Behçet's syndrome: A critical digest of the recent literature

Clinical and Experimental Rheumatology

Volumn 30, Issue SUPPL.72, 2012, Pages

  Hatemi, Gulen ^a   Seyahi, Emire ^a   Fresko, Izzet ^a   Hamuryudan, Vedat ^a  

^a ISTANBUL UNIVERSITY   (Turkey)

Author keywords

Beh et's syndrome; Epidemiology; Genetics; pathogenesis; Th17; Treatment; Vascular involvement

Indexed keywords

ADALIMUMAB; ALPHA INTERFERON; ALPHA2A INTERFERON; AZATHIOPRINE; CANAKINUMAB; CORTICOSTEROID; CYCLOPHOSPHAMIDE; CYCLOSPORIN A; GAMMA INTERFERON; GEVOKIZUMAB; HLA B51 ANTIGEN; IMMUNOSUPPRESSIVE AGENT; INFLIXIMAB; INTERLEUKIN 10; METHOTREXATE; METHYLPREDNISOLONE; MYCOPHENOLIC ACID; PREDNISOLONE; TOCILIZUMAB; TRIAMCINOLONE ACETONIDE; BIOLOGICAL PRODUCT;

ARTERY DISEASE; BEHCET DISEASE; CEREBRAL SINUS THROMBOSIS; CLINICAL FEATURE; CYTOKINE PRODUCTION; DISEASE ASSOCIATION; DRUG DOSE INCREASE; DRUG DOSE REDUCTION; DRUG EFFICACY; DRUG MEGADOSE; DRUG SUBSTITUTION; DRUG TREATMENT FAILURE; DRUG WITHDRAWAL; GENE FREQUENCY; GENE LINKAGE DISEQUILIBRIUM; GENETIC ASSOCIATION; GENETIC POLYMORPHISM; HEART DISEASE; HEREDITY; HUMAN; IMMUNOSUPPRESSIVE TREATMENT; JAPAN; LYMPHOCYTE FUNCTION; NEUROLOGIC DISEASE; ONSET AGE; PATHOGENESIS; PREVALENCE; PRIORITY JOURNAL; RETINA VASCULITIS; REVIEW; SEX DIFFERENCE; SKIN DISEASE; TH17 CELL; TREATMENT DURATION; TREATMENT RESPONSE; UNITED STATES; UNSPECIFIED SIDE EFFECT; UVEITIS; UVEORETINITIS; VASCULAR DISEASE; VEIN THROMBOSIS; ANIMAL; DISEASE COURSE; ETHNOLOGY; GENETIC PREDISPOSITION; GENETICS; IMMUNOLOGY; PHENOTYPE; RISK ASSESSMENT; RISK FACTOR;

ANIMALS; BEHCET SYNDROME; BIOLOGICAL AGENTS; DISEASE PROGRESSION; GENETIC PREDISPOSITION TO DISEASE; HUMANS; IMMUNOSUPPRESSIVE AGENTS; PHENOTYPE; RISK ASSESSMENT; RISK FACTORS; TH17 CELLS;

EID: 84873259046     PISSN: 0392856X     EISSN: 1593098X     Source Type: Journal    
DOI: None     Document Type: Review

References (95)

1. YURDAKUL S, YAZICI Y: Epidemiology of Behçet's syndrome and regional differences in disease expression. In: YAZICI Y, YAZICI H (Eds.). Behçet's syndrome. New York, Springer; 2010: 35-53.
2. OLIVEIRA AC, BUOSI AL, DUTRA LA, DE SOUZA AW: Behçet disease: clinicalfeatures and management in a Brazilian tertiary hospital. J Clin Rheumatol 2011; 17: 416-20.
3. OHARA Y, KISHIMOTO M, KOBAYASHI T et al.: Difference of Manifestations and Treatment Among Behçet's Syndrome Patients in the United States and Japan. Arthritis Rheum 2011; 63 (Suppl. 10): s933.
4. ZHANG Z, HE F, SHI Y: Behçet's disease seen in China: analysis of 334 cases. Rheumatol Int 2012 Apr 11. [Epub ahead of print]
5. KOBAYASHI T, KISHIMOTO M, SWEARINGEN CJ et al.: Differences in clinical manifestations, treatment, and concordance rates with twomajor sets of criteria for Behçet's syndrome for patients in the US and Japan: data from a large, three-center cohort study. Mod Rheumatol 2012 Jun 30 [Epub ahead of print].
6. SAADOUN D, WECHSLER B, DESSEAUX K et al.: Mortality in Behçet's disease. Arthritis Rheum 2010; 62: 2806-12.
7. KURAL-SEYAHI E, FRESKO I, SEYAHI N et al.: The long-term mortality and morbidity of Behçet syndrome: a 2-decadeoutcome survey of 387 patients followed at a dedicated center. Medicine (Baltimore) 2003; 82: 60-76.
8. GUL A, OHNO S: Genetics of Behçet's disease. Behçet's Syndrome. YAZICI Y and YAZICI H. Springer, New York 2010: pp 265-276.
9. MAHR A, BELARBI L,WECHSLER B et al.: Population-based prevalence study of Behçet's disease: differences by ethnic origin and low variation by age at immigration. Arthritis Rheum 2008; 58: 3951-9.
10. SEYAHI E, TAHIRTURANLI E, MANGAN MS et al.: The prevalence of Behçet's syndrome, familial Mediterranean fever, HLA B51 and MEVF gene mutations among ethnic Armenians living in Istanbul, Turkey. Clin Exp Rheumatol 2010; 28 (Suppl. 60): S67-75.
11. KONE-PAUT I, GEISLER B, WECHSLER A et al.: Familial aggregation in Behçet's disease: high frequency in siblings and parents of pediatric probands. J Pediatr 1999; 135: 89-93.
12. MASATLIOGLU S, SEYAHI E, TAHIR-TURANLI E et al.: A twin study in Behçet's syndrome. Clin Exp Rheumatol 2010, 28 (Suppl. 60); S62-6.
13. DE MENTHON M, LAVALLEY MP, MALDINI C et al.: HLAB51/B5 and the risk of Behçet's disease: a systematic review and meta-analysis of case control genetic association studies. Arthritis Rheum 2009; 61: 1287-96.
14. DUYMAZ-TOZKIR J, UYAR A, NORMAN PJ et al.: Distribution of killer immunoglobulin like receptor 3DL1/3DS1 alleles in Behçet's disease. Arthritis Rheum 2008; 58 (Suppl.): S855.
15. TAKENO M, KARIYONE A, YAMASHITA N et al.: Excessive function of peripheral blood neutrophils from patients with Behçet's disease and from HLAB51 transgenic mice. Arthritis Rheum 1995; 38: 426-33.
16. MEGURO A, INOKO H, OTA M et al.: Genetics of Behçet's Disease inside and outside the MHC. Ann Rheum Dis 2012; 69: 747-54.
17. MALDINI C, LAVALLEY MP, CHEMINANT M et al.: Relationships of HLA-B51 or B5 genotype with Behcet's disease clinical characteristics: systematic review and meta-analyses of observational studies. Rheumatology (Oxford) 2012; 51: 887-900.
18. KANG EH, KIM JY, TAKEUCHI F et al.: Associations between the HLA-A polymorphism and the clinical manifestations of Behçet's disease. Arthritis Res Ther 2011; 13: R49.
19. HTOON J, NADIG A, HUGHES T et al.: IL18 polymorphism is associated with Behçet's disease but not Lupus in patients from Turkey. J Rheumatol 2011; 38: 962-3.
20. RUSTEMOGLU A, GUL U, GUMUS-AKAY G et al.: MDR! Gene polymorphisms may be associated with Behçet's disease and its colchicum treatment response. Gene 2012 [Epub ahead of print].
21. DEMIR HD, YALCINDAG FN, OZTURK A, AKAR N: Intron F G79A polymorphism of the Protein Z Gene in Turkish Behçet's Patients. Curr Eye Res 2012; 37: 630-2.
22. KIM SK, JANG WC, AHN YC et al.: Promoter- 2518 single nucleotide polymorphism of monocyte chemoattractant protein-1 is associated with clinical severity in Behçet's disease. Inflamm Res 2012; 61: 541-5.
23. CHEN Y, YANG P, LI F et al.: Association analysis of TGFBR3 gene with Vogt-Koyanagi- Harada disease and Behçet's disease in the Chinese Han population. Curr Eye Res 2012: 37: 312-7.
24. PARK KS, MIN Y, PARK SR et al.: Matrix metalloproteinase 2,9,12 and tissue inhibitor of metalloproteinase 2 gene polymorphisms and cutaneous expressions in patients with Behçet's disease. Tissue Antigens 2012; 79: 333-9.
25. AR MC, HATEMI G, EKIZOGLU S et al.: JAK2 V617F mutation is not associated with thrombosis in Behçet's syndrome. Clin Appl Thromb Hemost 2012; 18: 421-6.
26. HU K, HOU S, JIANG Z et al.: JAK2 and STAT3 polymorphisms in a Han Chinese population with Behçet's disease. Invest Ophthalmol Vis Sci 2012; 53: 538-541.
27. ATZENI F, BOIARDI L, NICOLI D et al.: PLA1/ A2 polymorphism of the platelet glycoprotein receptors IIIA in Behçet's disease. Clin Exp Rheumatol 2011; 29 (Suppl. 67): S38-43
28. SHAHRAM F, NIKOOPOUR E, REZAEI N et al.: Association of interleukin-2, interleukin- 4 and transforming growth factor-beta gene polymorphisms with Behçet's disease. Clin Exp Rheumatol 2011; 29 (Suppl. 67): S28-31.
29. PARK G, KIM HS, CHOE JY, KIM SK: SUMO4 C438T polymorphism is associated with papulopustular skin lesion in Korean patients with Behçet's disease. Rheumatol Int 2011 [Epub ahead of print].
30. HAMZAOUI K, ABID H, BERRAIES A, AMMAR J, HAMZAOUI A: NOD2 is highly expressed in Behçet disease with pulmonary manifestations. J Inflam 2012; 9: 3.
31. AHN JK, CHA HS, LEE J et al.: Correlation of DEFA1 gene copy number variation with intestinal involvement in Behçet's disease. J Korean Med Sci 2012; 27: 107-9.
32. PARK JJ, OH BR, KIM JY et al.: Copy number variation of ß-defensin genes in Behçet's disease. Clin Exp Rheumatol 2011; 29 (Suppl. 67): S20-3.
33. BLACK R, LESTER S, DUNSTAN E et al.: Fcgamma receptor 3B copy number variation is not a risk factor for Behçet's disease. Int J Rheumatol 2012; 167096.
34. FEI Y, WEBB R, COBB BL et al.: Identification of a novel genetic susceptibility loci for Behçet's disease using a genome-wide association study. Arthritis Res Ther 2009: 11: R66.
35. SAWALHA AH, HUGHES T, NADIG A et al.: A putative functional variant within the UBAC2 gene is associated with increased risk of Behçet's disease. Arthritis Rheum 2011; 63: 3607-12.
36. HOU S, SHU Q, JIANG Z et al.: Replication study confirms the association between UBAC2 and Behçet's disease in two independent Chinese sets of patients and controls. Arthritis Res Ther 2012; 14: R70.
37. REMMERS EF, COSAN F, KIRINO Y et al.: Genome-wide association study identifies variants in the MHC class I, IL10 and IL- 23R-IL12RB2 regions associated with Behçet's disease. Nat Genet 2010; 42: 698-702.
38. MIZUKI N, MEGURO A, OTA M et al.: Genome-wide association studies identify IL23R-IL12RB2 and IL10 as Behçet's disease susceptibility loci. Nat Genet 2010; 42: 703-6.
39. DE CRAEN AJ, POSTHUMA D, REMARQUE EJ et al.: Heritable estimates of innate immunity: an extended twin study. Genes Immun 2005; 6: 167-70.
40. YAZICI H, TUZUN Y, PAZARLI H et al.: Influence of age of onset and patients sex on the prevalence and severity of manifestations of Behçet's syndrome. Ann Rheum Dis 1984; 43: 783-9.
41. GUL A: Genome-wide association studies in Behçet's disease: expectations and promises. Clin Exp Rheumatol 2011; 29 (Suppl. 67): S3-5.
42. KARACA M, HATEMI G, SUT N, YAZICI H: The papulopustular lesion/arthritis cluster in Behçet's syndrome also clusters in families. Rheumatology (Oxford) 2012; 51: 1053-60.
43. YAZICI H, UGURLU S, SEYAHI E: Behçet's syndrome: is it one condition? Clin Rev Allergy Immunol 2012 June 7 [Epub ahead of print].
44. DIRESKENELI H, FUJITA H, AKDIS CA Regulation of TH17 and regulatory T cellsin patients with Behçet disease. J Allergy Clin Immunol 2011; 128: 665-6.
45. HAMZAOUI K: Th17 cells in Behçet's disease: a new immunoregulatory axis. Clin Exp Rheumatol 2011; 29 (Suppl. 67): S71-6.
46. GERI G, TERRIER B, ROSENZWAJG M et al.: Critical role of IL-21 in modulating TH17 and regulatory T cells in Behçet disease. J Allergy Clin Immunol 2011; 128: 655-64.
47. KIM J, PARK JA, LEE EY, LEE YJ, SONG YW, LEE EB: Imbalance of Th17 to Th1cells in Behçet's disease. Clin Exp Rheumatol 2010; 28 (Suppl. 60): S16-9.
48. FERRANTE A, CICCIA F, PRINCIPATO A et al.: A Th1 but not a Th17 response is present in the gastrointestinalinvolvement of Behçet's disease. Clin Exp Rheumatol 2010; 28 (Suppl. 60): S27-30.
49. SUGITA S, KAWAZOE Y, IMAI A, YAMADA Y, HORIE S, MOCHIZUKI M: Inhibition ofTh17 differentiation by anti-TNF-alpha therapy in uveitis patients with Behçet's disease. Arthritis Res Ther 2012; 14: R99.
50. LIU X, YANG P, WANG C, LI F, KIJLSTRA A: IFN-alpha blocks IL-17 production by peripheral blood mononuclear cells in Behçet's disease. Rheumatology (Oxford) 2011; 50: 293-8.
51. CHI W, YANG P, ZHU X et al.: Production of interleukin-17 in Behçet's disease is inhibited by cyclosporin A. Mol Vis 2010; 16: 880-6.
52. DIRI E, MAT C, HAMURYUDAN V, YURDAKUL S, HIZLI N, YAZICI H: Papulopustular skin lesions are seen more frequently in patients with Behçet's syndrome who have arthritis: a controlled and masked study. Ann Rheum Dis 2001; 60: 1074-6.
53. TUNC R, KEYMAN E, MELIKOGLU M, FRESKO I, YAZICI H: Target organ associations in Turkish patients with Behçet's disease: a cross sectional study by exploratory factor analysis. J Rheumatol 2002; 29: 2393-6.
54. HATEMI G, BAHAR H, UYSAL S et al.: The pustular skin lesions in Behçet's syndrome are not sterile. Ann Rheum Dis 2004; 63: 1450-2.
55. HATEMI G, FRESKO I, TASCILAR K, YAZICI H: Increased enthesopathy among Behçet's syndrome patients with acne and arthritis: an ultrasonography study. Arthritis Rheum 2008; 58: 1539-45.
56. HATEMI G, FRESKO I, YURDAKUL S et al.: Reply to letter by Priori et al. commenting on whether Behçet's syndrome patients with acne and arthritis comprise a true subset. Arthritis Rheum 2010; 62: 305-6.
57. HATEMI G, YAZICI H: Behçet's syndrome and micro-organisms. Best Pract Res Clin Rheumatol 2011; 25: 389-406.
58. SEYAHI E, YURDAKUL S: Behçet's Syndrome and Thrombosis. Mediterr J Hematol Infect Dis 2011; 3: e2011026.
59. IDEGUCHI H, SUDA A, TAKENO M, UEDA A, OHNO S, ISHIGATSUBO Y: Characteristics of vascular involvement in Behçet's disease in Japan: a retrospective cohort study. Clin Exp Rheumatol 2011; 29 (Suppl. 67): S47-53.
60. SAADOUN D, ASLI B, WECHSLER B et al.: Long-term outcome of arterial lesions in Behçet disease: a series of 101 patients. Medicine (Baltimore) 2012; 91: 18-24.
61. SEYAHI E, MELIKOGLU M, AKMAN C et al.: Pulmonary artery involvement and associated lung disease in Behçet disease: a series of 47 patients. Medicine (Baltimore) 2012; 91: 35-48.
62. SEYAHI E, BASKURT M, MELIKOGLU M et al.: The estimated pulmonary artery pressure can be elevated in Behçet's syndrome. Respir Med 2011; 105: 1739-47.
63. GERI G, WECHSLER B, THI HUONG DU L et al.: Spectrum of cardiac lesions in Behçet disease: a series of 52 patients and review of the literature. Medicine (Baltimore) 2012; 91: 25-34.
64. AGUIAR DE SOUZA D, MESTRE T, FERRO JM: Cerebral venous thrombosis in Behçet's disease: a systematic review. J Neurol 2011; 258: 719-27.
65. SAADOUN D, WECHSLER B, RESCHE-RIGON M et al.: Cerebral venous thrombosis in behçet's disease. Arthritis Care Res 2009; 61: 218-26.
66. ULUDUZ D, KÜRTÜNCÜ M, YAPICI Z et al.: Clinical characteristics of pediatric-onset neuro-Behçet disease. Neurology 2011; 77: 1900-5.
67. SIVA A, SAIP S: The spectrum of nervous system involvement in Behçet's syndrome and its differential diagnosis. J Neurol 2009; 256: 513-29.
68. YESILOT N, BAHAR S, YILMAZER S et al.: Cerebral venous thrombosis in Behçet's disease compared to those associated with other etiologies. J Neurol 2009; 256: 1134-42.
69. IDEGUCHI H, SUDA A, TAKENO M, UEDA A, OHNO S, ISHIGATSUBO Y: Behçet disease: evolution of clinical manifestations. Medicine (Baltimore) 2011; 90: 125-32.
70. TURKSTRA F, VAN VUGT RM, DIJKMANS BAC, YAZICI Y, YAZICI H: Results of a questionnaire on the treatment of patients with Behçet's syndrome: a trend for more intensive treatment. Clin Exp Rheumatol 2012; (Epub ahead of print).
71. TAYER-SHIFMAN OE, SEYAHI E, NOWATZKY J, BEN-CHETRIT E: Anticoagulant therapy in Behçet's disease. Clin Exp Rheumatol 2012; (Epub ahead of print).
72. DESBOIS AC, WECHSLER B, RESCHE RIGON M et al.: Immunosuppressants reduce venous thrombosis relapse in Behçet's disease. Arthritis Rheum 2012; 64: 2753-60.
73. SAADOUN D, WECHSLER B, TERRADA C et al.: Azathioprine in severe uveitis of Behçet's disease. Arthritis Care Res (Hoboken) 2010; 62: 1733-8.
74. KOSE O, SIMSEK I, PAY S: Mycophenolate sodium in the treatment of mucocutaneous Behcet's disease. International J Dermatol 2011; 50: 893-900.
75. HENDERSON CF, BRODSKY RA, JONES RJ, LEVINE SM: High dose cyclophosphamide without stem cell rescue for the treatment of refractory Behçet's disease. Semin Arthritis Rheum 2011; 41: 301-4.
76. ONAL S, KAZOKOGLU H, KOC A et al.: Long-term efficacy and safety of low-dose and dose escalating interferon alpha-2a therapy in refractory Behçet uveitis. Arch Ophthalmol 2011; 129: 288-94.
77. OLIVIERI I, LECCESE P, D'ANGELO S et al.: Efficacy of adalimumab in patients with Behçet's disease unsuccessfully treated with infliximab. Clin Exp Rheumatol 2011; 29 (Suppl. 67): S54-7.
78. ARIDA A, FRAGIADAKI K, GIAVRI E, SFIKAKIS PP: Anti TNF agents for Behçet's disease: Analysis of published data on 369 patients. Semin Arthritis Rheum 2011; 41: 61-70.
79. OKADA AA, GOTO H, OHNO S, MOCHIZUKI M: Multicenter study of infliximab for refractory uveoretinitis in Behçet disease. Arch Ophthalmol 2012; 130: 592-8.
80. HANDA T, TSUNEKAWA H, YONEDA M et al.: Long term remission of ocular and extraocular manifestations in Behçet's disease using infliximab. Clin Exp Rheumatol 2011; 29 (Suppl. 67): S58-63.
81. CANTINI F, NICCOLI L, KALOUDI O, CASSARA E, SUSINI M, LENZETTI I: Efficacy of infliximab in refractory Behçet's disease associated uveitis and idiopathic posterior segmentuveitis: a prospective, follow-up study of 50 patients. Biologics 2012; 6: 5-12.
82. KEINO H, OKADA AA, WATANABE T, TAKI W: Decreased ocular inflammatory attacks and background retinal and disc vascular leakage in patients with Behçet disease on infliximab therapy. Br J Ophthalmol 2011; 95: 1245-50.
83. MARKOMICHELAKIS N, DELICHA E, MASSELOS S, FRAGIADAKI K, KAKLAMANIS P, SFIKAKIS PP: A single infliximab infusion vs corticosteroids for acute panuveitis attacks in Behçet's disease: a comparative 4-week study. Rheumatology (Oxford) 2011; 50: 593-7.
84. MARKOMICHELAKIS N, DELICHA E, MASSELOS S, SFIKAKIS PP: Intravitreal infliximab for sight threatening relapsing uveitis in Behçet disease: a pilot study in 15 patients. Am J Ophthalmol 2012;
85. IWATA S, SAITO K, YAMAOKA K et al.: Efficacy of combination therapy of anti TNF-a antibody infliximab and methotrexate in refractory entero-Behçet's disease. Mod Rheumatol 2011; 21: 184-91.
86. ADLER S, BAUMGARTNER I, VILLIGER PM: Behçet's disease: Successful treatment with infliximab in 7 patients with severe vascular manifestations. A retrospective analysis. Arthritis Care Res 2012; 64: 607-11.
87. SCHREIBER BE, NOOR N, JULI CF, HASKARD DO: Resolution of Behçet's syndrome associated pulmonary arterial aneurysms with infliximab. Semin Arthritis Rheum 2011; 41: 482-7.
88. TOLOSA-VILELLA C, CAPELA CAP, MONTEAGUDO-JIMENEZ M, MARI-ALFONSO B: Infliximab for life threatening pulmonary artery aneurysms in Behçet's disease: a case report. Clin Exp Rheumatol 2011; 29 (Suppl. 67): S94-5.
89. D'AGOSTINO M, CALDAROLA G, FELICIANI C, DE SIMONE C: Infliximab monotherapy in Neuro-Behçet's disease: Four year follow-up in a long-standing case resistant to conventional therapies. J Neuroimmunol 2011; 239: 105-7.
90. PERRA D, ALBA MA, CALLEJAS JL et al.: Adalimumab for the treatment of Behçet's disease: experience in 19 patients. Rheumatology (Oxford) 2012;
91. GUL A, TUGAL-TUTKUN I, DINARELLO CA et al.: Interleukin-1 regulating antibody XOMA 052 (gevokizumab) in the treatment of acute exacerbations of resistant uveitis of Behçet's disease: an open label pilot study. Ann Rheum Dis 2012; 71: 563-6.
92. UGURLU S, UCAR D, SEYAHI E, HATEMI G, YURDAKUL S: Canakunimab in a patient with juvenile Behçet's syndrome with refractory eye disease. Ann Rheum Dis 2012, 71: 1589-91.
93. SHAPIRO LS, FARRELL J, HAGHIGHI AB: Tocilizumab treatment for neuro-Behçet's disease, the first report. Clin Neurology Neurosurg 2012; 114: 297-8.
94. JUNG YS, HONG SP, KIM TI et al.: Long-term clinical outcomes and factors predictive of relapse after 5-aminosalicylate or sulfasalazine therapy in patients with intestinal Behçet's disease. J Clin Gastroenterol 2012; 46: e38-e45.
95. TUZUN H, SEYAHI E, ARSLAN C, HAMURYUDAN V, BESIRLI K, YAZICI H: Management and prognosis of nonpulmonary large arterial disease in patients with Behçet disease. J Vasc Surg 2012; 55: 157-63.