Background/Purpose:

To report the efficacy and safety of tocilizumab(TCZ) for the treatment of severe and/or refractory Behçet’s disease(BD).

Methods:

We retrospectively analyzed the efficacy and safety profile of BD patients with severe and/or refractory BD treated with tocilizumab in our medical center between 2014 and 2017.

Results:

Ten BD patients (9 male and 1 female) were enrolled, with a mean age and median course of 34.6±8.6 year and 80 month (range 35 to132), respectively. Vascular, cardiac and neurological involvements were presented in 7, 2 and 1 patients, respectively. Of the seven vascular BD patients, pseudoaneurysms were presented in five patients, including one patients with recurrent venous thrombosis and arterial pseudoaneurysms， one patient with internal leakage after the stent placement of abdominal aortic pseudoaneurysm, one patient with coronary sinus rupture and pseudoaneurysm formation, two patients with aortic pseudoaneurysm and multiple artery stenosis or occlusion. Two patients with severe aortic regurgitation developed post-operative paravalvular leakage（PVL） after valve surgery. One neuro-BD patient presented with brainstem, spinal cord and peripheral involvements.

Prior to TCZ therapy, all patients experienced disease progression and elevated serum inflammation markers despite of high-dose glucocorticoids in combination with multiple immunosuppressants. They were then treated with TCZ, 8mg/kg every 4 weeks, in combination with background low- or medium-dose glucocorticoids and immunosuppressants, for a median of 6 (range 3-15) months.

After a median follow-up of 10 month (range 3 to 22), all patients achieved improvement both in clinical symptoms and serum inflammation markers（ESR and hsCRP）. Vascular lesions were stable and no recurrent aneurysm was observed. No PVL were observed in both patients with valvular involvement after repeated surgery at 6 and 8 months, respectively. The neuro-BD patient achieved both clinical and imaging improvement. The corticosteroid dose was tapering in most cases, indicating a potential steroid-sparing effect. TCZ was well-tolerated and no serious adverse event was observed.

Conclusion:

Our data suggest that TCZ is safe and effective for the treatment of patients with severe and / or refractory BD. Further controlled studies are warranted to confirm the therapeutic potential of TCZ in BD patients.

ACR Meeting Abstracts - https://acrabstracts.org/abstract/tocilizumab-in-the-treatment-of-severe-andor-refractory-behcets-disease%ef%bc%9aa-single-centre-experience-in-china/