AB0372 TOCILIZUMAB WAS EFFECTIVE IN REFRACTORY ARTERIAL INVOLVEMENT OF BEHCET’S DISEASE: A REAL-LIFE SINGLE- CENTER EXPERIENCE IN CHINA
Authors: H. Zhong, T. Liu, Y. Liu, X. Zhang, Y. Zhou, Y. Su
Journal: Annals of the Rheumatic Diseases (2021)

Background: Behcet’s disease (BD) is a chronic and relapsing vasculitis, in which major vessel involvement is a main cause of mortality and morbidity. The therapeutic arsenal is mainly composed of classical immunosuppressants. However, when faced with resistance to these drugs, no alternative therapeutic strategy is currently recommended. Objectives: To assess the efficacy and safety of interleukin 6 receptor inhibitor tocilizumab (TCZ) in refractory arterial involvement of BD in a real-life observational setting. Methods: 10 patients were enrolled in our center between 2014 and 2019. All patients met the international criteria for BD and had severe arterial involvement evaluated by echocardiography, angio-Computerized Tomography scan and vascular Doppler. The diagnosis of refractory arterio-BD was based on objective vascular symptoms not explained by any other known disease and non-response to conventional immunosuppressants combined with glucocorticoids therapy. All patients underwent TCZ infusions at 8mg/kg every 4 weeks. Concomitant therapy with immunosuppressants and glucocorticoids was continued. Clinical and imaging findings were assessed before and after TCZ therapy. All adverse events were recorded during follow-up. Results: All the patients were males, with a mean age of 44.3±10.5 years in this study. The mean age at presentation of arterial involvement was 40.8±9.2 years old. The patterns of arterial involvement were aneurysm (n=9), stenosis (n=3) and aortic valve lesion (n=2). After a mean follow-up of 26.8±7.2 months, TCZ yielded rapid and maintained clinical improvement in 9 patients, with complete remission in 6 of them and partial response in 3 of them. Discontinuation of TCZ treatment due to relapse occurred in one case as the enlargement of abdominal aortic aneurysm. The mean glucocorticoid dosage was tapered from 54.5±20.6mg/d to 8.3±3.6mg/d (p&lt;0.001). And the use of immunosuppressants was tapered in 4 (40.0%) patients. As for serological improvement, the median ESR and CRP levels decreased from 50 (2-82) mm/h and 32.9 (2.1-62.3) mg/dL to 4 (1-10) mm/h (p&lt;0.001) and 2.9 (0.2-12.1) mg/dL (p&lt;0.001), respectively. Radiologic improvement of artery lesion was demonstrated in 4(40%) patients. None of the patients had serious adverse events during follow-up. Conclusion: TCZ was a safe and effective therapeutic option for refractory arterial involvement of BD, with a favorable steroid-sparing effect. References: [1]G Hatemi, R Christensen, D Bang, et al. 2018 update of the EULAR recommendations for the management of Behçet’s syndrome. Ann Rheum Dis. 2018;77(6):808-818. [2]Y Ozguler, P Leccese, R Christensen, et al. Management of major organ involvement of Behcet’s syndrome: a systematic review for update of the EULAR recommendations. Rheumatology (Oxford). 2018;57(12):2200-2212. [3]M Akiyama, Y Kaneko, T Takeuchi. Effectiveness of tocilizumab in Behcet’s disease: A systematic literature review. Semin Arthritis Rheum. 2020;50(4):797-804. Table 1. Tocilizumab therapy in ten cases of refractory arterio-BD Patient Age, year Disease duration, months Clinical features * Arterial lesions Previous therapy Response at week 24 1 34 228 O, G, P, S, I Stenosis of CA/ SMA/RA/SCA, aortic valve prolapse Pred/CYC/MMF CR 2 20 24 O, S Dissecting aneurysm of AA (Debakey I) Pred/MMF PR 3 67 276 O, G, P, S, A thoracoabdominal aortic aneurysm Pred/CYC/TAC CR 4 67 75 O, S, U Stenosis of LAD/LCX/RCA Pred /CYC CR 5 50 80 O, G, A Abdominal and coronary aortic aneurysms Pred /CYC Relapse 6 48 26 O, G Aortic insufficiency Pred /CYC PR 7 26 147 O, P, S, V Iliac artery aneurysm Pred /MMF CR 8 49 466 O, S, V Thoracoabdominal and coronary aortic aneurysms Pred /CYC/AZA CR 9 27 181 O, P, S Pseudoaneurysm of CCA Pred /CYC PR 10 Male/55 354 O, P, S Abdominal aneurysm, stenosis of LAD/LCX/RCA Pred /CYC/AZA CR *O: oral ulcer; G: genital ulcer; P: pathergy test; S: skin lesions; I: intestinal ulcer; A: arthritis; U: uveitis; V: venous thrombosis Figure 1. Changes from baseline in BSAS, BDCAF, ESR, CRP and steroid daily dose at 24 weeks Disclosure of Interests: None declared