Treatment of intestinal Behcet's syndrome with chimeric tumour necrosis factor alpha antibody

Few patients with Behcet's syndrome have gastrointestinal ulceration. Such patients are difficult to treat and have a higher mortality. Faced with ref ractory symptoms in two patients with intestinal Behcet's, we used the tumo ur necrosis factor alpha (TNF-alpha) monoclonal antibody infliximab to indu ce remission. Both women (one aged 27 years, the other 30 years) presented with orogenital ulceration, pustular rash, abdominal pain, bloody diarrhoea due to colonic ulceration, weight loss, and synovitis. One had thrombophle bitis, digital vasculitis, perianal fistula, and paracolic abscess; the oth er had conjunctivitis and an ulcer in the natal cleft. Treatment with predn isolone, methyl prednisolone, and thalidomide in one and prednisolone, colc hicine, and cyclosporin in the other was ineffective. After full discussion , infliximab (3 mg/kg, dose reduced because of recent sepsis in one, and 5 mg/kg in the other) was administered. Within 10 days the ulcers healed, wit h resolution of bloody diarrhoea and all extraintestinal manifestations. A second infusion of infliximab was necessary eight weeks later in one case, followed by sustained (> 15 months) remission on low dose thalidomide. Remi ssion was initially sustained for 12 months in the other but thalidomide ha d to be stopped due to intolerance, and a good response to retreatment last ed only 12 weeks without immunosuppression, before a third infusion. The ca use of Behcet's syndrome is unknown but peripheral blood CD45 gamma delta T cells in Behcet's produce > 50-fold more TNF-alpha than controls when stim ulated with phorbol myristate acetate and and-CD3. Infliximab could have a role for inducing remission in Behcet's syndrome.