She received subcutaneous injections of adalimumab at a dose of 160?mg in week 0, 80?mg in week 2, and 40?mg every other week thereafter. adalimumab was used for pediatric gastrointestinal Behcet’s disease. Adalimumab is a good choice for intestinal Behcet’s disease refractory to conventional treatment. 1. Introduction Behcet’s disease is a chronic relapsing vasculitis that is characterized by recurrent oral aphthous and genital ulcers with uveitis [1]. In addition to the main symptoms, there are some much less common manifestations, such as gastrointestinal or central nervous system disease. Pediatric Behcet’s disease occurs in approximately 0.3 cases per 100,000 amongst Japanese children [2]. Although the etiology of Behcet’s disease is still unknown, studies have revealed an association between tumor necrosis factor-alpha (TNF-monoclonal antibody. She received subcutaneous injections of adalimumab at a dose of 160?mg in week 0, 80?mg in week 2, and 40?mg every other week thereafter. The symptoms and clinical manifestations resolved soon after the initiation of adalimumab. The RO462005 circulating inflammatory markers converted to negative. Initiation of the adalimumab made it possible to reduce the corticosteroid dose without any symptoms (Figure 4). Epithelialization of the ulcers was demonstrated on lower gastrointestinal endoscopy, which was performed 1 week after the initiation of adalimumab (Figure 3(b)). Although the oral corticosteroid dose was gradually decreased, the symptoms never recurred. No Rabbit Polyclonal to IRAK2 adverse effects of adalimumab were observed. Open in a separate window Figure 4 Clinical course of the patient. Corticosteroid pulse therapy with methylprednisolone was administered to the patient. After two courses of corticosteroid pulse therapy, the symptoms and blood test results showed improvement. The abdominal pain relapsed while tapering the dose of corticosteroid. The patient was begun on adalimumab at a dose of 160?mg in week 0, 80?mg in week 2, and 40?mg every other week thereafter. The clinical manifestations of Behcet’s disease resolved soon after the initiation of adalimumab. 3. Discussion The first case in which adalimumab was used for pediatric gastrointestinal Behcet’s disease has been reported. Behcet’s Disease Working Group of the Pediatric Rheumatoid Association of Japan reported that pediatric cases have more gastrointestinal manifestations (62% versus 23% in adults) and less uveitis (32% versus 61% in adults) than in adults. Because the frequency of RO462005 clinical features between adults and children is different, approximately one-half of the pediatric cases have not satisfied international diagnostic criteria, and many juvenile patients have never been diagnosed. The ulcers in patients with Behcet’s disease and gastrointestinal involvement are most commonly found in the terminal ileum and cecum. Sometimes it is difficult to distinguish Behcet’s disease with gastrointestinal involvement from inflammatory bowel diseases, such as Crohn’s disease. It was reported that 16 of 43 adults with RO462005 intestinal Behcet’s disease underwent surgery because of acute abdomens and/or massive bleeding [6]. Moreover, a history of frequent relapses is a risk factor for intestinal stenosis; thus, it is essential to achieve remission. The etiology of Behcet’s disease is still unknown. The strongest genetic marker in Behcet’s disease is HLABagents in the treatment of Behcet’s disease (Table 1 [10]). Although the mechanism of action is not completely clear, the following anti-TNF-agent findings are relevant: (1) neutralizing activity by binding to soluble/membrane-bound TNF-on T cells or monocytes; and (3) regulating the immune system via production of anti-inflammatory cytokines, inhibiting T cell amplification, and binding to macrophages or Fc receptors. Table 1 Summary of the highest level of evidence for immunosuppressants and anti-TNF biologics [10]. thead th align=”left” rowspan=”1″ colspan=”1″ Therapy /th th align=”left” rowspan=”1″ colspan=”1″ Level(s) of published evidence /th /thead 5-ASA/sulfasalazineRetrospective cohort study [13]CorticosteroidsExpert opinion, European League Against Rheumatism Recommendations [14]ThalidomideCase reports [15C17]Azathioprine, 6-MPRetrospective cohort studies [13]MycophenolateCase report [18]MethotrexateCase series [19]TacrolimusCase report [20]InfliximabSingle arm clinical trial [21]Retrospective cohort study [22]Case series [23]AdalimumabProspective, nonplacebo controlled clinical trial [24]EtanerceptCase report [25] Open in a separate window In gastrointestinal Behcet’s disease, infliximab and adalimumab are the two best studied biologic agents. Currently, etanercept has no role in the management of refractory intestinal Behcet’s disease in the recent guideline by the Japanese Inflammatory Bowel Disease Research Group though one case report was reported. The first reports of infliximab success in patients with glucocorticoid dependent Behcet’s disease were published in 2001 [26, 27]. A Korean multicenter retrospective study of infliximab with 28 intestinal Behcet’s disease patients demonstrated that older age at diagnosis ( 40 years), female sex, longer duration of disease ( 5 years), concomitant immunomodulator use, and achievement of remission within 4 weeks were predictive of sustained response.