Effectiveness and Tolerability of Anti-Tumor Necrosis Factor Alpha Therapy in Refractory Intestinal Behçet's Disease: A Large Single-Center Study.

Background/Aims: Intestinal Behçet's disease (BD) is a rare, chronic intestinal vascular disorder often refractory to conventional therapy. We aimed to assess the effectiveness and tolerability of anti-tumor necrosis factor alpha (anti-TNF-α) therapy in patients with moderate to severe refractory intestinal BD. Methods: Clinical remission, clinical response, and biological response rates at 4, 12, and 24 months, as well as the adverse effects of anti-TNF-α therapy were investigated at the Inflammatory Bowel Disease Center of Severance Hospital, Seoul, Korea. We also examined the relapse rates and predictive factors for disease relapse. Results: Of the 119 patients, 15 (12.6%) were bio-exposed, 68 (57.1%) received concomitant immunomodulators, and 56 (47.1%) received concomitant corticosteroids at anti-TNF-α treatment induction. At 4, 12, and 24 months, clinical remission rates were 23.5%, 40.3%, and 42.0%; clinical response rates were 84.0%, 62.2%, and 62.2%; and biological response rates were 61.3%, 68.9%, and 58.8%, respectively. Sixty-three patients (52.9%) relapsed, with a mean relapse time of 2.8 years. Higher initial C-reactive protein levels (hazards ratio [HR], 1.013; 95% confidence interval [CI], 1.008 to 1.018; p<0.001), history of previous intestinal surgery (HR, 4.282; 95% CI, 2.379 to 7.709; p<0.001), concomitant immunomodulator use (HR, 0.455; 95% CI, 0.267 to 0.775; p=0.004), and clinical response at 4 months (HR, 0.353; 95% CI, 0.181 to 0.687; p=0.002) were independent factors associated with the disease relapse. No mortality was observed during the study period; 26 (21.8%) and three patients (2.5%) experienced mild infection and infusion reactions, respectively. Conclusions: Anti-TNF-α therapy could be an effective and tolerable option for refractory intestinal BD.