Skip to main content

Treatment of Neuro-Behçet’s Disease

  • Chapter
  • First Online:
Neuro-Behçet’s Disease

Abstract

There are no randomized trials on the treatment of neuro-Behçet’s disease (NBD). Therefore, management is mainly based on studies performed on patients with systemic involvements or case studies. The management of NBD can be summarized as acute treatment and maintenance treatment. The acute treatment consists of high-dose intravenous steroids, followed by an oral taper. An escalation treatment strategy is employed for the maintenance treatment that includes first-line oral immunosuppressants such as azathioprine and mycophenolate mofetil. Cyclophosphamide and anti-TNF-α monoclonal antibodies can be used in refractory cases as second-line treatments. An increasing body of evidence suggests the use of anti-IL-6 and IL-1 monoclonal antibodies for patients with progressive or relapsing disease course on second-line agents.

This is a preview of subscription content, log in via an institution to check access.

Access this chapter

Chapter
USD 29.95
Price excludes VAT (USA)
  • Available as PDF
  • Read on any device
  • Instant download
  • Own it forever
eBook
USD 139.00
Price excludes VAT (USA)
  • Available as EPUB and PDF
  • Read on any device
  • Instant download
  • Own it forever
Softcover Book
USD 179.99
Price excludes VAT (USA)
  • Compact, lightweight edition
  • Dispatched in 3 to 5 business days
  • Free shipping worldwide - see info
Hardcover Book
USD 179.99
Price excludes VAT (USA)
  • Durable hardcover edition
  • Dispatched in 3 to 5 business days
  • Free shipping worldwide - see info

Tax calculation will be finalised at checkout

Purchases are for personal use only

Institutional subscriptions

References

  1. Kotake S, Higashi K, Yoshikawa K, Sasamoto Y, Okamoto T, Matsuda H. Central nervous system symptoms in patients with Behçet disease receiving cyclosporine therapy. Ophthalmology. 1999;106(3):586–9.

    Article  CAS  PubMed  Google Scholar 

  2. Akman-Demir G, Ayranci O, Kurtuncu M, Vanli EN, Mutlu M, Tugal-Tutkun I. Cyclosporine for Behçet’s uveitis: is it associated with an increased risk of neurological involvement? Clin Exp Rheumatol. 2008;26(4 Suppl 50):S84–90.

    CAS  PubMed  Google Scholar 

  3. Kürtüncü M, Tüzün E, Akman-Demir G. Behçet’s disease and nervous system involvement. Curr Treat Options Neurol. 2016;18(5):19.

    Article  PubMed  Google Scholar 

  4. Sorensen PS, Mellgren SI, Svenningsson A, Elovaara I, Frederiksen JL, Beiske AG, Myhr KM, Søgaard LV, Olsen IC, Sandberg-Wollheim M. NORdic trial of oral Methylprednisolone as add-on therapy to Interferon beta-1a for treatment of relapsing-remitting Multiple Sclerosis (NORMIMS study): a randomized, placebo-controlled trial. Lancet Neurol. 2009;8(6):519–29.

    Article  CAS  PubMed  Google Scholar 

  5. Seider N, Beiran I, Scharf J, Miller B. Intravenous immunoglobulin therapy for resistant ocular Behçet’s disease. Br J Ophthalmol. 2001;85(11):1287–8.

    Article  CAS  PubMed  PubMed Central  Google Scholar 

  6. Cantarini L, Stromillo ML, Vitale A, Lopalco G, Emmi G, Silvestri E, Federico A, Galeazzi M, Iannone F, De Stefano N. Efficacy and safety of intravenous immunoglobulin treatment in refractory Behcet’s disease with different organ involvement: a case series. Isr Med Assoc J. 2016;18(3–4):238–42.

    PubMed  Google Scholar 

  7. Noel N, Bernard R, Wechsler B, Resche-Rigon M, Depaz R, Le Thi Huong Boutin D, et al. Long-term outcome of neuro-Behçet’s disease. Arthritis Rheumatol. 2014;66D5:1306–14.

    Article  Google Scholar 

  8. Kikuchi H, Aramaki K, Hirohata S. Low dose MTX for progressive neuro-Behçet’s disease. A follow-up study for 4 years. Adv Exp Med Biol. 2003;528:575–8.

    Article  CAS  PubMed  Google Scholar 

  9. Hirohata S, Kikuchi H, Sawada T, Nagafuchi H, Kuwana M, Takeno M, et al. Retrospective analysis of long- term outcome of chronic progressive neurological manifestations in Behcet’s disease. J Neurol Sci. 2015;349(1–2):143–8.

    Article  PubMed  Google Scholar 

  10. Uygunoğlu U, Siva A. Nervous system involvement in Behçet’s syndrome. Curr Opin Rheumatol. 2019;31(1):32–9.

    Article  PubMed  Google Scholar 

  11. Hatemi G, Christensen R, Bang D, Bodaghi B, Celik AF, Fortune F, et al. 2018 update of the EULAR recommendations for the management of Behçet’s syndrome. Ann Rheum Dis. 2018;77:808–18.

    PubMed  Google Scholar 

  12. Yazici H, Pazarli H, Barnes CG, Tüzün Y, Özyazgan Y, Silman A, et al. A controlled trial of azathioprine in Behçet’s syndrome. N Engl J Med. 1990;322:281–5.

    Article  CAS  PubMed  Google Scholar 

  13. Hamuryudan V, Ozyazgan Y, Hizli N, Mat C, Yurdakul S, Tüzün Y, et al. Azathioprine in Behcet’s syndrome: effects on long-term prognosis. Arthritis Rheum. 1997;40:769–74.

    Article  CAS  PubMed  Google Scholar 

  14. Kurtuncu M, Tuzun E, Pehlivan M, et al. Clinical patterns and treatment effect on neuro-Behçet’s disease: analysis of 351 patients. Clin Exp Rheumatol. 2008;26(Suppl 50):S43.

    Google Scholar 

  15. Yazici H, Seyahi E, Hatemi G, Yazici Y. Behçet syndrome: a contemporary view. Nat Rev Rheumatol. 2018;14:107–19.

    Article  CAS  PubMed  Google Scholar 

  16. Akman-Demir G, Saip S, Siva A. Behcet’s disease. Curr Treat Options Neurol. 2011;13(3):290–310.

    Article  PubMed  Google Scholar 

  17. Hamuryudan V, Ozyazgan Y, Fresko Y, Mat C, Yurdakul S, Yazici H. Interferon alfa combined with azathioprine for the uveitis of Behçet’s disease: an open study. Isr Med Assoc J. 2002;4:928–30.

    CAS  PubMed  Google Scholar 

  18. Shahram F, Davatchi F, Chams H, Nadji A, Jamshidi A, Akbarian M, et al. Azathioprine and low dose pulse cyclophosphamide in severe ocular lesions of Behçet’s disease. A preliminary report. Adv Exp Med Biol. 2003;528:571–3.

    Article  CAS  PubMed  Google Scholar 

  19. Saip S, Akman-Demir G, Siva A. Neuro-Behçet syndrome. Handb Clin Neurol. 2014;121:1703–23.

    Article  PubMed  Google Scholar 

  20. Allison AC, Almquist SJ, Muller CD, Eugui EM. In vitro immunosuppressive effects of mycophenolic acid and an ester prodrug, RS- 61443. Transplant Proc. 1991;23(suppl 2):10–4.

    CAS  PubMed  Google Scholar 

  21. Allison AC, Eugui EM. Mycophenolate mofetil, a rationally designed immunosuppressive drug. Clin Transpl. 1993;7:96–112.

    Google Scholar 

  22. Durez P, Appelboom T, Pira C, Stordeur P, Vray B, Goldman M. Anti-inflammatory properties of mycophenolate mofetil in murine endotoxemia: inhibition of TNF-alpha and upregulation of IL-10 release. Int J Immunopharmacol. 1999;21:581–7.

    Article  CAS  PubMed  Google Scholar 

  23. Shugaiv E, Tüzün E, Mutlu M, Kiyat-Atamer A, Kurtuncu M, Akman-Demir G. Mycophenolate mofetil as a novel immunosuppressant in the treatment of neuro-Behçet’s disease with parenchymal involvement: presentation of four cases. Clin Exp Rheumatol. 2011;29(4 Suppl 67):S64–7.

    PubMed  Google Scholar 

  24. Ferreira BFA, Rodriguez EEC, Prado LLD, Gonçalves CR, Hirata CE, Yamamoto JH. Frosted branch angiitis and cerebral venous sinus thrombosis as an initial onset of neuro-Behçet’s disease: a case report and review of the literature. J Med Case Rep. 2017;11(1):104.

    Article  PubMed  PubMed Central  Google Scholar 

  25. Kalra S, Silman A, Akman-Demir G, Bohlega S, Borhani-Haghighi A, Constantinescu CS, et al. Diagnosis and management of Neuro-Behcet’s disease: international consensus recommendations. J Neurol. 2014;261:1662–76.

    Article  PubMed  Google Scholar 

  26. Borhani Haghighi A, Kardeh B, Banerjee S, Yadollahikhales G, Safari A, Sahraian MA, Shapiro L. Neuro-Behçet’s disease: an update on diagnosis, differential diagnosis, and treatment. Mult Scler Relat Disord. 2019;39:101906.

    Article  PubMed  Google Scholar 

  27. Shahram F, Davatchi F, Chams H, Akbarian M, Chams C, Tebbi ME. Methotrexate in ocular Behçet’s disease, Preliminary report, Abstract FP-23. First APLAR Symposium on the Therapy of Rheumatic Diseases, Seoul, Korea, 1990.

    Google Scholar 

  28. Shahram F, Davatchi F, Chams H, Akbarian M, Chams C. Pulse cyclophosphamide versus pulse methotrexate in ophthalmological manifestations of Behcet’s disease. In: Nasution AR, Darmawan J, Isbagio H, editors. APLAR rheumatology. Tokyo: Churchill Livingstone; 1992. p. 119–22.

    Google Scholar 

  29. Shahram F, Davatchi F, Chams H, Akbarian M, Gharibdoost F. Comparing 3 methods of Cytotoxic therapy in ophthalmologic lesions of Behcet’s disease. In: Godeau P, Wechsler B, editors. Behcet’s disease. Amsterdam: Elsevier Science Publishers BV; 1993. p. 635–40.

    Google Scholar 

  30. Shahram F, Chams H, Davatchi F, Nadji A, Jamshidi AR, Akbarian M. Intermediate-term results of combination therapy with low dose pulse cyclophosphamide and methotrexate in ocular lesions of Behcet’s disease. In: Bang D, Lee ES, Lee S, editors. Behcet’s disease. Seoul: Design Mecca Publishing; 2000. p. 453–6.

    Google Scholar 

  31. Davatchi F, Shahram F, Chams H, et al. Methotrexate for ocular lesions of Behçet’s disease. Cohort study on 262 patients. Arthritis Rheum. 1998;41:S356.

    Google Scholar 

  32. Davatchi F, Shahram F, Chams H, et al. High dose methotrexate for ocular lesions of Behçet’s disease. Preliminary short-term results. Adv Exp Med Biol. 2003;528:579–84.

    Article  CAS  PubMed  Google Scholar 

  33. Davatchi F, Shams H, Shahram F, et al. Methotrexate in ocular manifestations of Behçet’s disease: a longitudinal study up to 15 years. Int J Rheum Dis. 2013;16:568–77.

    Article  CAS  PubMed  Google Scholar 

  34. Park J, Cheon JH, Park Y, Park SJ, Kim TI, Kim WH. Efficacy and tolerability of methotrexate therapy for refractory intestinal Behçet’s disease: a single-center experience. Intest Res. 2018;16(2):315–8.

    Article  PubMed  PubMed Central  Google Scholar 

  35. Hirohata S, Suda H, Hashimoto T. Low-dose weekly methotrexate for progressive neuropsychiatric manifestations in Behçet’s disease. J Neurol Sci. 1998;159:181–5.

    Article  CAS  PubMed  Google Scholar 

  36. Saleh Z, Arayssi T. Update on the therapy of Behçet disease. Ther Adv Chronic Dis. 2014;5:112–34.

    Article  CAS  PubMed  PubMed Central  Google Scholar 

  37. Hatemi G, Silman A, Bang D, et al. EULAR recommendations for the management of Behçet disease. Ann Rheum Dis. 2008;67:1656–62.

    Article  CAS  PubMed  Google Scholar 

  38. Hamuryudan V, Yurdakul S, Moral F, et al. Pulmonary arterial aneurysms in Behçet’s syndrome: a report of 24 cases. Br J Rheumatol. 1994;33:48–51.

    Article  CAS  PubMed  Google Scholar 

  39. Hamuryudan V, et al. Pulmonary artery aneurysms in Behçet syndrome. Am J Med. 2004;117:867–70.

    Article  PubMed  Google Scholar 

  40. Davatchi F, Shahram F, Chams H, Akbarian M, et al. Pulse cyclophosphamide (PCP) for ocular lesions of Behçet’s disease: double-blind crossover study. Arthritis Rheum. 1999;42:S320.

    Google Scholar 

  41. Davatchi F, Sadeghi Abdollahi B, Shams H, Shahram F, Nadji A, et al. Combination of pulse cyclophosphamide and azathioprine in ocular manifestations of Behçet’s disease: a longitudinal study of up to 10 years. Int J Rheum Dis. 2014;17:444–52.

    Article  CAS  PubMed  Google Scholar 

  42. Ait Ben Haddou E, Imounan F, Regragui W, et al. Neurological manifestations of Behçet’s disease: evaluation of 40 patients treated by cyclophosphamide. Rev Neurol Paris. 2012;168:344–9.

    Article  CAS  PubMed  Google Scholar 

  43. Noel N, Bernard R, Wechsler B, Resche-Rigon M, Depaz R, Le Thi Huong Boutin D, et al. Long-term outcome of neuro-Behçet’s disease. Arthritis Rheumatol. 2014;66:1306–14.

    Article  PubMed  Google Scholar 

  44. Yoon D-L, Kim YJ, Koo BS, Kim Y-G, Lee C-K, Yoo B. Neuro-behçet’s disease in South Korea: clinical characteristics and treatment response. Int J Rheum Dis. 2014;17:453–8.

    Article  PubMed  Google Scholar 

  45. Esatoglu SN, Hatemi G. Update on the treatment of Behçet’s syndrome. Intern Emerg Med. 2019;14:661–75.

    Article  PubMed  Google Scholar 

  46. Yilmaz N, Emmungil H, Gucenmez S, Ozen G, Yildiz F, Balkarli A, et al. Incidence of cyclophosphamide-induced urotoxicity and protective effect of mesna in rheumatic diseases. J Rheumatol. 2015;42(9):1661–6.

    Article  CAS  PubMed  Google Scholar 

  47. 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(1):61–70.

    Article  CAS  PubMed  Google Scholar 

  48. Vallet H, Riviere S, Sanna A, Deroux A, Moulis G, Addimanda O, et al. Efficacy of anti-TNF alpha in severe and/or refractory Behçet’s disease: multicenter study of 124 patients. J Autoimmun. 2015;62:67–74.

    Article  CAS  PubMed  Google Scholar 

  49. Adler S, Baumgartner I, Villiger PM. Behcet’s disease: successful treatment with infliximab in 7 patients with severe vascular manifestations. A retrospective analysis. Arthritis Care Res (Hoboken). 2012;64:607–11.

    Article  CAS  Google Scholar 

  50. Emmi G, Bettiol A, Silvestri E, Di Scala G, Becatti M, Fiorillo C, et al. Vascular Behçet’s syndrome: an update. Intern Emerg Med. 2018;14:645–65.

    Article  PubMed  Google Scholar 

  51. Hamuryudan V, Seyahi E, Ugurlu S, Melikoglu M, Hatemi G, Ozguler Y, et al. Pulmonary artery involvement in Behçet’s syndrome: effects of anti-TNF treatment. Semin Arthritis Rheum. 2015;45:369–73.

    Article  CAS  PubMed  Google Scholar 

  52. Desbois AC, Biard L, Addimanda O, Lambert M, Hachulla E, Launay D, Ackermann F, Perard L, Hot A, Maurier F, Mausservey C, Bernard F, Noel N, Alric L, Mirault T, Cohen F, Boussouar S, Resche-Rigon M, Cacoub P, Saadoun D. Efficacy of anti-TNF alpha in severe and refractory major vessel involvement of Behcet’s disease: a multicenter observational study of 18 patients. Clin Immunol. 2018;197:54–9.

    Article  CAS  PubMed  Google Scholar 

  53. Sfikakis PP, Theodossiadis PG, Katsiari CG, Kaklamanis P, Markomichelakis NN. Effect of infliximab on sight-threatening panuveitis in Behçet’s disease. Lancet. 2001;358:295–6.

    Article  CAS  PubMed  Google Scholar 

  54. Sfikakis PP, Kaklamanis PH, Elezoglou A, et al. Infliximab for recurrent, sight-threatening ocular inflammation in Adamantiades-Behcet disease. Ann Intern Med. 2004;140(5):404–6.

    Article  CAS  PubMed  Google Scholar 

  55. Cantini F, Niccoli L, Nannini C, et al. Efficacy of infliximab in refractory Behçet’s disease-associated and idiopathic posterior segment uveitis: a prospective, follow-up study of 50 patients. Biologics. 2012;6(5):5–12.

    CAS  PubMed  Google Scholar 

  56. Tugal-Tutkun I, Mudun A, Urgancioglu M, Kamali S, Kasapoglu E, Inanc M, et al. Efficacy of infliximab in the treatment of uveitis that is resistant to treatment with the combination of azathioprine, cyclosporine, and corticosteroids in Behçet’s disease: an open-label trial. Arthritis Rheum. 2005;52:2478–84.

    Article  CAS  PubMed  Google Scholar 

  57. Fabiani C, Sota J, Rigante D, Vitale A, Emmi G, Lopalco G, Vannozzi L, Guerriero S, Bitossi A, Orlando I, Franceschini R, Frediani B, Galeazzi M, Iannone F, Tosi GM, Cantarini L. Efficacy of adalimumab and infliximab in recalcitrant retinal vasculitis inadequately responsive to other immunomodulatory therapies. Clin Rheumatol. 2018;37:2805–9.

    Article  PubMed  Google Scholar 

  58. Guzelant G, Uçar D, Esatoglu SN, et al. Infliximab for uveitis of Behçet’s syndrome: a trend for earlier initiation. Clin Exp Rheumatol. 2017;35(Suppl. 108):S86–9.

    Google Scholar 

  59. Keino H, Okada AA, Watanabe T, Nakayama M, Nakamura T. Efficacy of infliximab for early remission induction in refractory uveoretinitis associated with Behçet’s disease: a 2-year follow-up study. Ocul Immunol Inflamm. 2017;25:46–51.

    Article  CAS  PubMed  Google Scholar 

  60. Naganuma M, Sakuraba A, Hisamatsu T, et al. Efficacy of infliximab for induction and maintenance of remission in intestinal Behçetʼs disease. Inflamm Bowel Dis. 2008;14:1259–64.

    Article  PubMed  Google Scholar 

  61. Iwata S, Saito K, Yamaoka K, et al. Efficacy of combination therapy of anti-TNF-α antibody infliximab and methotrexate in refractory entero-Behçet’s disease. Mod Rheumatol. 2011;21:184–91.

    Article  CAS  PubMed  Google Scholar 

  62. Lee JH, Cheon JH, Jeon SW, et al. Efficacy of infliximab in intestinal Behçet’s disease: a Korean multicenter retrospective study. Inflamm Bowel Dis. 2013;19:1833–8.

    PubMed  Google Scholar 

  63. Kinoshita H, Kunisaki R, Yamamoto H, et al. Efficacy of infliximab in patients with intestinal Behçet’s disease refractory to conventional medication. Intern Med. 2013;52:1855–62.

    Article  PubMed  CAS  Google Scholar 

  64. Inoue N, Kobayashi K, Naganuma M, et al. Long-term safety and efficacy of adalimumab for intestinal Behçet’s disease in the open-label study following a phase 3 clinical trial. Intest Res. 2017;15:395–401.

    Article  PubMed  PubMed Central  Google Scholar 

  65. Licata G, Pinto A, Tuttolomondo A, Banco A, Ciccia F, Ferrante A, Triolo G. Anti-tumour necrosis factor-alpha monoclonal antibody therapy for recalcitrant cerebral vasculitis in a patient with Behcet’s syndrome. Ann Rheum Dis. 2003;62:280–1.

    Article  CAS  PubMed  PubMed Central  Google Scholar 

  66. Sarwar H, McGrath H Jr, Espinoza LR. Successful treatment of long-standing neuro-Behcet’s disease with infliximab. J Rheumatol. 2005;32:181–3.

    PubMed  Google Scholar 

  67. Ribi C, Sztajzel R, Delavelle J, Chizzolini C. Efficacy of TNF(alpha) blockade in cyclophosphamide resistant neuro-Behcet disease. J Neurol Neurosurg Psychiatry. 2005;76:1733–5.

    Article  CAS  PubMed  PubMed Central  Google Scholar 

  68. Fujikawa K, Aratake K, Kawakami A, Aramaki T, Iwanaga N, Izumi Y, Arima K, Kamachi M, Tamai M, Huang M, Nakamura H, Nishiura Y, Origuchi T, Ida H, Eguchi K. Successful treatment of refractory neuro-Behcet’s disease with infliximab: a case report to show its efficacy by magnetic resonance imaging, transcranial magnetic stimulation and cytokine profile. Ann Rheum Dis. 2007;66:136–7.

    Article  CAS  PubMed  PubMed Central  Google Scholar 

  69. Alty JE, Monaghan TM, Bamford JM. A patient with neuro-Behcet’s disease is successfully treated with etanercept: further evidence for the value of TNFalpha blockade. Clin Neurol Neurosurg. 2007;109:279–81.

    Article  PubMed  Google Scholar 

  70. Belzunegui J, Lopez L, Paniagua I, Intxausti JJ, Maiz O. Efficacy of infliximab and adalimumab in the treatment of a patient with severe neuro-Behcet’s disease. Clin Exp Rheumatol. 2008;26:S133–4.

    CAS  PubMed  Google Scholar 

  71. Madanat WY, Madanat AY. Worsening of osteomalacia in a 918 patient successfully treated for neuro-Behcet’s disease with infliximab. Clin Exp Rheumatol. 2008;26:S128–9.

    CAS  PubMed  Google Scholar 

  72. Abalos-Medina GM, Sanchez-Cano D, Ruiz-Villaverde G, Ruiz-Villaverde R, Quirosa Flores S, Raya Alvarez E. Successful use of infliximab in a patient with neuro-Behcet’s disease. Int J Rheum Dis. 2009;12:264–6.

    Article  PubMed  Google Scholar 

  73. Borhani-Haghighi A, Safari A, Nazarinia MA, Habibagahi Z, Shenavandeh S. Infliximab for patients with neuro-Behcet’s disease: case series and literature review. Clin Rheumatol. 2011;30(7):1007–12.

    Article  PubMed  Google Scholar 

  74. Fasano A, D’Agostino M, Caldarola G, Feliciani C, De Simone C. Infliximab monotherapy in neuro-Behcet’s disease: four-year follow-up in a long-standing case resistant to conventional therapies. J Neuroimmunol. 2011;239:105–7.

    Article  CAS  PubMed  Google Scholar 

  75. Coulter I, Huda S, Baborie A, et al. Longitudinally extensive transverse myelitis as the sole presentation of neuro-Behçet’s disease responding to infliximab. J Spinal Cord Med. 2012;35:122–4.

    Article  PubMed  PubMed Central  Google Scholar 

  76. Kuroda R, Suzuki J, Muramatsu M, Tasaki A, Yano M, Imai N, et al. Efficacy of infliximab in neuro-Behçet’s disease presenting with isolated longitudinally extensive transverse myelitis. J Neurol. 2013;260(12):3167–70.

    Article  PubMed  PubMed Central  Google Scholar 

  77. Di Filippo M, Di Gregorio M, Nannini C, Gaetani L, Gallina C, Floridi P, et al. Infliximab monotherapy for neuro-Behçet’s disease: a case report. J Neurol Sci. 2014;347(1–2):389–90.

    Article  PubMed  Google Scholar 

  78. Uygunoglu U, Pasha M, Saip S, Siva A. Recurrent longitudinal extensive transverse myelitis in a neuro-Behçet syndrome treated with infliximab. J Spinal Cord Med. 2015;38(1):111–4.

    Article  PubMed  PubMed Central  Google Scholar 

  79. Barešić M, Reihl M, Habek M, Vukojević N, Anić B. Improvement of neurological and ocular symptoms of Behçet’s disease after the introduction of infliximab. Rheumatol Int. 2018;38:1301–6.

    Article  PubMed  CAS  Google Scholar 

  80. Nigro A, Zuccoli G, Boiardi L, Salvarani C. Infliximab for the treatment of neuro-Behcet’s disease: a case series and review of the literature. Arthritis Rheum. 2008;59:285–90.

    Article  PubMed  CAS  Google Scholar 

  81. Kikuchi H, Aramaki K, Hirohata S. Effect of infliximab in progressive Neuro-Behçet’s syndrome. J Neurol Sci. 2008;272:99–105.

    Article  CAS  PubMed  Google Scholar 

  82. Matsui T, Ishida T, Tono T, Yoshida T, Sato S, Hirohata S. An attack of acute neuro-Behçet’s disease during the course of chronic progressive neuro-Behçet’s disease: report of two cases. Mod Rheumatol. 2010;20:621–6.

    Article  PubMed  Google Scholar 

  83. Al-araji A, Saip ASS, Constantinescu C, Akman-Demir G, Arayssi T, Espinoza L, Findling O, Garcia F, Hirohata S, Keogan M, Lo Monaco A, Pay S, Ramo C, Van Laar J, Zandi M. Treatment of neuro-Behcet’s disease with infliximab. An International Multicentre Case-Series of 18 patients. Clin Exp Rheumatol. 2010;28:S119.

    Google Scholar 

  84. Zeydan B, Uygunoglu U, Saip S, et al. Infliximab is a plausible alternative for & neurologic complications of Behcet disease. Neurol Neuroimmunol Neuroinflamm. 2016;3:e258.

    Article  PubMed  PubMed Central  Google Scholar 

  85. Giardina A, Ferrante A, Ciccia F, Vadalà M, Giardina E, Triolo G. 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. 2011;31:33–7.

    Article  CAS  PubMed  Google Scholar 

  86. Desbois AC, Addimanda O, Bertrand A, Deroux A, Pérard L, Depaz R, et al. Efficacy of anti-TNFα in severe and refractory neuro-Behcet disease. Medicine. 2016;95:e3550.

    Article  CAS  PubMed  PubMed Central  Google Scholar 

  87. Hibi T, Hirohata S, Kikuchi H, et al. Infliximab therapy for intestinal, neurological, and vascular involvement in Behcet disease: efficacy, safety, and pharmacokinetics in a multicenter, prospective, open-label, single-arm phase 3 study. Medicine (Baltimore). 2016;95:e3863.

    Article  CAS  Google Scholar 

  88. Sfikakis PP, Arida A, Panopoulos S, Fragiadaki K, Pentazos G, Laskari K, et al. Brief report: drug-free long-term remission in severe Behçet’s disease following withdrawal of successful anti-tumorr necrosis factor treatment. Arthritis Rheumatol. 2017;69(12):2380–5.

    Article  CAS  PubMed  Google Scholar 

  89. Ryu YS, Park SH, Kim JM, Kim EJ, Lee J, Kwok SK, et al. A case of leukoencephalopathy associated with adalimumab-treated rheumatoid arthritis and a review of literature. Rheumatol Int. 2011;32(11):3481–5.

    Article  PubMed  Google Scholar 

  90. Melikoglu M, Fresko I, Mat C, et al. Short-term trial of etanercept in Behçet’s disease: a double blind, placebo controlled study. J Rheumatol. 2005;32:98–105.

    CAS  PubMed  Google Scholar 

  91. Atzeni F, Sarzi-Puttini P, Capsoni F, Mecchia M, Marazza MG, Carrabba M. Successful treatment of resistant Behcet’s disease with etanercept. Clin Exp Rheumatol. 2005;23:729.

    CAS  PubMed  Google Scholar 

  92. Scheinberg MA. Treatment of recurrent oral aphthous ulcers with etanercept. Clin Exp Rheumatol. 2005;23:733–4.

    Google Scholar 

  93. Sommer A, Altmeyer P, Kreuter A. A case of mucocutaneous Behçet’s disease responding to etanercept. J Am Acad Dermatol. 2005;52:717–9.

    Article  PubMed  Google Scholar 

  94. Curigliano V, Giovinale M, Fonnesu C, et al. Efficacy of etanercept in the treatment of a patient with Behçet’s disease. Clin Rheumatol. 2008;27:933–6.

    Article  PubMed  Google Scholar 

  95. Zhang MF, Zhao C, Wen X, et al. The short-term efficacy and safety treatment study of recurrent uveitis in Behçet’s disease with etanercept. Zhonghua Yan Ke Za Zhi. 2010;46(2):145–50.

    CAS  PubMed  Google Scholar 

  96. Ma D, Zhang CJ, Wang RP, Wang L, Yang H. Etanercept in the treatment of intestinal Behçet’s disease. Cell Biochem Biophys. 2014;69:735–9.

    Article  CAS  PubMed  Google Scholar 

  97. Alty JE, Monaghan TM, Bamford JM. A patient with neuro-Behçet’s disease is successfully treated with etanercept: further evidence for the value of TNFalpha blockade. Clin Neurol Neurosurg. 2007;109:279–81.

    Article  PubMed  Google Scholar 

  98. Sfikakis PP, Markomichelakis N, Alpsoy E, Assaad-Khalil S, Bodaghi B, Gul A, et al. Anti-TNF therapy in the management of Behçet’s disease–review and basis for recommendations. Rheumatology (Oxford). 2007;46:736–41.

    Article  CAS  Google Scholar 

  99. Cantarini L, Tinazzi I, Caramaschi P, Bellisai F, Brogna A, Galeazzi M. Safety and efficacy of etanercept in children with juvenile-onset Behçet’s disease. Int J Immunopathol Pharmacol. 2009;22(2):551–5.

    Article  CAS  PubMed  Google Scholar 

  100. Saurenmann RK, Levin AV, Rose JB, et al. Tumour necrosis factor {alpha} inhibitors in the treatment of childhood uveitis. Rheumatology. 2006;45:982–9.

    Article  CAS  PubMed  Google Scholar 

  101. Jaffe GJ, Dick AD, Brézin AP, Nguyen QD, Thorne JE, Kestelyn P, et al. Adalimumab in patients with active noninfectious uveitis. N Engl J Med. 2016;375:932–43.

    Article  CAS  PubMed  Google Scholar 

  102. Nguyen QD, Merrill PT, Jaffe GJ, Dick AD, Kurup SK, Sheppard J, et al. Adalimumab for prevention of uveitic flare in patients with inactive non-infectious uveitis controlled by corticosteroids (VISUAL II): a multicentre, double-masked, randomized, placebo-controlled phase 3 trial. Lancet. 2016;388:1183–92.

    Article  CAS  PubMed  Google Scholar 

  103. Fabiani C, Vitale A, Emmi G, Vannozzi L, Lopalco G, Guerriero S, et al. Efficacy and safety of adalimumab in Behçet’s disease-related uveitis: a multicenter retrospective observational study. Clin Rheumatol. 2017;36:183–9.

    Article  PubMed  Google Scholar 

  104. Fabiani C, Sota J, Vitale A, et al. Cumulative retention rate of adalimumab in patients with Behçet’s disease-related uveitis: a four-year follow-up study. Br J Ophthalmol. 2018;102:637–41.

    Article  PubMed  Google Scholar 

  105. Fabiani C, Vitale A, Emmi G, et al. Long- term retention rates of adalimumab and infliximab in non-infectious intermediate, posterior and panuveitis. Clin Rheumatol. 2018;38:63–70.

    Article  PubMed  Google Scholar 

  106. Bitossi A, Bettiol A, Silvestri E, Di Scala G, Bacherini D, Lopalco G, et al. Adalimumab accounts for long-term control of non-infectious uveitis also in the absence of concomitant DMARD treatment: a multicenter retrospective study. Mediat Inflamm. 2019;2019:1623847.

    Article  CAS  Google Scholar 

  107. Atienza-Mateo B, Martin-Varillas JL, Calvo-Rio V, et al. Comparative study of infliximab versus adalimumab in refractory uveitis due to Behçet’s disease. National multicenter study of 177 cases. Arthritis Rheumatol. 2019;71(12):2081–9.

    Article  CAS  PubMed  Google Scholar 

  108. Fabiani C, Vitale A, Rigante D, et al. Comparative efficacy between adalimumab and infliximab in the treatment of non-infectious intermediate uveitis, posterior uveitis, and panuveitis: a retrospective observational study of 107 patients. Clin Rheumatol. 2019;38:407–15.

    Article  PubMed  Google Scholar 

  109. Emmi G, Vitale A, Silvestri E, et al. Adalimumab-based treatment versus disease-modifying antirheumatic drugs for venous thrombosis in Behcet’s syndrome. Arthritis Rheumatol. 2018;70:1500–7.

    Article  CAS  PubMed  Google Scholar 

  110. Tanida S, Inoue N, Kobayashi K, et al. Adalimumab for the treatment of Japanese patients with intestinal Behçet’s disease. Clin Gastroenterol Hepatol. 2015;13:940–8 e3.

    Article  CAS  PubMed  Google Scholar 

  111. Mizoshita T, Katano T, Tanida S, Hirano A, Miyaki T, Ozeki K, et al. Prospective comparison of preference and efficacy of adalimumab and infliximab for treating ulcerative colitis naive to anti-tumor necrosis factor therapy. Medicine. 2017;96:e7800.

    Article  CAS  PubMed  PubMed Central  Google Scholar 

  112. Inoue N, Kobayashi K, Naganuma M, Hirai F, Ozawa M, Arikan D, et al. Long-term safety and efficacy of adalimumab for intestinal Behcet’s disease in the open-label study following a phase 3 clinical trial. Intest Res. 2017;15:395–401.

    Article  PubMed  PubMed Central  Google Scholar 

  113. Belzunegui J, Lopez L, Paniagua I, et al. Efficacy of infliximab and adalimumab in the treatment of a patient with severe neuro-Behçet’s disease. Clin Exp Rheumatol. 2008;26:133–4.

    Google Scholar 

  114. Bawazeer A, Raffa L, Nizamuddin S. Clinical experience with adalimumab in the treatment of ocular Behçet’s disease. Ocul Immunol Inflamm. 2010;18:226–32.

    Article  CAS  PubMed  Google Scholar 

  115. Olivieri I, D’Angelo S, Padula A, et al. Successful treatment of recalcitrant genital ulcers of Behçet’s disease with adalimumab after failure of infliximab and etanercept. Clin Exp Rheumatol. 2009;27:112.

    Google Scholar 

  116. Lee S, Lee S, Kim K, et al. Adalimumab treatment for life threatening pulmonary artery aneurysm in Behcet disease: a case report. Clin Rheumatol. 2010;29:91–3.

    Article  PubMed  Google Scholar 

  117. Fox JD, Baquerizo-Nole KL, Keegan BR, Macquhae F, Escandon J, Espinosa A, Kirsner RS. Adalimumab treatment leads to reduction of tissue tumor necrosis factor-alpha correlated with venous leg ulcer improvement: a pilot study. Int Wound J. 2015;13(5):963–6.

    Article  PubMed  PubMed Central  Google Scholar 

  118. Interlandi E, Leccese P, Olivieri I, Latanza L. Adalimumab for treatment of severe Behçet’s uveitis: a retrospective long-term follow-up study. Clin Exp Rheumatol. 2014;32:S58–62.

    PubMed  Google Scholar 

  119. Nanthapisal S, Klein NJ, Ambrose N, Eleftheriou D, Brogan PA. Paediatric Behçet’s disease: a UK tertiary centre experience. Clin Rheumatol. 2016;35:2509–16.

    Article  PubMed  PubMed Central  Google Scholar 

  120. Lim KI, Yang DH, Ryoo E. Behçet’s disease with multiple splenic abscesses in a child. Intest Res. 2017;15:422–8.

    Article  PubMed  PubMed Central  Google Scholar 

  121. Atienza-Mateo B, Calvo-Río V, Beltrán E, et al. Anti-interleukin 6 receptor tocilizumab in refractory uveitis associated with Behçet’s disease: multicentre retrospective study. Rheumatology (Oxford). 2018;57:856–64.

    Article  CAS  Google Scholar 

  122. Robinson AB, Gallentine WB, Rabinovich CE. Pediatric neuro-Behçet’s disease responsive to adalimumab. Pediatr Neurol. 2010;43(4):291–3.

    Article  PubMed  Google Scholar 

  123. Marsili M, Marzetti V, Lucantoni M, Lapergola G, Gattorno M, Chiarelli F, Breda L. Autoimmune sensorineural hearing loss as presenting manifestation of paediatric Behçet disease responding to adalimumab: a case report. Ital J Pediatr. 2016;42:81.

    Article  PubMed  PubMed Central  Google Scholar 

  124. Ozguler Y, Leccese P, Christensen R, Esatoglu SN, Bang D, Bodaghi B, Celik AF, Fortune F, Gaudric J, Gul A, Kotter I, Mahr A, Moots RJ, Richter J, Saadoun D, Salvarani C, Scuderi F, Sfikakis PP, Siva A, Stanford M, Tugal-Tutkun I, West R, Yurdakul S, Olivieri I, Yazici H, Hatemi G. Management of major organ involvement of Behcet’s syndrome: a systematic review for update of the EULAR recommendations. Rheumatology (Oxford). 2018 Dec 1;57(12):2200–12.

    Article  Google Scholar 

  125. Botsios C, Sfriso P, Furlan A, et al. Resistant Behçet disease responsive to anakinra. Ann Intern Med. 2008;149:284–6.

    Article  PubMed  Google Scholar 

  126. Cantarini L, Vitale A, Scalini P, Dinarello CA, Rigante D, Franceschini R, et al. Anakinra treatment in drug-resistant Behcet’s disease: a case series. Clin Rheumatol. 2015;34:1293–301.

    Article  PubMed  Google Scholar 

  127. Cantarini L, Talarico R, Generali E, et al. Safety profile of biologic agents for Behçet’s disease in a multicenter observational cohort study. Int J Rheum Dis. 2017;20(1):103–8.

    Article  CAS  PubMed  Google Scholar 

  128. Emmi G, Talarico R, Lopalco G, Cimaz R, Cantini F, Viapiana O, et al. Efficacy and safety profile of anti-interleukin-1 treatment in Behcet’s disease: a multicenter retrospective study. Clin Rheumatol. 2016;35(5):1281–6.

    Article  PubMed  Google Scholar 

  129. Grayson PC, Yazici Y, Merideth M, Sen HN, Davis M, Novakovich E, et al. Treatment of mucocutaneous manifestations in Behcet’s disease with anakinra: a pilot open-label study. Arthritis Res Ther. 2017;19(1):69.

    Article  PubMed  PubMed Central  CAS  Google Scholar 

  130. Fabiani C, Vitale A, Emmi G, Lopalco G, Vannozzi L, Guer-riero S, Gentileschi S, Bacherini D, Franceschini R, Frediani B, Galeazzi M, Iannone F, Tosi GM, Cantarini L. Interleukin (IL)-1 inhibition with anakinra and canakinumab in Behcet’s disease-related uveitis: a multicenter retrospective observational study. Ocul Immunol Inflamm. 2020;28(2):298–304.

    Article  CAS  PubMed  Google Scholar 

  131. Fabiani C, Vitale A, Rigante D, Emmi G, Lopalco G, Sota J, Vannozzi L, di Scala G, Guerriero S, Orlando I, Franceschini R. The presence of uveitis is associated with a sustained response to the interleukin (IL)-1 inhibitors anakinra and canakinumab in Behçet’s disease. Ocul Immunol Inflamm. 2018;28:1–7. https://doi.org/10.1080/09273948.2018.1511810.

    Article  CAS  Google Scholar 

  132. Caso F, Rigante D, Vitale A, et al. Efficacy of anakinra in refractory Behçet’s disease sacroiliitis. Clin Exp Rheumatol. 2014;32:S171.

    PubMed  Google Scholar 

  133. Vitale A, Rigante D, Caso F, et al. Interleukin-1 blockade in neuro-Behçet’s disease: a case-based reflection. Int J Rheum Dis. 2017;20(8):1046–9.

    Article  PubMed  Google Scholar 

  134. Ugurlu S, Ucar D, Seyahi E, Hatemi G, Yurdakul S. Canakinumab in a patient with juvenile Behcet’s syndrome with refractory eye disease. Ann Rheum Dis. 2012;71:1589–91.

    Article  PubMed  Google Scholar 

  135. Emmi G, Silvestri E, Ciucciarelli L, Squatrito D, Emmi L. Reply: anti-IL1 blocking agents in drug-resistant Behçet’s syndrome: our little case series. Clin Exp Rheumatol. 2014;32:S172.

    PubMed  Google Scholar 

  136. Vitale A, Rigante D, Caso F, et al. Inhibition of interleukin-1by canakinumab as a successful mono-drug strategy for the treatment of refractory Behçet’s disease: a case series. Dermatology. 2014;228:211–4.

    Article  PubMed  Google Scholar 

  137. Pagnini I, Bondi T, Simonini G, Giani T, Marino A, Cimaz R. Successful treatment with canakinumab of a paediatric patient with resistant Behçet’s disease. Rheumatology (Oxford). 2015;54:1327–8.

    Article  Google Scholar 

  138. Emmi G, Talarico R, Lopalco G, et al. Efficacy and safety profile of anti- interleukin-1 treatment in Behçet’s disease: a multicenter retrospective study. Clin Rheumatol. 2016;35(5):1281–6.

    Article  PubMed  Google Scholar 

  139. Orlando I, Vitale A, Rigante D, et al. Long-term efficacy and safety of the interleukin-1 inhibitors anakinra and canakinumab in refractory Behçet disease uveitis and concomitant bladder papillary carcinoma. Intern Med J. 2017;47:1086–8.

    Article  CAS  PubMed  Google Scholar 

  140. Tanaka T, Narazaki M, Ogata A, Kishimoto T. A new era for the treatment of inflammatory autoimmune diseases by interleukin-6 blockade strategy. Semin Immunol. 2014;26:88–96.

    Article  CAS  PubMed  Google Scholar 

  141. Hirano T, Ohguro N, Hohki S, Hagihara K, Shima Y, Narazaki M, et al. A case of Behçet’s disease treated with a humanized anti-interleukin-6 receptor antibody, tocilizumab. Mod Rheumatol. 2012;22:298–302.

    Article  PubMed  Google Scholar 

  142. Calvo-Rio V, de la Hera D, Beltran-Catalan E, et al. Tocilizumab in uveitis refractory to other biologic drugs: a study of 3 cases and a literature review. Clin Exp Rheumatol. 2014;32(4 Suppl 84):S54–7.

    PubMed  Google Scholar 

  143. Papo M, Bielefeld P, Vallet H, et al. Tocilizumab in severe and refractory non-infectious uveitis. Clin Exp Rheumatol. 2014;32:S75–9.

    PubMed  Google Scholar 

  144. Deroux A, Chiquet C, Bouillet L. Tocilizumab in severe and refractory Behçet’s disease: four cases and literature review. Semin Arthritis Rheum. 2016;45:733–7.

    Article  CAS  PubMed  Google Scholar 

  145. Alokaily F, Al Saati A, Jawad A. Successful treatment of Behçet’s uveitis with Tocilizumab. Saudi J Ophthalmol. 2017;31(1):42–4.

    Article  CAS  PubMed  Google Scholar 

  146. Ozturk HE, Oray M, Tugal-Tutkun I. Tocilizumab for the treatment of Behçet uveitis that failed interferon alpha and anti-tumor necrosis factor alpha therapy. Ocul Immunol Inflamm. 2018;26:1005–14.

    Article  CAS  Google Scholar 

  147. Atienza-Mateo B, Calvo-Río V, Beltrán E, Martínez-Costa L, Valls-Pascual E, Hernández-Garfella M, et al. Anti-interleukin 6 receptor tocilizumab in refractory uveitis associated with Behçet’s disease: multicentre retrospective study. Rheumatology. 2018;57:856–64.

    Article  CAS  PubMed  Google Scholar 

  148. Diamantopoulos AP, Hatemi G. Lack of efficacy of tocilizumab in mucocutaneous Behçet’s syndrome: report of two cases. Rheumatology (Oxford). 2013;52:1923–4.

    Article  Google Scholar 

  149. Cantarini L, Lopalco G, Vitale A, et al. Paradoxical mucocutaneous flare in a case of Behçet’s disease treated with tocilizumab. Clin Rheumatol. 2015;34:1141–3.

    Article  PubMed  Google Scholar 

  150. Emmi G, Silvestri E, Squatrito D, Emmi L, Cantarini L, Prisco D. Tocilizumab-induced exacerbation of mucosal ulcers in a patient with multi-refractory Behçet’s disease. Semin Arthritis Rheum. 2016;46(1):e1–2.

    Article  PubMed  Google Scholar 

  151. Ding Y, Li C, Liu J, et al. Tocilizumab in the treatment of severe and/or refractory vasculo-Behçet’s disease: a single-centre experience in China. Rheumatology (Oxford). 2018;57:2057–9.

    Google Scholar 

  152. Hirohata S, et al. Cerebrospinal fluid interleukin-6 in progressive Neuro-Behçet’s syndrome. Clin Immunol Immunopathol. 1997;82:12–7.

    Article  CAS  PubMed  Google Scholar 

  153. Akman-Demir G, et al. Interleukin-6 in neuro-Behçet’s disease: association with disease subsets and long-term outcome. Cytokine. 2008;44:373–6.

    Article  CAS  PubMed  Google Scholar 

  154. Urbaniak P, Hasler P, Kretzschmar S. Refractory neuro-Behçet treated by tocilizumab: a case report. Clin Exp Rheumatol. 2012;30(3 Suppl 72):S73–5.

    CAS  PubMed  Google Scholar 

  155. Shapiro LS, Farrell J, Borhani Haghighi A. Tocilizumab treatment for neuro-Behçet’s disease, the first report. Clin Neurol Neurosurg. 2012;114:297–8.

    Article  PubMed  Google Scholar 

  156. Addimanda O, Pipitone N, Pazzola G, Salvarani C. Tocilizumab for severe refractory neuro-Behçet: three cases IL-6 blockade in neuro-Behçet. Semin Arthritis Rheum. 2015;44:472–5.

    Article  CAS  PubMed  Google Scholar 

  157. Hatemi G, Seyahi E, Fresko I, Talarico R, Hamuryudan V. One year in review 2019: Behçet’s syndrome. Clin Exp Rheumatol. 2019;37 Suppl 121(6):3–17.

    PubMed  Google Scholar 

  158. Alpsoy E, Durusoy C, Yilmaz E, Ozgurel Y, Ermis O, Yazar S, et al. Interferon alfa-2a in the treatment of Behçet disease: a randomized placebo-controlled and double-blind study. Arch Dermatol. 2002;138:467–71.

    Article  CAS  PubMed  Google Scholar 

  159. Azizlerli G, Sarica R, Köse A, Ovül C, Kavala M, Kayabali M, et al. Interferon alfa-2a in the treatment of Behçet’s disease. Dermatology. 1996;192:239–41.

    Article  CAS  PubMed  Google Scholar 

  160. Tsambaos D, Eichelberg D, Goos M. Behçet’s syndrome: treatment with recombinant leukocyte alpha-interferon. Arch Dermatol Res. 1986;278(4):335–6.

    Article  CAS  PubMed  Google Scholar 

  161. Monastirli A, Chroni E, Georgiou S, Ellul J, Pasmatzi E, Papathanasopoulos P, Tsambaos D. Interferon-alpha treatment for acute myelitis and intestinal involvement in severe Behcet’s disease. QJM. 2010;103(10):787–90.

    Article  CAS  PubMed  Google Scholar 

  162. Yalçındag N, Köse HC. Comparison of the treatment results for Behçet uveitis in patients treated with infliximab and interferon. Ocul Immunol Inflamm. 2019;3:1–10.

    CAS  Google Scholar 

  163. Nichols JC, Ince A, Akduman L, Mann ES. Interferon-alpha 2a treatment of neuro-Behçet disease. J Neuroophthalmol. 2001;21(2):109–11.

    Article  CAS  PubMed  Google Scholar 

  164. Chroni E, Tsambaos D. Interferon-alpha for the treatment of severe entero- and neuro-Behçet’s disease. J Dermatol. 2015;42(2):229–30.

    Article  CAS  PubMed  Google Scholar 

  165. Calguneri M, Onat AM, Ozturk MA, Ozcakar L, Ureten K, Akdogan A, Ertenli I, Kiraz S. Transverse myelitis in a patient with Behcet’s disease: favorable outcome with a combination of interferon-alpha. Clin Rheumatol. 2005;24(1):64–6.

    Article  PubMed  Google Scholar 

  166. Calguneri M, Ozturk MA, Ertenli I, Kiraz S, Apras S, Ozbalkan Z. Effects of interferon-alpha treatment on the clinical course of refractory Behcet’s disease: an open study. Ann Rheum Dis. 2003;62(5):492–3.

    Article  CAS  PubMed  PubMed Central  Google Scholar 

  167. Shi J, Zhao C, Zhou J, et al. Effectiveness and safety of interferon α2a as an add-on treatment for refractory Behçet’s uveitis. Ther Adv Chronic Dis. 2019;10:1–9.

    Article  Google Scholar 

  168. Yang DS, Taylor SR, Lightman SL. Interferon-alpha in the Management of Patients with Behçet’s disease. Br J Hosp Med (Lond). 2005;69(2008):575–9.

    Google Scholar 

  169. Imrie FR, Dick AD. Biologics in the treatment of uveitis. Curr Opin Ophthalmol. 2007;18(6):481–6.

    Article  PubMed  Google Scholar 

  170. Hensel M, Breitbart A, Ho AD. Autologous hematopoietic stem-cell transplantation for Behcet’s disease with pulmonary involvement. N Engl J Med. 2001;344:69.

    Article  CAS  PubMed  Google Scholar 

  171. Marmont AM, Gualandi F, Van Lint MT, et al. Autologous hematopoietic stem cell transplant for refractory Behçet’s disease. Paper presented at Bone Marrow Transplant. Oral presentation number O157, 2003.

    Google Scholar 

  172. Marmont AM, Gualandi F, Piaggio G, et al. Allogeneic bone marrow transplantation (BMT) for refractory Behçet’s diseasee with severe CNS involvement. Bone Marrow Transplant. 2006;37:1061–3.

    Article  CAS  PubMed  Google Scholar 

  173. Yamato K. Successful cord blood stem cell transplantation for myelodysplastic syndrome with Behcet disease. Int J Hematol. 2003;77:82–5.

    Article  PubMed  Google Scholar 

  174. Rossi G, Moretta A, Locatelli F. Autologous hematopoietic stem cell transplantation for severe/refractory intestinal Behcet disease. Blood. 2004;103:748–50.

    Article  CAS  PubMed  Google Scholar 

  175. Tomonari A, Tojo A, Takahashi T, et al. Resolution of Behçet’s disease after HLA-mismatched unrelated cord blood transplantation for myelodysplastic syndrome. Ann Hematol. 2004;83:464–6.

    Article  PubMed  Google Scholar 

  176. Maurer B, Hensel M, Max R, et al. Autologous hematopoietic stem cell transplantation for Behçet’s disease with pulmonary involvement: analysis after 5 years of follow up. Ann Rheum Dis. 2006;65:127–9.

    Article  CAS  PubMed  Google Scholar 

  177. Daikeler T, Hugle T, Farge D, et al. Allogeneic hematopoietic SCT for patients with autoimmune diseases. Bone Marrow Transplant. 2009;44:27–33.

    Article  CAS  PubMed  Google Scholar 

  178. De Cata A, Intiso D, Bernal M, et al. Prolonged remission of neuro-Behcet disease following autologous transplantation. Int J Immunopathol Pharmacol. 2007;20:91–6.

    Article  PubMed  Google Scholar 

  179. Nonami A, Takenaka K, Sumida C, et al. Successful treatment of myelodysplastic syndrome (MDS)-related intestinal Behcet’s disease by up-front cord blood transplantation. Intern Med. 2007;46:1753–6.

    Article  PubMed  Google Scholar 

  180. Chauhan S, Olujohungbe A, Moots RJ. Treatment of Behcet’s syndrome and plasmacytoma in a patient with peripheral blood stem cell transplantation. Leuk Lymphoma. 2008;49:2377–9.

    Article  PubMed  Google Scholar 

  181. Statkute L, Oyama Y, Barr WG, et al. Autologous non-myeloablative hematopoietic stem cell transplantation for refractory systemic vasculitis. Ann Rheum Dis. 2008;67:991–7.

    Article  CAS  PubMed  Google Scholar 

  182. Daikeler T, Kotter I, Tyndall CB, Attarbaschi A, Apperley J, Guardiola P, et al. Haematopoietic stem cell transplantation for vasculitis including Behcet’s disease and polychondritis – a retrospective analysis of patients recorded to the European Bone Marrow Transplantation (EBMT) and European League Against Rheumatism (EULAR) databases and a review of the literature. Ann Rheum Dis. 2007;66:202–7.

    Article  PubMed  Google Scholar 

  183. Lim SH, Hulsey M, Esler WV. Resolution of Behcet’s disease after non-myeloablative allogeneic stem cell transplant for acute myeloid leukemia. Rheumatology. 2009;48:88–9.

    Article  CAS  PubMed  Google Scholar 

  184. Gooley TA, Chien JW, Pergam SA, et al. Reduced mortality after allogeneic hematopoietic-cell transplantation. N Engl J Med. 2010;363:2091–101.

    Article  CAS  PubMed  PubMed Central  Google Scholar 

  185. Soysal T, Salihoğlu A, Esatoğlu SN, Gültürk E, Eşkazan AE, Hatemi G, et al. Bonemarrow transplantation for Behçet’s disease: a case report and systematic review of the literature. Rheumatology. 2014;53:1136–41.

    Article  CAS  PubMed  Google Scholar 

  186. Lee WS, Kang MH, Hwang JH, Oh YJ, Yoo WH. Allogeneic hematopoietic stem cell transplantation for refractory Behcet’s disease with myelodysplastic syndrome: a case report. Int J Rheum Dis. 2017;20(11):1860–1.

    Article  PubMed  Google Scholar 

  187. Kürtüncü M, Aydın BN, Gündüz T, Ala S, Akman G, World Congress of Neurology Kyoto, WCN17–2801, 2017.

    Google Scholar 

Download references

Author information

Authors and Affiliations

Authors

Editor information

Editors and Affiliations

Rights and permissions

Reprints and permissions

Copyright information

© 2021 Springer Nature Switzerland AG

About this chapter

Check for updates. Verify currency and authenticity via CrossMark

Cite this chapter

Kürtüncü, M., Altunrende, B., Akman, G. (2021). Treatment of Neuro-Behçet’s Disease. In: Tüzün, E., Kürtüncü, M. (eds) Neuro-Behçet’s Disease. Springer, Cham. https://doi.org/10.1007/978-3-030-55273-2_12

Download citation

Publish with us

Policies and ethics