Objective: 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. Methods: 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. Results: 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. Conclusion: 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.
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