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
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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.
Keywords
Behçet’s disease Etanercept Intestinal Behçet Multidrug therapy Pediatric-onset casesNotes
Acknowledgments
We thank Dr. N. Hanabata, Department of Gastroenterology, Hirosaki University Hospital, for performing the endoscopic studies.
Conflict of interest
The authors declare no conflicts of interest.
References
- 1.Kone-Paut I, Darce-Bello M, Shahram F et al (2011) Registries in rheumatological and musculoskeletal conditions. Paediatric Behçet’s disease: an international cohort study of 110 patients, On-year follow-up data. Rheumatology (Oxf) 50:184–188CrossRefGoogle Scholar
- 2.La Regina M, Gasparyan AY, Orlandini F, Prisco D (2010) Behçet’s disease as a model of venous thrombosis. Open Cardiovasc Med J 4:71–77PubMedGoogle Scholar
- 3.Saulsbury FT, Mann JA (2003) Treatment with infliximab for a child with Behçet’s disease. Arthritis Rheum 49:599–600PubMedCrossRefGoogle Scholar
- 4.Kaneko U, Kishi T, Kikuchi M et al (2010) Two patients with childhood-onset Behçet’s disease successfully treated by anti-tumor necrosis factor therapy. Jpn J Clin Immunol 33:157–161CrossRefGoogle Scholar
- 5.Matsumura K, Nakase H, Chiba T (2010) Efficacy of oral tacrolimus on intestinal Behçet’s disease. Inflamm Bowel Dis 16:188–189PubMedCrossRefGoogle Scholar
- 6.Sfikakis PP, Markomichelakis N, Alpsoy E et al (2007) Anti-TNF therapy in the management of Behçet’s disease—review and basis for recommendations. Rheumatology (Oxf) 46:736–741CrossRefGoogle Scholar
- 7.Giardina AR, Ferrante A, Ciccia F, Vadala M, Giardina E, Triolo G (2011) One year study of efficacy and safety of infliximab in the treatment of patients with ocular and neurological Behçet’s disease refractory to standard immunosuppressive drugs. Rheumatol Int 31:33–37PubMedCrossRefGoogle Scholar
- 8.Aikawa NE, Gonçalves C, Silva CAA, Gonçalves C, Bonfa E, de Carvalho JF (2011) Late response to anti-TNF-α therapy in refractory mucocutaneous lesions of Behçet’s disease. Rheumatol Int 31:1097–1099PubMedCrossRefGoogle Scholar
- 9.Studer U, Ruehe B, Waldegg G, Vajtai I, Escher R, Aeberli D (2012) Atypical presentation of Behçet’s disease with central nervous system involvement successfully treated with infliximab. Rheumatol Int 32:1431–1435PubMedCrossRefGoogle Scholar
- 10.Seyahi E, Hamurydan V, Hatemi G et al (2007) Infliximab in the treatment of hepatic vein thrombosis (Budd–Chiari syndrome) in three patients with Behçet’s disease. Rheumatology (Oxf) 46:1213–1214CrossRefGoogle Scholar
- 11.Evereklioglu C, Borlu M (2008) Sustained remission after infliximab in a child with vasculitis refractory to conventional immunosuppressives including interferon-α. Br J Ophthalmol 92:1148–1149CrossRefGoogle Scholar
- 12.Cantarini L, Tinazzi I, Caramaschi P, Bellisai F, Brogna A, Galeazzi M (2009) Safety and efficacy of etanercept in children with juvenile-onset Behçet’s disease. Int J Immunopathol Phamacol 22:551–555Google Scholar
- 13.Robinson AB, Gallentine WB, Rabinovich CE (2010) Pediatric neuro-Behçet’s disease responsive to adalimumab. Pediatr Neurol 43:291–293PubMedCrossRefGoogle Scholar
- 14.Keogan MT (2009) Clinical immunology review series: an approach to the patient with recurrent orogenital ulceration, including Behçet’s syndrome. Clin Exp Immunol 156:1–11PubMedCrossRefGoogle Scholar
- 15.Watanabe S, Tsuruga K, Aizawa-Yashiro T, Takahashi T, Ito E, Tanaka H (2011) Successful multidrug treatment of a pediatric case of severe Churg–Strauss syndrome refractory to prednisolone. Tohoku J Exp Med 225:117–121PubMedCrossRefGoogle Scholar
- 16.Takase K, Ohno S, Ideguchi H, Uchio E, Takeno M, Ishigatsubo Y (2011) Successful switching to adalimumab in an infliximab-allergic patient with severe Behçet’s disease-related uveitis. Rheumatol Int 31:243–245PubMedCrossRefGoogle Scholar