Efficacy of Anti-Tumor Necrosis Factor-α Therapy Against Intestinal Behçet’s Disease Complicated by Recurrent Enterocutaneous Fistulae

Abstract

A 55-year-old man presented with recurrent ulcers and an enterocutaneous fistula at the anastomotic site after surgery for an ileovesical fistula and was diagnosed with intestinal Behçet's disease after undergoing surgery for enterocutaneous fistulae twice. The patient was transferred to our hospital because of recurrent enterocutaneous fistulae. He had a history of recurrent oral aphthous ulcers, folliculitis, and epididymitis and met the diagnostic/classification criteria for incomplete Behçet's disease and thus was diagnosed as having intestinal Behçet's disease. Remission induction therapy with steroids was administered for an ileal ulcer and an enterocutaneous fistula, and adalimumab was initiated for maintenance therapy. The fistula was closed, and the clinical course was favorable. Two months after initiating adalimumab, a subcutaneous abscess was detected at the site of the enterocutaneous fistula scar, and relapse of intestinal Behçet's disease was suspected. Steroids were re-administered for remission induction, followed by maintenance therapy, for which adalimumab was switched to infliximab. No relapse was detected after steroid withdrawal. No therapeutic strategies have been established for intestinal Behçet's disease. Moreover, there have been very few reports on therapeutic strategies and postoperative maintenance therapy for enterocutaneous fistulae. We thus consider this case valuable.

Entero-Behcet: A Challenging Aspect of Behçet’s Disease

“…Anti-TNF-α antibodies were also effective in preventing postoperative recurrence with a recurrence-free state for 2-3 years. Studies have reported that infliximab at 5 mg/kg is effective in improving ulcers and subsequently maintaining remission in patients with ulcers occurring at the anastomotic site within 2 weeks after ileoceocal resection who fail to respond to conventional therapy with prednisolone, azathioprine, mesalamine, and colchicine [25].…”