title: A young girl with refractory intestinal Behçet's disease: a case report and review of literatures on pediatric cases who received an anti-tumor necrosis factor agent.
abstract: Behçet's disease (BD) is a chronic, relapsing, multisystem inflammatory disorder classified as vasculitis and characterized by recurrent oral and genital ulcerations, uveitis, and protean clinical signs of skin, central nervous system, musculoskeletal, and gastrointestinal involvements. Among the protean manifestations due to BD, intestinal BD is often intractable, but effective treatment for intestinal BD has not been fully established. Tumor necrosis factor (TNF)-α plays a central role in inflammation in BD patients; however, there are very few reports on the successful treatment of pediatric-onset cases of intestinal BD using anti-TNF-α agents. We report the case of a 6-year-old Japanese girl with refractory intestinal BD who was successfully treated with multidrug therapy including etanercept (ETN). Also, we performed a review of literatures on pediatric cases who received an anti-TNF-α agent. To our knowledge, this is the youngest patient with intestinal BD who was successfully treated using ETN. Although further studies are needed to determine the efficacy and safety of anti-TNF-α agents in the treatment for pediatric-onset BD, we believe that even in very young patients with refractory BD, an anti-TNF-α agent may be beneficial.
authors.affiliation: Department of Pediatrics, Hirosaki University Hospital, Hirosaki, 036-8563, Japan.
authors.forename: Shojiro
authors.initials: S
authors.lastname: Watanabe
authors.forename: Tomomi
authors.initials: T
authors.lastname: Aizawa-Yashiro
authors.forename: Kazushi
authors.initials: K
authors.lastname: Tsuruga
authors.forename: Manabu
authors.initials: M
authors.lastname: Kinjo
authors.forename: Etsuro
authors.initials: E
authors.lastname: Ito
authors.forename: Hiroshi
authors.initials: H
authors.lastname: Tanaka
mesh_terms: D000367:Age Factors; D001528:Behcet Syndrome / Q000188:drug therapy*; D002675:Child, Preschool; D000068800:Etanercept; D005260:Female; D006801:Humans; D007074:Immunoglobulin G / Q000627:therapeutic use*; D007166:Immunosuppressive Agents / Q000627:therapeutic use; D018124:Receptors, Tumor Necrosis Factor / Q000627:therapeutic use*; D017211:Treatment Failure; D016896:Treatment Outcome; D014409:Tumor Necrosis Factor-alpha / Q000037:antagonists & inhibitors*
chemical_list: D007074:Immunoglobulin G; D007166:Immunosuppressive Agents; D018124:Receptors, Tumor Necrosis Factor; D014409:Tumor Necrosis Factor-alpha; D000068800:Etanercept