Background/Purpose: In Behçet’s disease (BD), vascular complications affect more than 30% patients. Cardiovascular involvement is the main cause of death, especially for pulmonary or aortic aneurysms and large vein thrombosis. Immunosuppressants (azathioprine, methotrexate, cyclophosphamide) are not always effective and are often associated with significant side effects. TNF antagonists have been shown to be very quickly effective in severe ocular manifestations of BD. Data on their efficacy in BD vascular complications are scarce. Objectives: To evaluate the efficacy and safety of anti-TNF alpha in severe and/or refractory vascular involvements in BD.

Methods: A multicenter and observational study evaluating 18 patients with severe BD associated- cardiac or vascular disease (defined by international standards) treated with anti-TNF [infliximab (n=15) and adalimumab (n=3)]. Anti-TNF alpha antibodies were initiated because of severe aneurysmal arterial disease [lung (n=4) or the aorta/one of its branches (n=4) or peripheral (n=1)] and/or large vein thrombosis [pulmonary artery (n=7), thrombosis of the inferior vena cava (n = 4) or Budd-Chiari syndrome (n=3)] and/or cardiac involvement [aortic valve disease (n=1), left ventricular aneurysm (n=1), myocardial ischemia (n=1) or intra-cardiac thrombosis (n=2)]. Clinical remission was defined by resolution of clinical and biological symptoms and the absence of occurrence of new vascular lesions or worsening of existing vascular lesions.

Results: Eighteen patients [(89% male) with mean age of 30 [10; 55] years] were included. Fourteen (78%) were refractory to immunosuppressants (n=12) and / or high doses of systemic corticosteroids (n=2). Fifteen patients received infliximab injections [5mg / kg (n=13) and 3mg / kg (n=2)] and 3 adalimumab [40mg / 15 days]. Fourteen patients also received immunosuppressants [azathioprine (n=7), methotrexate (n=5), mycophenolate mofetil (n=2)] and 17 corticosteroids associated with anti-TNF alpha. Vascular lesions observed (before or at the same time of anti-TNF initiation) included: arterial aneurysms (n=11), [pulmonary (n=7), aortic (n=3), spleen (n=1) or peripheral (n = 3)], arterial occlusions (n=3) and / or venous thrombosis [pulmonary embolism (n=9), lower vena cava thrombosis (n=7), deep venous thrombosis of lower limbs (n = 2) or Budd-Chiari syndrome (n=4)]. Eight patients also had cardiac involvement [intracardiac thrombosis (n=4), valvulopathy (n=2), myocardial ischemia (n=2) or pericardial effusion (n=1)]. Vascular remission was achieved in 16 patients (89%), partial (n=3) or complete (n=13). No patients died and 2 relapsed under TNF-antagonists. Two patients who stopped anti-TNFα antibodies [side effect (n=1) and poor compliance (n=1)] had neurological or vascular flare 3 and 5 months after treatment cessation, respectively. Side effects were observed in 4 patients [pulmonary edema (n=1) infection (n=3)], requiring discontinuation in 2 patients. After a median follow up of 15 [4, 164] months, 16/18 were still under anti-TNF [cessation for side effects (n=2)]. The median dose of corticosteroids was significantly decreased in all patients at 12 months after treatment initiation [30 mg initially versus 6 mg, p=0.004]. At the end of follow-up, 94% achieved a dose of ≤10mg corticosteroids.

Conclusion: Anti TNFα antibodies represent an effective and safe treatment in 89% of BD patients with severe and refractory cardiovascular involvement. Results: Conclusion:

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ACR Meeting Abstracts - https://acrabstracts.org/abstract/efficacy-of-anti-tnf-alpha-in-severe-and-refractory-cardiovascular-involvement-of-behcets-disease-a-multicenter-observational-study-of-18-patients/