Download PDF
Journal of Pediatric Neurology 2020; 18(04): 175-181
DOI: 10.1055/s-0039-1692451
Review Article
Georg Thieme Verlag KG Stuttgart · New York

Behçet's Disease from Rheumatology and Neurology Perspectives with Special Reference to Children

Yaşar B. Turgut
1   Department of Internal Medicine, Muğla Sıtkı Koçman University School of Medicine, Muğla, Turkey
,
Ayşe F. Tosun
2   Department of Pediatric Neurology, Adnan Menderes University School of Medicine, Aydın, Turkey
,
Beste K. Yüzbaşı
3   Clinic of Pediatric Neurology, Denizli State Hospital, Denizli, Turkey
,
Hasan Tunca
1   Department of Internal Medicine, Muğla Sıtkı Koçman University School of Medicine, Muğla, Turkey
› Author Affiliations
Further Information

Publication History

08 April 2019

08 May 2019

Publication Date:
01 July 2019 (online)

    Permissions and Reprints

Abstract

Behçet's disease (BD) is an idiopathic chronic relapsing multisystemic vascular inflammatory disease. Although it is generally seen in young adults with male predominance, the onset age of the disease is the pediatric age group in only 4 to 26% of all cases of BD. According to the International Study Group for Behçet's Disease Classification Criteria, a diagnosis of BD requires recurrent oral aphthous ulcerations with two of the following: genital ulcerations, skin lesions, eye lesions, or a positive pathergy test. In this article, we extensively reviewed the current literature with respect to the rheumatological and neurological findings of BD in childhood.

Keywords

Behçet's Disease - neuro-Behçet - neurology - rheumatology
 

  • References

  • 1 Saylan T. Life story of Dr. Hulusi Behçet. Yonsei Med J 1997; 38 (06) 327-332
    CrossrefPubMedGoogle Scholar
  • 2 UStün C. A famous Turkish dermatologist, Dr. Hulusi Behçet. Eur J Dermatol 2002; 12 (05) 469-470
    PubMedGoogle Scholar
  • 3 Turgut YB, Turgut M. Turkish scientist Hulusi Behçet (1889-1948) and his contribution to the medical world. Childs Nerv Syst 2019; DOI: 10.1007/s00381-019-04081-8.
    CrossrefPubMedGoogle Scholar
  • 4 Yemni O. Ord. Prof. Dr. Hulusi Behçet. Deri Hast Frengi Arş 1964; 1: 58-59
    PubMedGoogle Scholar
  • 5 Behcet H. Uber rezidivierende, aphthose, dürchein Virus verursachte Geshwure am Munde, am Auge und an den Genitalien. Dermatol Wochenschr 1937; 36: 1152-1157
    PubMedGoogle Scholar
  • 6 Caruso P, Moretti R. Focus on neuro-Behçet's disease: a review. Neurol India 2018; 66 (06) 1619-1628
    CrossrefPubMedGoogle Scholar
  • 7 Jennette JC, Falk RJ, Bacon PA. , et al. 2012 revised International Chapel Hill Consensus Conference Nomenclature of Vasculitides. Arthritis Rheum 2013; 65 (01) 1-11
    CrossrefPubMedGoogle Scholar
  • 8 Hatemi G, Christensen R, Bang D. , et al. 2018 update of the EULAR recommendations for the management of Behçet's syndrome. Ann Rheum Dis 2018; 77 (06) 808-818
    PubMedGoogle Scholar
  • 9 International Study Group for Behçet's Disease. Criteria for diagnosis of Behçet's disease. Lancet 1990; 335 (8697): 1078-1080
    PubMedGoogle Scholar
  • 10 International Team for the Revision of the International Criteria for Behçet's Disease (ITR-ICBD). The International Criteria for Behçet's Disease (ICBD): a collaborative study of 27 countries on the sensitivity and specificity of the new criteria. J Eur Acad Dermatol Venereol 2014; 28 (03) 338-347
    CrossrefPubMedGoogle Scholar
  • 11 Koné-Paut I. Behçet's disease in children, an overview. Pediatr Rheumatol Online J 2016; 14 (01) 10
    CrossrefPubMedGoogle Scholar
  • 12 Koné-Paut I, Shahram F, Darce-Bello M. , et al; PEDBD group. Consensus classification criteria for paediatric Behçet's disease from a prospective observational cohort: PEDBD. Ann Rheum Dis 2016; 75 (06) 958-964
    CrossrefPubMedGoogle Scholar
  • 13 Batu ED, Sönmez HE, Sözeri B, Butbul Aviel Y, Bilginer Y, Özen S. The performance of different classification criteria in paediatric Behçet's disease. Clin Exp Rheumatol 2017; 35 (6, Suppl 108): 119-123
    PubMedGoogle Scholar
  • 14 Uluduz D, Kürtüncü M, Yapıcı Z. , et al. Clinical characteristics of pediatric-onset neuro-Behçet disease. Neurology 2011; 77 (21) 1900-1905
    CrossrefPubMedGoogle Scholar
  • 15 Zouboulis CC, Kötter I, Djawari D. , et al. Epidemiological features of Adamantiades-Behçet's disease in Germany and in Europe. Yonsei Med J 1997; 38 (06) 411-422
    CrossrefPubMedGoogle Scholar
  • 16 Koné-Paut I, Yurdakul S, Bahabri SA. , et al. Clinical features of Behçet's disease in children: an International Collaborative Study of 86 cases. J Pediatr 1998; 132 (04) 721-725
    CrossrefPubMedGoogle Scholar
  • 17 Nanthapisal S, Klein NJ, Ambrose N, Eleftheriou D, Brogan PA. Paediatric Behçet's disease: a UK tertiary centre experience. Clin Rheumatol 2016; 35 (10) 2509-2516
    CrossrefPubMedGoogle Scholar
  • 18 Koné-Paut I, Darce-Bello M, Shahram F. , et al; PED-BD International Expert Committee. Registries in rheumatological and musculoskeletal conditions. Paediatric Behçet's disease: an International Cohort Study of 110 patients. One-year follow-up data. Rheumatology (Oxford) 2011; 50 (01) 184-188
    CrossrefPubMedGoogle Scholar
  • 19 Atmaca L, Boyvat A, Yalçındağ FN, Atmaca-Sonmez P, Gurler A. Behçet disease in children. Ocul Immunol Inflamm 2011; 19 (02) 103-107
    CrossrefPubMedGoogle Scholar
  • 20 Davatchi F, Shahram F, Chams-Davatchi C. , et al. Behcet's disease in Iran: analysis of 6500 cases. Int J Rheum Dis 2010; 13 (04) 367-373
    CrossrefPubMedGoogle Scholar
  • 21 Houman MH, Bel Feki N. Pathophysiology of Behçet's disease [article in French]. Rev Med Interne 2014; 35 (02) 90-96
    CrossrefPubMedGoogle Scholar
  • 22 Direskeneli H. Autoimmunity vs autoinflammation in Behcet's disease: do we oversimplify a complex disorder?. Rheumatology (Oxford) 2006; 45 (12) 1461-1465
    CrossrefPubMedGoogle Scholar
  • 23 Yazici H, Fresko I, Yurdakul S. Behçet's syndrome: disease manifestations, management, and advances in treatment. Nat Clin Pract Rheumatol 2007; 3 (03) 148-155
    CrossrefPubMedGoogle Scholar
  • 24 Alpsoy E, Zouboulis CC, Ehrlich GE. Mucocutaneous lesions of Behcet's disease. Yonsei Med J 2007; 48 (04) 573-585
    CrossrefPubMedGoogle Scholar
  • 25 Bulur I, Onder M. Behçet disease: new aspects. Clin Dermatol 2017; 35 (05) 421-434
    CrossrefPubMedGoogle Scholar
  • 26 Karincaoglu Y, Borlu M, Toker SC. , et al. Demographic and clinical properties of juvenile-onset Behçet's disease: a controlled multicenter study. J Am Acad Dermatol 2008; 58 (04) 579-584
    CrossrefPubMedGoogle Scholar
  • 27 Dalvi SR, Yildirim R, Yazici Y. Behcet's syndrome. Drugs 2012; 72 (17) 2223-2241
    CrossrefPubMedGoogle Scholar
  • 28 Ahn JK, Lee YS, Jeon CH, Koh EM, Cha HS. Treatment of venous thrombosis associated with Behcet's disease: immunosuppressive therapy alone versus immunosuppressive therapy plus anticoagulation. Clin Rheumatol 2008; 27 (02) 201-205
    CrossrefPubMedGoogle Scholar
  • 29 Connors JM. Thrombophilia testing and venous thrombosis. N Engl J Med 2017; 377 (12) 1177-1187
    CrossrefPubMedGoogle Scholar
  • 30 Shimizu T, Ehrlich GE, Inaba G, Hayashi K. Behçet disease (Behçet syndrome). Semin Arthritis Rheum 1979; 8 (04) 223-260
    CrossrefPubMedGoogle Scholar
  • 31 Akman-Demir G, Serdaroglu P, Tasçi B. ; The Neuro-Behçet Study Group. Clinical patterns of neurological involvement in Behçet's disease: evaluation of 200 patients. Brain 1999; 122 (Pt 11): 2171-2182
    CrossrefPubMedGoogle Scholar
  • 32 Kidd D, Steuer A, Denman AM, Rudge P. Neurological complications in Behçet's syndrome. Brain 1999; 122 (Pt 11): 2183-2194
    CrossrefPubMedGoogle Scholar
  • 33 Landeyro J, Vidaur-Tello L, García-Fontgivell JF, Elguezábal A, Gené-Hijós M, Mayayo-Artal E. [Neuro-Behçet: clinicopathological findings in an autopsy case] (in Spanish). Rev Neurol 2008; 47 (11) 575-578
    PubMedGoogle Scholar
  • 34 Sakane T, Takeno M, Suzuki N, Inaba G. Behçet's disease. N Engl J Med 1999; 341 (17) 1284-1291
    CrossrefPubMedGoogle Scholar
  • 35 Al-Araji A, Kidd DP. Neuro-Behçet's disease: epidemiology, clinical characteristics, and management. Lancet Neurol 2009; 8 (02) 192-204
    CrossrefPubMedGoogle Scholar
  • 36 Uygunoğlu U, Saip S, Siva A. Behçet's disease and neuro-Behçet's syndrome. EMJ Neurol 2018; 6: 77-85
    PubMedGoogle Scholar
  • 37 Kalra S, Silman A, Akman-Demir G. , et al. Diagnosis and management of neuro-Behçet's disease: international consensus recommendations. J Neurol 2014; 261 (09) 1662-1676
    CrossrefPubMedGoogle Scholar
  • 38 Deniz O, Cayköylü A, Vural G. , et al. A case study of neuro-psycho-Behçet's syndrome presenting with psychotic attack. Clin Neurol Neurosurg 2009; 111 (10) 877-879
    CrossrefPubMedGoogle Scholar
  • 39 Yeo M, Lee HL, Cha M. , et al. Neuro-Behcet disease presenting as a solitary cerebellar hemorrhagic lesion: a case report and review of the literature. J Med Case Reports 2016; 10 (01) 360
    CrossrefPubMedGoogle Scholar
  • 40 Rottenstreich A, Machol K, Eisenstein EM. , et al. Behçet's disease and cerebral sinus vein thrombosis in children: a case study and review of the literature. Clin Exp Rheumatol 2015; 33 (6, Suppl 94): S163-S168
    PubMedGoogle Scholar
  • 41 D’Angelo T, Gallizzi R, Romano C, Cicero G, Mazziotti S. Cicero G, Mazziotti S. Magnetic resonance enterography findings of intestinal Behçet disease in a child. Case Rep Radiol 2017; 2017: 8061648
    PubMedGoogle Scholar
  • 42 Mascalchi M, Cosottini M, Cellerini M, Paganini M, Arnetoli G. MRI of spinal cord involvement in Behçet's disease: case report. Neuroradiology 1998; 40 (04) 255-257
    CrossrefPubMedGoogle Scholar
  • 43 Coban O, Bahar S, Akman-Demir G, Taşci B, Yurdakul S, Yazici H, Serdaroğlu P. Masked assessment of MRI findings: is it possible to differentiate neuro-Behçet's disease from other central nervous system diseases?. [corrected] Neuroradiology 1999; 41: 255-260
    CrossrefPubMedGoogle Scholar
  • 44 Wechsler B, Vidailhet M, Piette JC. , et al. Cerebral venous thrombosis in Behçet's disease: clinical study and long-term follow-up of 25 cases. Neurology 1992; 42 (3 Pt 1): 614-618
    CrossrefPubMedGoogle Scholar
  • 45 Cengiz N, Sahin M, Onar M. Correlation of clinical, MRI and Tc-99m HMPAO SPECT findings in neuro-Behçet's disease. Acta Neurol Belg 2004; 104 (03) 100-105
    PubMedGoogle Scholar
  • 46 Leiba M, Seligsohn U, Sidi Y. , et al. Thrombophilic factors are not the leading cause of thrombosis in Behçet's disease. Ann Rheum Dis 2004; 63 (11) 1445-1449
    CrossrefPubMedGoogle Scholar
  • 47 Al-Araji A, Sharquie K, Al-Rawi Z. Prevalence and patterns of neurological involvement in Behcet's disease: a prospective study from Iraq. J Neurol Neurosurg Psychiatry 2003; 74 (05) 608-613
    CrossrefPubMedGoogle Scholar
  • 48 Boulter E, Brogan P. Behcet's disease. In: Foster H, Brogan P. , eds. Paediatric Rheumatology. Oxford: Oxford University Press; 2012: 205-208
    Google Scholar
  • 49 Ozguler Y, Yazici H. Behçet's syndrome: new insights into pathogenesis and management. Ind Journal of Rheum 2014; 9: 184-191
    PubMedGoogle Scholar
  • 50 Evereklioglu C. Current concepts in the etiology and treatment of Behçet disease. Surv Ophthalmol 2005; 50 (04) 297-350
    CrossrefPubMedGoogle Scholar
  • 51 Vallet H, Riviere S, Sanna A. , et al; French Behçet Network. Efficacy of anti-TNF alpha in severe and/or refractory Behçet's disease: multicenter study of 124 patients. J Autoimmun 2015; 62: 67-74
    CrossrefPubMedGoogle Scholar
  • 52 Shapiro LS, Farrell J, Borhani Haghighi A. Tocilizumab treatment for neuro-Behcet's disease, the first report. Clin Neurol Neurosurg 2012; 114 (03) 297-298
    CrossrefPubMedGoogle Scholar
  • 53 Saadoun D, Wechsler B, Desseaux K. , et al. Mortality in Behçet's disease. Arthritis Rheum 2010; 62 (09) 2806-2812
    CrossrefPubMedGoogle Scholar