Background:Vascular involvement is one of the leading causes of mortality and morbidity in Behcet’s Disease (BD)1. Surgical treatments are difficult for Vaculo-BD (VBD) patients due to the high risk of serious postoperative complications without effective and promptly perioperative immunotherapy2, 3. Anti-tumor necrosis factor alpha (TNF-α) therapy has been reported as a potential treatment in severe VBD, e.g. infliximab (IFX) and adalimumab (ADA). However, only few case reports are available regarding the fully humanized monoclonal antibody to TNF-α, golimumab (GOL), in the management of VBD4.Objectives:The objective of this study was to report the efficacy and safety of GOL for the treatment of severe and/or refractory VBD.Methods:We retrospectively analyzed the efficacy and safety profile of patients with severe and/or refractory VBD treated with GOL in our medical center between 2018 to 2020.Results:Nine VBD patients (8 male and 1 female) were enrolled, with a mean age and median course of 37±8.6 years and 72 months (range 12 to 300), respectively. Cardiac involvements (severe aortic regurgitation secondary to BD) were presented in 7 patients, including 2 patients with post-operative paravalvular leakage (PVL) after aortic valve replacement surgery. Multiple vascular lesions were documented in the other 2 patients, including one patient with life-threatening multiple pulmonary aneurysms, pulmonary thromboembolism and recurrent deep vein thrombosis, and another patient with abdominal aortic pseudoaneurysm and multiple artery stenosis and occlusion. Prior to GOL therapy, all patients experienced disease progression despite high-dose glucocorticoids combined with multiple immunosuppressants. Moreover, seven patients required effective and fast control of inflammation and a decrease of glucocorticoid dose during the perioperative period. They were treated with GOL, 50mg 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 mean duration of follow-up of 10 (range 2-6) months, all patients achieved improvement both in clinical symptoms and serum inflammation markers. The ESR level [4.88±4.94 mm/h vs 31.13±31.78mm/h, P<0.01] and CRP level [1.9 (0.11-3.73)mg/L vs 24.3 (0.4-85.57)mg/L, P<0.01] significantly decreased. The dosage of glucocorticoid[10 (0-15) vs 40 (0-100)mg/d, P<0.01] effectively tapered, indicating a potential steroid-sparing effect. No newly-onset aneurysm and recurrent venous thrombosis were observed. Also, one patient had a marked reduction in size and number of pulmonary aneurysms. No post-operative PVL was observed in the five patients after Bentall operation with a median follow-up of 10 months. One patient with severe aortic regurgitation remained stable and without surgical intervention with the treatment of GOL for 16 months. No severe complication occurred in one patient after underwent endovascular repair of abdominal aorta for 8 months. GOL was well-tolerated, and no serious adverse event was observed.Conclusion:Our results suggested that GOL is safe and effective for the treatment of patients with severe and / or refractory VBD. Further controlled studies are warranted to confirm the therapeutic potential of GOL in VBD patients.Disclosure of Interests:None declared
SEVERE AORTIC REGURGITATION PRODUCED BY MITRAL VALVE REPLACEMENT: MECHANISMS AND CHOICES
