Abstract
Behçet’s disease (BD) is a chronic relapsing disease with multiple organ system involvement characterized clinically by oral and genital aphthae, cutaneous lesions, and ophthalmological, neurological, and/or gastrointestinal manifestations. Little clinical evidence is available regarding the management of patients with intestinal BD, despite recognition that the presence of intestinal lesions is a poor prognostic factor, causing perforation and massive bleeding. Many recent case reports have suggested that anti-tumor necrosis factor alpha (TNF)α monoclonal antibodies (mAbs) are effective in patients with intestinal BD. Adalimumab, a fully human anti-TNFα mAb, has been approved in Japan for the treatment of intestinal BD. Here, we review the pathogenesis, diagnosis and management of intestinal BD, including evidence of the efficacy of anti-TNFα mAbs.
Highlights
Behcet’s disease (BD) was first defined in 1937 by Hulusi Behcet [1], a Turkish dermatologist, as a triad of recurrent aphthous stomatitis, genital aphthae and relapsing uveitis
Little clinical evidence is available regarding the management of patients with intestinal BD, despite recognition that the presence of intestinal lesions is a poor prognostic factor, causing perforation and massive bleeding
Many recent case reports have suggested that anti-tumor necrosis factor alpha (TNF)a monoclonal antibodies are effective in patients with intestinal BD
Summary
Behcet’s disease (BD) was first defined in 1937 by Hulusi Behcet [1], a Turkish dermatologist, as a triad of recurrent aphthous stomatitis, genital aphthae and relapsing uveitis. Treatment with infliximab (IFX) of two patients with intestinal BD resistant to conventional therapy, including prednisolone, one with 3 mg/kg and the other with 5 mg/kg IFX, resulted in the rapid (within 10 days) reduction of intestinal lesions and extraintestinal manifestations [53] Remission in both patients was maintained with thalidomide, not IFX. A clinical trial has tested IFX for intestinal BD in Japan, and the second edition of consensus statements for the diagnosis and management of intestinal BD has proposed anti-TNFa mAb as a standard therapy for patients with moderate to severe intestinal BD [68]. Subgroup analysis of the CHARM trial showed that ADA was superior to placebo in maintaining clinical remission in patients with moderately to severely active CD after 1 year of treatment, regardless of disease duration [74].
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