manifestations during adalimumab treatment. Methods We conducted a chart review of all BS patients who received adalimumab in our centre between 2008 and 2023. Demographic data, reasons for initiating adalimumab, concurrent medications, previous treatments, and outcomes were recorded. We defined de novo manifestations as new BS manifestations that occurred for the first time during treatment with adalimumab. For patients with vascular involvement, a new vascular event at another vessel was also considered as a de novo manifestation. Results Among the 335 patients, a de novo manifestation developed in 14 (4%) patients. De novo manifestations were vascular involvement in five patients, arthritis in three, anterior uveitis in two, nervous system involvement in two, gastrointestinal involvement in one, and epididymitis in one patient. The primary reasons for adalimumab treatment were vascular involvement in five patients, uveitis in four, arthritis in three, mucocutaneous involvement in one, and epididymitis in one patient. Upon the development of de novo manifestation, adalimumab was switched to another biologic in four patients, dose was intensified in three, colchicine, conventional immunosuppressives and/or glucocorticoids were added in five, and topical eye drops were added in two patients, leading to remission of de novo manifestations in all patients. Conclusion De novo manifestations were infrequent (4%) among BS patients treated with adalimumab. Of these, 57% were major organ involvement, mainly vascular involvement. None of the patients developed posterior uveitis.

中文翻译：

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白塞综合征阿达木单抗治疗期间的新发表现


目的 治疗反应可能因 Behçet 综合征 （BS） 的器官表现而异。我们旨在确定阿达木单抗治疗期间新发表现的频率。方法 我们对 2008 年至 2023 年间在我们中心接受阿达木单抗治疗的所有 BS 患者进行了图表回顾。记录人口统计数据、开始阿达木单抗的原因、同时用药、既往治疗和结果。我们将新发表现定义为在阿达木单抗治疗期间首次出现的新 BS 表现。对于血管受累的患者，另一条血管的新血管事件也被认为是新发表现。结果 在 335 例患者中，14 例 （4%） 患者出现新发表现。新发表现为 5 例患者血管受累，3 例关节炎，2 例前葡萄膜炎，2 例神经系统受累，1 例胃肠道受累，1 例附睾炎。阿达木单抗治疗的主要原因是 5 例患者血管受累，4 例葡萄膜炎，3 例关节炎，1 例皮肤粘膜受累，1 例附睾炎。出现新发表现后，4 例患者改用另一种生物制剂，3 例增加剂量，5 例加入秋水仙碱、常规免疫抑制剂和/或糖皮质激素，2 例患者加入局部滴眼液，导致所有患者新发表现缓解。结论 在接受阿达木单抗治疗的 BS 患者中，新发表现很少 （4%）。其中，57% 是主要器官受累，主要是血管受累。所有患者均未发生后葡萄膜炎。