Division of Rheumatology, Department of Internal Medicine, Keio University School of Medicine, 35 Shinanomachi, Shinjuku-ku, Tokyo, Japan.
Division of Rheumatology, Department of Internal Medicine, Keio University School of Medicine, 35 Shinanomachi, Shinjuku-ku, Tokyo, Japan.
Semin Arthritis Rheum. 2020 Aug;50(4):797-804. doi: 10.1016/j.semarthrit.2020.05.017. Epub 2020 Jun 5.
Behcet's disease is a systemic disorder with ocular, mucocutaneous, and vascular involvement. The efficacy of tumor necrosis factor inhibition has been established; however, that of interluekin-6 inhibition is unclear. We investigated the effectiveness of tocilizumab, an anti-interleukin-6 receptor antibody, in patients with Behcet's disease.
We performed a systematic literature review from the inception dates until April 10, 2020 for articles reporting tocilizumab administration for the treatment of Behcet's disease.
We identified 47 patients with Behcet's disease treated with tocilizumab. The mean age at tocilizumab administration was 36.9 years, and 55% of the patients were female. The mean disease duration was 99.5 ± 61.4 months, and all patients had refractory disease in response to prior conventional and biologic agents. Clinical response to tocilizumab varied based on the target organs. Tocilizumab improved almost all patients with ocular (24/25), neurological (6/6), and vascular (7/7) involvement, as well as secondary amyloidosis (2/2). Tocilizumab was also effective in reducing glucocorticoid dose in almost all patients with ocular (19/21), neurological (6/6), and vascular (7/7) involvement. Further, glucocorticoid-free remission was achieved in 11 of 21 patients with ocular involvement and in 3 of 6 with neurological involvement. However, tocilizumab was not very effective for other manifestations, showing clinical improvement in only 8 of 21 patients with oral or genital ulcers, 6 of 14 with skin involvement, 4 of 11 with articular involvement and 3 of 4 with gastrointestinal involvement. Of note, tocilizumab even worsened oral or genital ulcers in 6 of 21 patients and skin lesion in 2 of 15 patients. No new safety signal was reported.
Tocilizumab was effective and can serve as an alternative treatment for refractory ocular-, neuro-, and vasculo-Behcet's disease, as well as secondary amyloidosis, but was not recommended for patients with mucocutaneous and articular involvement.
白塞病是一种全身性疾病，涉及眼部、黏膜皮肤和血管。肿瘤坏死因子抑制的疗效已经确立；然而，白细胞介素-6 抑制的疗效尚不清楚。我们研究了抗白细胞介素-6 受体抗体托珠单抗在白塞病患者中的疗效。
我们从成立日期到 2020 年 4 月 10 日进行了系统的文献回顾，以检索报告托珠单抗治疗白塞病的文章。
我们共纳入 47 例接受托珠单抗治疗的白塞病患者。托珠单抗治疗时的平均年龄为 36.9 岁，55%的患者为女性。平均疾病病程为 99.5±61.4 个月，所有患者均对既往常规和生物制剂治疗无效。托珠单抗对不同靶器官的临床反应不同。托珠单抗改善了几乎所有眼部（24/25）、神经（6/6）和血管（7/7）受累以及继发淀粉样变性（2/2）患者的疾病。托珠单抗还能有效减少几乎所有眼部（19/21）、神经（6/6）和血管（7/7）受累患者的糖皮质激素剂量。此外，21 例眼部受累患者中有 11 例、6 例神经受累患者中有 3 例达到无糖皮质激素缓解。然而，托珠单抗对其他表现并不十分有效，仅有 21 例口腔或生殖器溃疡患者中的 8 例、14 例皮肤受累患者中的 6 例、11 例关节受累患者中的 4 例和 4 例胃肠道受累患者中的 3 例出现临床改善。值得注意的是，托珠单抗甚至使 21 例患者中的 6 例口腔或生殖器溃疡和 15 例患者中的 2 例皮肤病变恶化。未报告新的安全信号。
托珠单抗对白塞病的难治性眼部、神经和血管病变以及继发淀粉样变性有效，可作为替代治疗方法，但不推荐用于黏膜皮肤和关节受累患者。
