POS0814 OUTCOME OF VASCULAR INVOLVEMENT OF BEHÇET’S SYNDROME TREATED WITH INFLIXIMAB: A RETROSPECTIVE COHORT STUDY

Abstract

BackgroundVascular involvement is the most common cause of mortality and an important cause of disability in patients with Behçet’s syndrome (BS). Cyclophosphamide has been the treatment choice for severe vascular involvement, but high frequency of adverse events such as infertility and infections cause concern. TNF inhibitors can be an alternative for BS patients with vascular involvement.ObjectivesTo survey the efficacy and safety of infliximab (IFX) in BS patients with arterial and venous vascular involvement.MethodsWe reviewed the charts of BS patients who used IFX for vascular involvement. We extracted data on demographic and clinical features, type of vascular involvement, laboratory tests, imaging modalities, concomitant immunosuppressives, duration of IFX use, and outcome. The primary endpoint was remission, defined as the presence of all of the following 3 parameters: 1) lack of new clinical symptoms/findings associated with the vascular lesion 2) normalization of CRP level defined as <10 mg/dl) 3) lack of worsening of the primary vascular lesion or a new lesion vascular at another site on imaging. Remission was assessed at month 6 and month 12. Secondary endpoints were relapse, overall disease activity assessed with BDCAF at baseline and at the final visit, development of new organ involvement other than vascular involvement during IFX treatment, severe adverse events leading to discontinuation of IFX therapy, hospitalization or death, and death.ResultsAmong the 371 patients who used IFX between 2004 and June 2021, 127 patients (102 men, 25 women, mean age 40 ± 8.7 years) had used it for vascular involvement. The types of vascular involvement that required IFX were venous thrombosis in 61 patients (48%), pulmonary artery involvement in 37 (29%), non-pulmonary artery involvement in 16 (13%), and venous ulcer in 13 (10%). Remission rate was 72% (92/127) at month 6 and 61% (71/117) at month 12. 17/99 (17%) patients experienced 22 relapses during a mean follow-up of 28.4±21 months of IFX therapy. Among the 22 relapses, 12 were the progression of the pre-existing vascular lesion and 10 were new vascular lesions. Overall disease activity improved with a decrease in mean BDCAF score from 1.76 ± 1.27 to 0.6 ± 0.8 at the final visit (p<0.001). Remission and relapse rates according to type of vascular involvement and causes of IFX discontinuation are presented in the Table 1. Adverse events leading to IFX discontinuation were infusion reactions in 5, tuberculosis, disseminated zona, lung adenocarcinoma, fibromyxoid sarcoma, heart failure, SLE, palmoplantar pustulosis, auricular chondritis, and aortic stent graft infection in 1 patient each.Table 1.The frequency of concomitant immunosuppressive use, duration of infliximab use and outcomes of BS patients with vascular involvement treated with IFXVenous thrombosis (n=61)Pulmonary artery involvement (n=37)Non-pulmonary arterial involvement (n=16)Venous ulcers (n=13)Overall (n=127)Number of patients who used concomitant immunosuppressives48 (79)24 (65)14 (87)7 (54)93 (73)Duration of IFX use (mean ± SD months)24 ± 19.725 ± 19.335 ± 29.626 ± 2425 ± 21Remission rate at month 650 (82)31 (84)10 (63)1 (8)92 (72)Remission rate at month 12a40 (70)21 (64)8 (53)2 (17)71 (60)Relapse rate4 (7)4 (11)9 (60)017 (13)Number of patients who discontinued IFX31 (51)23 (62)5 (31)9 (69)68 (54)Due to remission1560122Due to inefficacy313411Due to relapse10102Due to adverse event741113Due to noncompliance340310Due to new organ development10001Due to other reasonsb18009Death22004a Since 10 patients did not reach the 12th month yet, the percentages were calculated on 117 patients.b Other reasons were preparation for surgical operation (n=2), not wanting to come to the infusion frequently during the pandemic (n=2), pregnancy (n=1), willing to get pregnant (n=1), lack of health insurance (n=1), due to prison sentence (n=1), and death (n=1).ConclusionInfliximab may be beneficial in BS patients with vascular involvement, even in those who are refractory to immunosuppressives and corticosteroids.Disclosure of InterestsGulen Hatemi Speakers bureau: Gulen Hatemi has served as a speaker for AbbVie, Celgene, Novartis, and UCB Pharma, Grant/research support from: Gulen Hatemi has received grant/research support from Celgene, Beyza Tukek: None declared, Sinem Nihal Esatoglu Speakers bureau: Sinem Nihal Esatoglu has received honorariums for presentations from UCB Pharma, Roche, Pfizer, and Merck Sharp Dohme., Yesim Ozguler Speakers bureau: Yesim Ozguler has received honorariums for presentations from UCB Pharma, Novartis, and Pfizer., Sitki Safa Taflan: None declared, Melike Melikoglu: None declared, Serdal Ugurlu: None declared, Izzet Fresko: None declared, Zekayi Kutlubay: None declared, Sebahattin Yurdakul: None declared, Hasan Yazici: None declared, Vedat Hamuryudan Speakers bureau: Vedat Hamuryudan has served as a speaker for AbbVie, Celgene, Novartis, and UCB Pharma, Grant/research support from: Vedat Hamuryudan has received grant/research support from Celgene.