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Clinical Rheumatology

, Volume 30, Issue 2, pp 157–163 | Cite as

Evaluation of current therapeutic strategies in Behçet’s disease

  • Iliana Alexoudi
  • Violetta Kapsimali
  • Aristides Vaiopoulos
  • Meletios Kanakis
  • George Vaiopoulos
Review Article

Abstract

Behçet’s disease (BD) is a chronic relapsing vasculitis with multifunctional pathogenesis. The mucocutaneous and ocular lesions are the commonest manifestations, but BD also affects the musculoskeletal, intestinal, cardiac, and central nervous system. BD therapy is based on the suppression of the inflammatory process, using immunomodulating and immunosuppressive agents. In selected cases, invasive procedures may be required.

Keywords

Behcet’s disease Biologic agents Management Organs Therapy Treatment 

Abbreviations

BD

Behcet disease

IL

Interleukin

TNFα

Tumor Necrosis Factor

PTX

Pentoxifylline

CsA

Cyclosporin A

AZA

Azathioprine

IFNα

Interferon α

ANA

Antinuclear antibodies

MMP

Matrix metalloprotease

CNS

Central nervous system

CSF

Cerebrospinal fluid

Notes

Disclosures

None

References

  1. 1.
    Alpsoy E, Akman A (2009) Behçet’s disease: an algorithmic approach to its treatment. Arch Dermatol Res 301:693–702CrossRefPubMedGoogle Scholar
  2. 2.
    Ahmad T, Wallace GR, James T et al (2003) Mapping the HLA association in Behçet’s disease: a role for tumor necrosis factor polymorphisms? Arth Rheum 48:807–813CrossRefGoogle Scholar
  3. 3.
    Ceyran H, Akcali Y, Kahraman C (2003) Surgical treatment of vasculo-Behçet’s disease. A review of patients with concomitant multiple aneurysms and venous lesions. Vasa 32:149–153CrossRefPubMedGoogle Scholar
  4. 4.
    Kapsimali V, Kanakis M, Vaiopoulos G, et al (2010) Etiopathogenesis of Behçet’s disease with emphasis on the role of immunological aberrations. Clin Rheumatol (in press)Google Scholar
  5. 5.
    Calamia KT, Kaklamanis PG (2008) Behçet’s disease: recent advances in early diagnosis and effective treatment. Curr Rheumatol Rep 10:349–355CrossRefPubMedGoogle Scholar
  6. 6.
    Gul A (2007) Standard and novel therapeutic approaches to Behçet’s disease. Drugs 67:2013–2022CrossRefPubMedGoogle Scholar
  7. 7.
    Sfikakis PP, Theodossiadis PG, Katsiari CG et al (2001) Effect of infliximab on sight-threatening panuveitis in Behçet’s disease. Lancet 358:295–296CrossRefPubMedGoogle Scholar
  8. 8.
    Lynde CB, Bruce AS, Rogers RS 3rd (2009) Successful treatment of complex aphthosis with colchicine and dapsone. Arch Dermatol 145:273–276CrossRefPubMedGoogle Scholar
  9. 9.
    Debol SM, Herron MJ, Nelson RD (1997) Anti-inflammatory action of dapsone: inhibition of neutrophil adherence is associated with inhibition of chemoattractant-induced signal transduction. J Leukoc Biol 62:827–836PubMedGoogle Scholar
  10. 10.
    Kaklamanis P, Kaklamani V (2001) Treatment of Behçet’s disease—an update. Semin Arth Rheum 30:299–312CrossRefGoogle Scholar
  11. 11.
    Hatemi G, Silman A, Bang D et al (2009) Management of Behcet disease: a systematic literature review for the European League Against Rheumatism evidence-based recommendations for the management of Behcet disease. Ann Rheum Dis 68:1528CrossRefPubMedGoogle Scholar
  12. 12.
    Seyahi E, Fresko I, Yazici H et al (2006) The management of Behcet syndrome. Acta Rheum Port 31:125–131Google Scholar
  13. 13.
    Lin P, Liang G (2006) Behçet disease: recommendation for clinical management of mucocutaneous lesions. J Clin Rheumatol 12:282–286CrossRefPubMedGoogle Scholar
  14. 14.
    Duygulu F, Evereklioglu MM et al (2005) Synovial nitric oxide concentrations are increased and correlated with serum levels in patients with active Behçet’s disease: a pilot study. Clin Rheumatol 24:324–330CrossRefPubMedGoogle Scholar
  15. 15.
    Sfikakis PP, Markomichelakis N, Alpsoy E et al (2007) Anti-TNF therapy in the management of Behcet’s disease—review and basis for recommendations. Rheumatology (Oxford) 46:736–741CrossRefGoogle Scholar
  16. 16.
    Dé Tran QH, Guay E, Chartier S, Tousignant J (2001) Tacrolimus in dermatology. J Cutan Med Surg 5:329–335CrossRefPubMedGoogle Scholar
  17. 17.
    Bruynzeel I, Van Der Raaij LM, Willemze R, Stoof TJ (1997) Pentoxifylline inhibits human T-cell adhesion to dermal endothelial cells. Arch Dermatol Res 289:189–193CrossRefPubMedGoogle Scholar
  18. 18.
    Funk JO, Ernst M, Schonharting MM, Zabel P (1995) Pentoxifylline exerts synergistic immunomodulatory effects in combination with dexamethasone or cyclosporin A. Int J Immunopharmacol 17:1007–1016CrossRefPubMedGoogle Scholar
  19. 19.
    Zouboulis CC, Orfanos CE (1998) Treatment of Adamantiades-Behçet disease with systemic interferon alfa. Arch Dermatol 134:1010–1016CrossRefPubMedGoogle Scholar
  20. 20.
    Nakamura T, Yagi H, Kurachi K, Konno H (2006) Intestinal Behcet’s disease with pyoderma gangrenosum: a case report. World J Gastroenterol 12:979–981PubMedGoogle Scholar
  21. 21.
    Fresko I, Yazici H (2008) Treatment strategies for Behçet’s disease. Expert Opin Pharmacother 9:3211–3219CrossRefPubMedGoogle Scholar
  22. 22.
    Choi IJ, Kim JS, Cha SD et al (2000) Long-term clinical course and prognostic factors in intestinal Behçet’s disease. Dis Colon Rectum 43:692–700CrossRefPubMedGoogle Scholar
  23. 23.
    Lee KS, Kim SJ, Lee BC et al (1997) Surgical treatment of intestinal Behçet’s disease. Yonsei Med J 38:455–460PubMedGoogle Scholar
  24. 24.
    Kurata M, Nozue M, Seino K et al (2006) Indications for surgery in intestinal Behçet’s disease. Hepatogastroenterology 53:60–63PubMedGoogle Scholar
  25. 25.
    Sapadin AN, Fleischmajer R (2006) Tetracyclines: nonantibiotic properties and their clinical implications. J Am Acad Dermatol 54:258–265CrossRefPubMedGoogle Scholar
  26. 26.
    Deuter CM, Kotter I, Wallace GR et al (2008) Behçet’s disease: ocular effects and treatment. Prog Retin Eye Res 27:111–136CrossRefPubMedGoogle Scholar
  27. 27.
    Kaçmaz RO, Kempen JH, Newcomb C et al (2008) Systemic immunosuppressive therapy for eye diseases cohort study group. Ocular inflammation in Behçet disease: incidence of ocular complications and of loss of visual acuity. Am J Ophthalmol 146:828–836CrossRefPubMedGoogle Scholar
  28. 28.
    Tugal-Tutkun I, Mudun A, Urgancioglu M et al (2005) Efficacy of Infliximab in the treatment of uveitis that is resistant to treatment with the combination of azathioprine, cyclosporine and corticosteroids in Behcet disease: an open-label trial. Arthritis Rheum 52:2478–2484CrossRefPubMedGoogle Scholar
  29. 29.
    Yazici H, Pazarli H, Barnes CG et al (1990) A controlled trial of azathioprine in Behçet’s syndrome. N Engl J Med 322:281–285CrossRefPubMedGoogle Scholar
  30. 30.
    Akman-Demir G, Ayranci O, Kurtuncu M et al (2008) Cyclosporine for Behçet’s uveitis: is it associated with an increased risk of neurological involvement? Clin Exp Rheumatol 26(Suppl):S84–S90PubMedGoogle Scholar
  31. 31.
    Krause L, Altenburg A, Pleyer U et al (2008) Longterm visual prognosis of patients with ocular Adamantiades-Behçet’s disease treated with interferon-alpha-2a. J Rheumatol 35:896–903PubMedGoogle Scholar
  32. 32.
    Tugal-Tutkun I, Onal S, Altan-Yaycioglu R et al (2006) Neovascularization of the optic disc in Behçet’s disease. Jpn J Ophthalmol 50:256–265CrossRefPubMedGoogle Scholar
  33. 33.
    Sfikakis PP (2002) Behçet’s disease: a new target for anti-tumour necrosis factor treatment. Ann Rheum Dis 61(Suppl 2):ii51–ii53PubMedGoogle Scholar
  34. 34.
    Elezoglou A, Kafasi N, Kaklamanis PH et al (2007) Infliximab treatment-induced formation of autoantibodies is common in Behçet’s disease. Clin Exp Rheumatol 25(Suppl):S65–S69PubMedGoogle Scholar
  35. 35.
    Krause L, Altenburg A, Bechrakis NE et al (2007) Intraocular surgery under systemic interferon-alpha therapy in ocular Adamantiades-Behçet’s disease. Graefes Arch Clin Exp Ophthalmol 245:1617–1621CrossRefPubMedGoogle Scholar
  36. 36.
    Elgin U, Berker N, Batman A, Soykan E (2007) Nd:YAG laser iridotomy in the management of secondary glaucoma associated with Behçet’s disease. Eur J Ophthalmol 17:191–195PubMedGoogle Scholar
  37. 37.
    Sullu Y, Alotaiby H, Beden U, Erkan D (2005) Pars plana vitrectomy for ocular complications of Behçet’s disease. Ophthalmic Surg Lasers Imaging 36:292–297PubMedGoogle Scholar
  38. 38.
    Soylu M, Demircan N, Pelit A (2001) Pars plana vitrectomy in ocular Behçet’s disease. Int Ophthalmol 24:219–223CrossRefPubMedGoogle Scholar
  39. 39.
    Han JK, Kim HK, Kim YJ et al (2009) Behçet’s disease as a frequently unrecognized cause of aortic regurgitation: suggestive and misleading echocardiography findings. J Am Soc Echocardiogr 22:1269–1274CrossRefPubMedGoogle Scholar
  40. 40.
    Marzban M, Mandegar MH, Karimi A et al (2008) Cardiac and great vessel involvement in “Behcet’s disease”. J Card Surg 23:765–768CrossRefPubMedGoogle Scholar
  41. 41.
    Imazio M, Brucato A, Trinchero R et al (2009) Colchicine for pericarditis: hype or hope? Eur Heart J 30:532–539CrossRefPubMedGoogle Scholar
  42. 42.
    Arishiro K, Nariyama J, Hoshiga M et al (2006) Vascular Behçet’s disease with coronary artery aneurysm. Intern Med 45:903–907CrossRefPubMedGoogle Scholar
  43. 43.
    Ozen S, Bilginer Y, Besbas N et al (2010) Behçet disease: treatment of vascular involvement in children. Eur J Pediatr 169:427–430CrossRefPubMedGoogle Scholar
  44. 44.
    Oh SH, Lee JH, Shin JU, Bang D (2008) Dermatological features in Behçet disease-associated vena cava obstruction. Br J Dermatol 159:555–560CrossRefPubMedGoogle Scholar
  45. 45.
    Alpagut U, Ugurlucan M, Dayioglu E (2007) Major arterial involvement and review of Behçet’s disease. Ann Vasc Surg 21:232–239CrossRefPubMedGoogle Scholar
  46. 46.
    Abdul-Hussien H, Hanemaaijer R, Verheijen JH et al (2009) Doxycycline therapy for abdominal aneurysm: improved proteolytic balance through reduced neutrophil content. J Vasc Surg 49:741–749CrossRefPubMedGoogle Scholar
  47. 47.
    Siva A, Saip S (2009) The spectrum of nervous system involvement in Behçet’s syndrome and its differential diagnosis. J Neurol 256:513–529CrossRefPubMedGoogle Scholar
  48. 48.
    Borhani Haghighi A (2009) Treatment of neuro-Behçet’s disease: an update. Expert Rev Neurother 9:565–574CrossRefPubMedGoogle Scholar
  49. 49.
    Gono T, Murata M, Kawaguchi Y et al (2009) Successful treatment for sympathetic storms in a patient with neuro-Behçet’s disease. Clin Rheumatol 28:357–359CrossRefPubMedGoogle Scholar

Copyright information

© Clinical Rheumatology 2010

Authors and Affiliations

  • Iliana Alexoudi
    • 1
  • Violetta Kapsimali
    • 2
  • Aristides Vaiopoulos
    • 1
  • Meletios Kanakis
    • 1
  • George Vaiopoulos
    • 1
  1. 1.First Department of Internal MedicineUniversity of Athens, Laikon HospitalAthensGreece
  2. 2.Department of Microbiology, Medical SchoolUniversity of AthensAthensGreece

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