POS0820 INFLIXIMAB FOR VASCULAR INVOLVEMENT IN BEHÇET SYNDROME

Abstract

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 usePulmonary artery aneurysm and/or thrombosis, n=29Peripheral artery aneurysm, n=4Aortic aneurysm, n=3Vena cava superior and/or inferior thrombosis, n=5Lower extremity DVT, n=24Budd-Chiari syndrome, n=4Intracardiac thrombosis, n=2Dural sinus thrombosis, n=7Leg ulcer, n=5Previous treatmentFirst line infliximab413Corticosteroids3438Cyclophosphamide (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-13Azathioprine2117Mycophenolate Mofetil12Cyclosporine A-2None due to non-compliance21OutcomeRemission, still on infliximab1623Discontinued due to remission48Discontinued due to adverse events59Discontinued due to relapse53Discontinued due to non-compliance53Died2 (1 had previously discontinued due to relapse)1Disclosure of Interests:None declared