POS0820 INFLIXIMAB FOR VASCULAR INVOLVEMENT IN BEHÇET SYNDROME
Authors: G. Hatemi, B. Tukek, S. N. Esatoglu, Y. Ozguler, M. Melikoglu, E. B. Caliskan, S. Ugurlu, I. Fresko, S. Yurdakul, H. Yazici, V. Hamuryudan
Journal: Annals of the Rheumatic Diseases (2021)

Background: Vascular involvement is an important cause of morbidity and mortality in patients with Behçet syndrome (BS). TNF inhibitors have been reported to be effective for almost all serious manifestations of BS but data on vascular involvement is still limited. Objectives: To survey the efficacy and safety of infliximab (IFX) in BS patients with vascular involvement followed in a dedicated tertiary center. Methods: We reviewed the charts of all BS patients who used IFX and identified those who used this drug for vascular involvement. A standard form was used for extracting data on demographic and clinical features, type of vascular involvement, concomitant immunosuppressives, duration of IFX use, relapses, adverse events and outcome. Results: 83 patients (67 men, 16 women, mean age 39.5 ± 8.4 SD years) had used IFX for vascular involvement. The number of patients with each type of vascular involvement as the main indication for IFX, and previous treatment modalities right before IFX are presented in the Table 1. 33 patients had more than 1 type of vascular lesion. Additional organ manifestations were eye involvement in 32, central nervous system involvement in 4 and gastrointestinal involvement in 3 patients. All but one patient used IFX at a dose of 5 mg/kg every 6-8 weeks. All patients used concomitant prednisolone and 51 received pulse methylprednisolone during induction. Other concomitant medications were azathioprine in 21 and mycophenolate mofetil in 4 patients. Outcome of IFX treatment at the end of a mean follow-up of 25.9 months (range 1-103) is tabulated according to arterial and venous involvement (Table 1). Overall, 57/83 (68%) patients obtained remission with no further relapses and IFX could be stopped in 12 (14%) of them. IFX was discontinued due to adverse events in 15 patients. These were allergic reactions in 8, tuberculosis, disseminated zona, lung adenocarcinoma, fibromyxoid sarcoma, heart failure, systemic lupus erythematosus, and palmoplantar pustulosis in 1 patient each. Three patients had died. Causes of death were lung adenocarcinoma in one patient and pulmonary hypertension related right heart failure due to pulmonary artery thrombosis in 2 patients. Conclusion: Infliximab seems to be beneficial in Behçet syndrome patients with vascular involvement, even in those who are refractory to immunosuppressives and corticosteroids. No further relapses occurred in 68% of the patients, and adverse events leading to discontinuation were observed in 18%. Arterial Involvement (n=36 ) Venous Involvement (n=47 ) Main reason for infliximab use Pulmonary artery aneurysm and/or thrombosis, n=29 Peripheral artery aneurysm, n=4 Aortic aneurysm, n=3 Vena cava superior and/or inferior thrombosis, n=5 Lower extremity DVT, n=24 Budd-Chiari syndrome, n=4 Intracardiac thrombosis, n=2 Dural sinus thrombosis, n=7 Leg ulcer, n=5 Previous treatment First line infliximab 4 13 Corticosteroids 34 38 Cyclophosphamide (CYC) 25 (15/25 refractory to CYC; 10/25 for maintenance after CYC) 11 (8/11 refractory to CYC; 3/11 for maintenance after CYC) Interferon-alpha - 13 Azathioprine 21 17 Mycophenolate Mofetil 1 2 Cyclosporine A - 2 None due to non-compliance 2 1 Outcome Remission, still on infliximab 16 23 Discontinued due to remission 4 8 Discontinued due to adverse events 5 9 Discontinued due to relapse 5 3 Discontinued due to non-compliance 5 3 Died 2 (1 had previously discontinued due to relapse) 1 Disclosure of Interests: None declared