Background Behçet disease is considered a systemic vasculitidis-like process that can express with different clinical phenotypes. Anti TNFs agents are a well established therapy for patients with refractory Behçet disease, particularly when severe visual involvement or life threatening complications are present.
Objectives To evaluate different clinical phenotypes, other than severe ocular-neuro Behçet, which required an anti-TNF immunosoppressive therapy to obtain Behçet disease control (arthritis-mucocutaneous Behcet, entero-Behcet, vascular-Behçet).
Methods We retrospectively evaluated clinical data of 93 patients (51 females, 42 males) who had been referred to our Center for overt or suspected Behçet disease over the last 10 years. Patients were hence divided into 2 groups: 75 patients fulfilling the International Study Group (ISG) criteria for Behçet syndrome (group 1) and 18 patients who, although not meeting international criteria, were considered to have a clinical presentation suggestive of “incomplete” Behçet disease (group 2).
Results 25/93 of patients (26%) were treated with an anti TNF agent in both the groups. In group 1 22/75 (29%) patients had been treated with at least one biological agent during the disease course. Fig. 1 describes the predominant Behçet phenotype that determined the choice of starting an anti-TNF therapy in group 1. Only 3/18 (16%) patients in group 2 were treated with biological therapy: 1 with etanercept (spondyloarthritis overlap) and 2 with adalimumab (recurrent superficial thrombophlebitis). Both Infliximab and adalimumab were well tolerated and lead to optimal disease control, since switching from one biological agent to another for therapeutic failure was necessary in less than 5% of patients.
Conclusions Infliximab and adalimumab were the most commonly used first line biological agents to treat severe or refractory Behçet patients. Infliximab was the first choice agent particularly when severe ocular or nervous system involvement were present. Over the last 5 years we have more commonly prescribed adalimumab to treat other protean features of Behçet syndrome, owing to good efficacy and its easier subcutaneous administration. Eye involvement was the main indication to start a biological anti-TNF therapy in patients with severe Behçet disease who have been followed at our center.
Disclosure of Interest: None Declared
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