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Behçet's Syndrome pp 165–176Cite as

Pediatric Onset of Behçet Syndrome

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Part of the book series: Rare Diseases of the Immune System ((RDIS))

Abstract

Behçet syndrome (BS) is a vasculitis characterized by mucous ulcers, uveitis, and skin lesions. The vasculitis is unique affecting all sizes of vasculature in both the arterial and venous systems. BS is a complex genetic disease; recent genetic studies have confirmed the association with HLA-B51 and showed new associations with different inflammatory pathways. Environmental factors are also effective. BS frequently affects the gastrointestinal system and central nervous system as well. Current criteria for BS do not address the differences that exist in childhood BS cases. A multicenter approach is needed to get more pediatric data in order to establish pediatric criteria for the classification and activity of BS. The adult experience guides us in the treatment of pediatric disease.

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References

  1. Behçet H (1937) Über rezidivierende aphthöse, durch ein virus verusachte geschwüre am mund, am auge und an den genitalien. Dermatol Wochenschr 105:1151–1157

    Google Scholar 

  2. International study group for Behçet’s disease (1990) Criteria for diagnosis of Behçet’s disease. Lancet 335:1070–1080

    Google Scholar 

  3. Ohno S, Ohguchi M, Hirose S et al (1982) Close association of HLA-Bw51 with Behçet’s disease. Arch Ophthalmol 100:1455–1458

    Article  PubMed  CAS  Google Scholar 

  4. Davatchi F, Jamshidi A, Tehrani Banihashemi A et al (2007) Prevalence of Behçet’s disease in Iran: a WHO-ILAR COPCORD stage I study. APLAR J Rheumatol 10:239–43

    Google Scholar 

  5. Azizlerli G, Köse AA, Sarica R et al (2003) Prevalence of Behçet’s disease in Istanbul, Turkey. Int J Dermatol 42:803–806

    Article  PubMed  Google Scholar 

  6. Mahr A, Belarbi L, Wechsler B et al (2008) Population based prevalence study of Behçet’s disease: differences by ethnic origin and low variation by age at immigration. Arthritis Rheum 58:3951–3959

    Article  PubMed  Google Scholar 

  7. Zouboulis CC, Kotter I, Djawari D et al (1997) Epidemiological features of Adamantiades Behçet’s disease in Germany and in Europe. Yonsei Med J 38:411–422

    PubMed  CAS  Google Scholar 

  8. Azizleri G (2002) Juvenile Behçet’s syndrome. In: Ball GV, Bridges SL Jr (eds) Vasculitis. Oxford University Press, Oxford, pp 441–444

    Google Scholar 

  9. Ozen S, Karaaslan Y, Ozdemir O et al (1998) Prevalence of juvenile chronic arthritis and familial mediterranean fever in Turkey. A field study. J Rheumatol 25:2445–2449

    PubMed  CAS  Google Scholar 

  10. Koné-Paut I, Yurdakul S, Bahabri SA et al (1998) Clinical features of Behçet’s disease in children: an international collaborative study of 86 cases. J Pediatr 132:721–725

    Article  PubMed  Google Scholar 

  11. Tugal-Tutkun I, Urgancioglu M (2003) Childhood-onset uveitis in Behçet disease: a descriptive study of 36 cases. Am J Ophthalmol 136:1114–1119

    Article  PubMed  Google Scholar 

  12. Krause I, Uziel Y, Guedj D et al (1999) Childhood Behçet’s disease: clinical features and comparison with adult-onset disease. Rheumatology 38:457–462

    Article  PubMed  CAS  Google Scholar 

  13. Al Mosawi ZS, Madan W, Fareed E (2012) Pediatric-onset Behçet disease in Bahrain: report of nine cases and literature review. Arch Iran Med 15(8):485–487

    PubMed  Google Scholar 

  14. Fujikawa S, Suemitsu T (1997) Behçet disease in children: a nationwide retrospective survey in Japan. Acta Paediatr Jpn 39(2):285–289

    Article  PubMed  CAS  Google Scholar 

  15. Atmaca L, Boyvat A, Yalçındağ FN et al (2011) Behçet disease in children. Ocul Immunol Inflamm 19(2):103–107

    Article  PubMed  Google Scholar 

  16. Koné-Paut I (1999) Behçet’s disease: pediatric features. Ann Med Interne (Paris) 150:571–575

    Google Scholar 

  17. Akpolat T, Koç Y, Yeniay I et al (1992) Familial Behçet disease. Eur J Med 1:391–395

    PubMed  CAS  Google Scholar 

  18. Gul A, Insanu M, Ocal L et al (2000) Familial aggregation of Behçet’s disease in Turkey. Ann Rheum Dis 59:622–625

    Article  PubMed  CAS  Google Scholar 

  19. Ohno S, Aoki K, Sugiura S et al (1973) Letter: HL-A5 and Behçet’s disease. Lancet 2:1383–4

    Google Scholar 

  20. Ohno S, Asanuma T, Sugiura S et al (1978) HLA-Bw51 and Behçet’s disease. JAMA 240:529

    Article  PubMed  CAS  Google Scholar 

  21. Horie Y, Meguro A, Kitaichi N et al (2012) Replication of a microsatellite genome-wide association study of Behçet’s disease in a Korean population. Rheumatology (Oxford) 51(6):983–986

    Article  CAS  Google Scholar 

  22. Mizuki N, Inoko H, Mizuki N et al (1992) Human leukocyte antigen serologic and DNA typing of Behçet’s disease and its primary association with B51. Invest Ophthalmol Vis Sci 33:3332–3340

    PubMed  CAS  Google Scholar 

  23. Pineton de Chambrun M, Wechsler B, Geri G et al (2012) New insights into the pathogenesis of Behçet’s disease. Autoimmun Rev 11(10):687–698

    Article  PubMed  CAS  Google Scholar 

  24. Maldini C, Lavalley MP, Cheminant M et al (2012) Relationships of HLA-B51 or B5 genotype with Behçet’s disease clinical characteristics: systematic review and meta-analyses of observational studies. Rheumatology (Oxford) 51(5):887–900

    Article  Google Scholar 

  25. Remmers EF, Cosan F, Kirino Y et al (2010) Genome-wide association study identifies variants in the MHC class I, IL10, and IL23R-IL12RB2 regions associated with Behçet’s disease. Nat Genet 42:698–702

    Article  PubMed  CAS  Google Scholar 

  26. Mizuki N, Meguro A, Ota M et al (2010) Genome-wide association studies identify IL23R-IL12RB2 and IL10 as Behçet’s disease susceptibility loci. Nat Genet 42:703–706

    Article  PubMed  CAS  Google Scholar 

  27. Lee YJ, Horie Y, Wallace GR et al (2013) Genome-wide association study identifies GIMAP as a novel susceptibility locus for Behçet’s disease. Ann Rheum Dis [Epub ahead of print]

    Google Scholar 

  28. Lee YH, Choi SJ, Ji JD et al (2012) Genome-wide pathway analysis of a genome-wide association study on psoriasis and Behçet’s disease. Mol Biol Rep 39(5):5953–5959

    Article  PubMed  CAS  Google Scholar 

  29. Zierhut M, Mizuki N, Ohno S et al (2003) Immunology and functional genomics of Behçet’s disease. Cell Mol Life Sci 60:1903–1922

    Article  PubMed  CAS  Google Scholar 

  30. Ozen S, Eroglu FK (2013) Pediatric-onset Behçet disease. Curr Opin Rheumatol 25(5):636–642

    Article  PubMed  CAS  Google Scholar 

  31. Touitou I, Magne X, Molinari N et al (2000) MEFV mutations in Behçet’s disease. Hum Mutat 16:271–272

    Article  PubMed  CAS  Google Scholar 

  32. Rabinovich E, Shinar Y, Leiba M et al (2007) Common FMF alleles may predispose to development of Behçet’s disease with increased risk for venous thrombosis. Scand J Rheumatol 36:48–52

    Article  PubMed  CAS  Google Scholar 

  33. Verity DH, Wallace GR, Vaughan RW et al (2003) Behçet’s disease: from Hippocrates to the third millennium. Br J Ophthalmol 87:1175–1183

    Article  PubMed  CAS  Google Scholar 

  34. Hirohata S, Oka H, Mizushima Y (1992) Streptococcal-related antigens stimulate production of IL6 and interferon-gamma by T cells from patients with Behçet’s disease. Cell Immunol 140(2):410–419

    Article  PubMed  CAS  Google Scholar 

  35. Mochizuki M, Suzuki N, Takeno M et al (1994) Fine antigen specificity of human gamma delta T cell lines (V gamma 9+) established by repetitive stimulation with a serotype (KTH-1) of a gram-positive bacterium. Streptococcus sanguis Eur J Immunol 24(7):1536–1543

    Article  CAS  Google Scholar 

  36. Direskeneli H, Saruhan-Direskeneli G (2003) The role of heat shock proteins in Behçet’s disease. Clin Exp Rheumatol 21(4 Suppl 30):S44–S48

    PubMed  CAS  Google Scholar 

  37. Imamura Y, Kurokawa MS, Yoshikawa H et al (2005) Involvement of Th1 cells and heat shock protein 60 in the pathogenesis of intestinal Behçet’s disease. Clin Exp Immunol 139(2):371–378

    Article  PubMed  CAS  Google Scholar 

  38. Amoura Z, Guillaume M, Caillat-Zucman S et al (2006) Pathophysiology of Behçet’s disease. Rev Med Interne 27(11):843–853

    Article  PubMed  CAS  Google Scholar 

  39. Pervin K, Childerstone A, Shinnick T et al (1993) T cell epitope expression of mycobacterial and homologous human 65-kilodalton heat shock protein peptides in short term cell lines from patients with Behçet’s disease. J Immunol 151(4):2273–2282

    PubMed  CAS  Google Scholar 

  40. Ermakova NA, Alekberova ZS, Prokaeva TB (2003) Autoimmunity to S-antigen and retinal vasculitis in patients with Behçet’s disease. Adv Exp Med Biol 528:279–281

    Article  PubMed  CAS  Google Scholar 

  41. Zhao C, Yang P, He H et al (2009) Retinal S-antigen Th1 cell epitope mapping in patients with Behçet’s disease. Graefes Arch Clin Exp Ophthalmol 247(4):555–560

    Article  PubMed  CAS  Google Scholar 

  42. Pay S, Simsek I, Erdem H et al (2007) Dendritic cell subsets and type I interferon system in Behçet’s disease: does functional abnormality in plasmacytoid dendritic cells contribute to Th1 polarization? Clin Exp Rheumatol 25(4 Suppl 45):S34–S40

    PubMed  CAS  Google Scholar 

  43. Zhou ZY, Chen SL, Shen N et al (2012) Cytokines and Behçet’s disease. Autoimmun Rev 11(10):699–704

    Article  PubMed  CAS  Google Scholar 

  44. Hamzaoui K, Hamzaoui A, Hentati F et al (1994) Phenotype and functional profile of T cells expressing gamma delta receptor from patients with active Behçet’s disease. J Rheumatol 21(12):2301–2306

    PubMed  CAS  Google Scholar 

  45. Fam AG, Siminovitch KA, Carette S et al (1982) Neonatal Behçet’s syndrome in an infant of a mother with the disease. Lancet 18;2(8312):1356–1361

    Google Scholar 

  46. Ozen S, Petty RE (2011) Behçet disease. In: Cassidy JT, Petty RE, Laxer R, Lindsley C (eds) Textbook of pediatric rheumatology. Saunders Elseiver, Philedelphia, pp 552–558

    Chapter  Google Scholar 

  47. Saadoun D, Wechsler B (2012) Behçet’s disease. Orphanet J Rare Dis 7:20

    Article  PubMed  Google Scholar 

  48. Davatchi F, Shahram F, Chams-Davatchi C et al (2010) Behçet’s disease in Iran: analysis of 6,500 cases. Int J Rheum Dis 13(4):367–373

    Article  PubMed  Google Scholar 

  49. Koné-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. One-year follow-up data. Rheumatology (Oxford) 50(1):184–188

    Article  Google Scholar 

  50. Sarica R, Azizlerli G, Kose A et al (1996) Juvenile Behçet’s disease among 1784 Turkish Behçet’s patients. Int J Dermatol 35:109–111

    Article  PubMed  CAS  Google Scholar 

  51. Kari JA, Shah V, Dillon MJ (2001) Behçet’s disease in UK children: clinical features and treatment including thalidomide. Rheumatology 40:933–938

    Article  PubMed  CAS  Google Scholar 

  52. Yazici H, Tuzun Y, Pazarli H et al (1980) The combined use of HLA-B5 and the pathergy test as diagnostic markers of Behçet’s disease in Turkey. J Rheumatol 7:206–210

    PubMed  CAS  Google Scholar 

  53. Davatchi F, Sadeghi Abdollahi B et al (2013) Impact of the positive pathergy test on the performance of classification/diagnosis criteria for Behçet’s disease. Mod Rheumatol 23(1):125–132

    Article  PubMed  Google Scholar 

  54. Pivetti-Pezzi P, Accorinti M, Abdulaziz MA et al (1995) Behçet’s disease in children. Jpn J Ophthalmol 39:309–314

    PubMed  CAS  Google Scholar 

  55. Uziel Y, Brik R, Padeh S et al (1998) Juvenile Behçet’s disease in Israel. The pediatric rheumatology study group of Israel. Clin Exp Rheumatol 16:502–505

    PubMed  CAS  Google Scholar 

  56. Eldem B, Onur C, Ozen S (1998) Clinical features of pediatric Behçet’s disease. J Pediatr Ophthalmol Strabismus 35:159–161

    PubMed  CAS  Google Scholar 

  57. Citirik M, Berker N, Songur MS et al (2009) Ocular findings in childhood-onset Behçet disease. J AAPOS 13:391–395

    Article  PubMed  Google Scholar 

  58. Ozen S (2010) The other vasculitis syndromes and kidney involvement. Pediatr Nephrol 25(9):1633–1639

    Article  PubMed  Google Scholar 

  59. Ozen S, Bilginer Y, Besbas N et al (2010) Behçet disease: treatment of vascular involvement in children. Eur J Pediatr 169:427–430

    Article  PubMed  CAS  Google Scholar 

  60. Kiraz S, Ertenli I, Ozturk MA et al (2002) Pathological haemostasis and pro-thrombotic state in Behçet’s disease. Thrombosis Res 105:125–133

    Article  CAS  Google Scholar 

  61. Krupa B, Cimaz R, Ozen S et al (2011) Pediatric Behçet’s disease and thromboses. J Rheumatol 38(2):387–390

    Article  PubMed  Google Scholar 

  62. Erkan F (1999) Pulmonary involvement in Behçet disease. Curr Opin Pulm Med 5:314–318

    Article  PubMed  CAS  Google Scholar 

  63. Sarica R et al (2003) Pulmonary artery involvement in Behçet’s disease. Adv Exp Med Biol 528:419–422

    PubMed  Google Scholar 

  64. Allali F, Benomar A, Karim A et al (2004) Behçet’s disease in moroccan children: a report of 12 cases. Scand J Rheumatol 33:362–363

    Article  PubMed  CAS  Google Scholar 

  65. Al-Araji A, Kidd DP (2009) Neuro-Behçet’s disease: epidemiology, clinical characteristics, and management. Lancet Neurol 8:192–204

    Article  PubMed  Google Scholar 

  66. Ideguchi H, Suda A, Takeno M et al (2010) Neurological manifestations of Behçet’s disease in Japan: a study of 54 patients. J Neurol 257:1012–1020

    Article  PubMed  Google Scholar 

  67. Mora P, Menozzi C, Orsoni JG et al (2013) Neuro-Behçet’s disease in childhood: a focus on the neuro-ophthalmological features. Orphanet J Rare Dis. doi:10.1186/1750-1172-8-18

    Google Scholar 

  68. Akman-Demir G, Serdaroglu P, Tasçi B (1999) Clinical patterns of neurological involvement in Behçet’s disease: evaluation of 200 patients. The neuro-Behçet study group. Brain 122:2171–2178

    Google Scholar 

  69. Hirohata S, Kikuchi H (2003) Behçet’s disease. Arthritis Res Ther 5:139–146

    Article  PubMed  CAS  Google Scholar 

  70. Hirohata S (2008) Histopathology of central nervous system lesions in Behçet’s disease. J Neurol Sci 267:41–47

    Article  PubMed  CAS  Google Scholar 

  71. Siva A, Altintas A, Saip S (2004) Behçet’s syndrome and the nervous system. Curr Opin Neurol 17:347–357

    Article  PubMed  Google Scholar 

  72. Siva A, Saip S (2009) The spectrum of nervous system involvement in Behçet’s syndrome and its differential diagnosis. J Neurol 256:513–529

    Article  PubMed  Google Scholar 

  73. Ogose T, Tamaki W, Shinahara K et al (2010) A case of recurrent myositis as the main manifestation of Behçet disease. Pediatr Int 52(2):e101–e104

    Article  PubMed  Google Scholar 

  74. Hatemi G, Silman A, Bang D et al (2008) EULAR expert committee. EULAR recommendations for the management of Behçet disease. Ann Rheum Dis 67:1656–1662

    Google Scholar 

  75. Arida A, Vaiopoulos G, Markomichelakis N et al (2009) Are clusters of patients with distinct clinical expression present in Behçet’s disease? Clin Exp Rheumatol 27(2 Suppl 53):S48–S51

    PubMed  CAS  Google Scholar 

  76. Coban O, Bahar S, Akman-Demir G et al (1999) Masked assessment of MRI findings: is it possible to differentiate neuro-Behçet’s disease from other central nervous system diseases? Neuroradiology 41:255–260

    Article  PubMed  CAS  Google Scholar 

  77. Akman-Demir G, Tuzun E, Icoz S et al (2008) Interleukin-6 in neuro-Behçet’s disease: association with disease subsets and long-term outcome. Cytokine 44:373–376

    Article  PubMed  CAS  Google Scholar 

  78. Yurdakul S, Yazici H, Tuzun Y et al (1983) The arthritis of Behçet’s disease: a prospective study. Ann Rheum Dis 42:505–515

    Article  PubMed  CAS  Google Scholar 

  79. Leiba M, Sidi Y, Gur H et al (2001) Behçet’s disease and thrombophilia. Ann Rheum Dis 60:1081–1085

    Article  PubMed  CAS  Google Scholar 

  80. Leiba M, Seligsohn U, Sidi Y et al (2004) Thrombophilic factors are not the leading cause of thrombosis in Behçet’s disease. Ann Rheum Dis 63:1445–1449

    Article  PubMed  CAS  Google Scholar 

  81. La Regina M, Orlandini F, Prisco D et al (2010) Homocysteine in vascular Behçet disease: a meta-analysis. Arterioscler Thromb Vasc Biol 30:2067–2074

    Article  PubMed  Google Scholar 

  82. Akman-Demir G, Bahar S, Coban O et al (2003) Cranial MRI in Behçet’s disease: 134 examinations of 98 patients. Neuroradiology 45:851–859

    Article  PubMed  CAS  Google Scholar 

  83. Kocer N, Islak C, Siva A et al (1999) CNS involvement in Neuro-Behçet’s syndrome: an MR study. Am J Neuroradiol 20:1015–1024

    PubMed  CAS  Google Scholar 

  84. Saltik S, Saip S, Kocer N et al (2004) MRI findings in pediatric neuro-Behçet’s disease. Neuropediatrics 35:190–193

    Article  PubMed  CAS  Google Scholar 

  85. Davatchi F, Sadeghi Abdollahi B, Tehrani Banihashemi A et al (2009) Colchicine versus placebo in Behçet’s disease: randomized, double-blind, controlled crossover trial. Mod Rheumatol 19(5):542–549

    Article  PubMed  CAS  Google Scholar 

  86. Hamuryudan V, Mat C, Saip S et al (1998) Thalidomide in the treatment of the mucocutaneous lesions of the Behçet syndrome. A randomized, double-blind, placebo-controlled trial. Ann Intern Med 128(6):443–450

    Article  PubMed  CAS  Google Scholar 

  87. Pipitone N, Olivieri I, Padula A et al (2008) Infliximab for the treatment of neuro-Behçet’s disease: a case series and review of the literature. Arthritis Rheum 59:285–290

    Article  PubMed  CAS  Google Scholar 

  88. Alty JE, Monaghan TM, Bamford JM (2007) A patient with neuro-Behçet’s disease is successfully treated with etanercept: further evidence for the value of TNF alpha blockade. Clin Neurol Neurosurg 109:279–281

    Article  PubMed  Google Scholar 

  89. Belzunegui J (2008) Efficacy of infliximab and adalimumab in the treatment of a patient with severe neuro-Behçet’s disease. Clin Exp Rheumatol 26(Suppl.):S133–4

    Google Scholar 

  90. Saadoun D, Wechsler B, Desseaux K et al (2010) Mortality in Behçet’s disease. Arthritis Rheum 62(9):2806–2812

    Article  PubMed  CAS  Google Scholar 

  91. Yazici H, Tüzün Y, Pazarli H et al (1984) Influence of age of onset and patient’s sex on the prevalence and severity of manifestations of Behçet’s syndrome. Ann Rheum Dis 43(6):783–789

    Article  PubMed  CAS  Google Scholar 

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Authors and Affiliations

  1. Department of Pediatric Rheumatology, Hacettepe University, Faculty of Medicine, Sıhhiye, 06410, Ankara, Turkey

    Seza Özen

  2. Department of Rheumatology, Meyer Pediatric Hospital, Viale Pieraccini 24, 50139, Florence, Italy

    Rolando Cimaz

  3. Department of Pediatrics, Hacettepe University, Faculty of Medicine, Sıhhiye, 06410, Ankara, Turkey

    Ezgi Deniz Batu

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  1. Ezgi Deniz Batu
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  2. Rolando Cimaz
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  3. Seza Özen
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Correspondence to Seza Özen .

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  1. SOD Patologia Medica, Center for Autoimmune Systemic Diseases, Behçet Center and Lupus Clinic - AOU Careggi, Florence, Italy

    Lorenzo Emmi

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Batu, E.D., Cimaz, R., Özen, S. (2014). Pediatric Onset of Behçet Syndrome. In: Emmi, L. (eds) Behçet's Syndrome. Rare Diseases of the Immune System. Springer, Milano. https://doi.org/10.1007/978-88-470-5477-6_15

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