Nöro-Behçet Hastalığı Güncel Tedavi Yaklaşımları

Özet

Bu bölümde Behçet hastalığının nörolojik tutulumu (Nöro-Behçet hastalığı) ve güncel tedavi yaklaşımları kapsamlı biçimde ele alınmaktadır. Behçet hastalığı, özellikle İpek Yolu coğrafyasında sık görülen, çok sistemli kronik inflamatuar bir hastalık olup nörolojik tutulum en ciddi komplikasyonları arasında yer alır. Nöro-Behçet hastalığı parenkimal ve non-parenkimal formlar şeklinde sınıflandırılmakta; klinik bulgular baş ağrısından fokal nörolojik defisitlere, serebral venöz trombozdan kronik progresif ensefalopatiye kadar geniş bir spektrum göstermektedir. Bölümde tanı kriterleri, ayırıcı tanı, manyetik rezonans görüntüleme ve beyin omurilik sıvısı bulgularının önemi ayrıntılı olarak tartışılmaktadır. Tedavi yaklaşımları EULAR ve İngiliz Romatoloji Derneği kılavuzları temel alınarak sunulmuş; kortikosteroidler, klasik immünsupresif ajanlar, anti-TNF tedaviler, interlökin-6 inhibitörleri ve JAK inhibitörleri gibi biyolojik ve hedefe yönelik tedaviler güncel kanıtlar ışığında değerlendirilmiştir. Ayrıca akut ve kronik progresif Nöro-Behçet için pratik tedavi algoritmaları ve izlem stratejileri paylaşılmıştır.

Referanslar

Direskeneli H. Behçet's disease: infectious aetiology, new autoantigens, and HLA-B51. Ann Rheum Dis. 2001; 60(11):996-1002. doi: 10.1136/ard.60.11.996.

Yazici H, Fresko I, Yurdakul S. Behçet's syndrome: disease manifestations, management, and advances in treatment. Nat Clin Pract Rheumatol. 2007; 3(3):148-55. doi: 10.1038/ncprheum0436.

Yurdakul S, Hamuryudan V, Yazici H. Behçet syndrome. Curr Opin Rheumatol. 2004;16(1):38-42. doi: 10.1097/00002281-200401000-00008.

Mahr A, Belarbi L, Wechsler B et al. Population‐based prevalence study of Behcet's disease: differences by ethnic origin and low variation by age at immigration. Arthritis Rheum. 2008;58(12):3951-9. doi: 10.1002/art.24149.

Salvarani C, Pipitone N, Catanoso MG et al. Epidemiology and clinical course of Behçet's disease in the Reggio Emilia area of Northern Italy: A seventeen‐year population‐based study. Arthritis Rheum. 2007;57(1):171-8. doi: 10.1002/art.22500.

Koné-Paut I, Geisler I, Wechsler B et al. Familial aggregation in Behçet’s disease: high frequency in siblings and parents of pediatric probands. J Pediatr. 1999;135(1):89-93. doi: 10.1016/s0022-3476(99)70333-1.

Nussenblatt RB. Uveitis in Behçet's disease. Int Rev Immunol. 1997;14(1):67-79. doi: 10.3109/08830189709116845.

Siva A, Saip S. The spectrum of nervous system involvement in Behçet’s syndrome and its differential diagnosis. J Neurol. 2009;256(4):513-29. doi: 10.1007/s00415-009-0145-6.

Al-Araji A, Kidd DP. Neuro-Behçet's disease: epidemiology, clinical characteristics, and management. Lancet Neurol. 2009;8(2):192-204. doi: 10.1016/S1474-4422(09)70015-8.

Ozcifci G, Aydin T, Atli Z et al. The incidence, clinical characteristics, and outcome of COVID-19 in a prospectively followed cohort of patients with Behçet’s syndrome. Rheumatol In. 2022;42(1):101-113. doi: 10.1007/s00296-021-05056-2

Akman-Demir G, Serdaroglu P, Tasçi B et al. Clinical patterns of neurological involvement in Behçet's disease: evaluation of 200 patients. Brain. 1999;122 ( Pt 11):2171-82. doi: 10.1093/brain/122.11.2171.

Uluduz D, Kürtüncü M, Yapıcı Z et al. Clinical characteristics of pediatric-onset neuro-Behçet disease. Neurology. 2011;77(21):1900-5. doi: 10.1212/WNL.0b013e318238edeb.

Kalra S, Silman A, Akman-Demir G et al. Diagnosis and management of Neuro-Behçet’s disease: international consensus recommendations. J Neurol. 2014;261(9):1662-76. doi: 10.1007/s00415-013-7209-3.

International Team for the Revision of the International Criteria for Behçet's Disease (ITR‐ICBD), Davatchi F, Assaad‐Khalil S et al. 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(3):338-47. doi: 10.1111/jdv.12107.

Ishido M, Horita N, Takeuchi M et al. Distinct clinical features between acute and chronic progressive parenchymal neuro-Behçet disease: meta-analysis. Sci Rep. 2017;7(1):10196. doi: 10.1038/s41598-017-09938-z.

Shi J, Huang X, Li G et al. Cerebral venous sinus thrombosis in Behçet’s disease: a retrospective case-control study. Clin Rheumatol. 2018;37(1):51-57. doi: 10.1007/s10067-017-3718-2.

Leccese P, Ozguler Y, Christensen R et al. Management of skin, mucosa and joint involvement of Behçet’s syndrome: a systematic review for update of the EULAR recommendations for the management of Behçet’s syndrome. Semin Arthritis Rheum. 2019;48(4):752-762. doi: 10.1016/j.semarthrit.2018.05.008

Murphy R, Moots RJ, Brogan P et al. British Association of Dermatologists and British Society for Rheumatology living guideline for managing people with Behçets 2024. Br J Dermatol. 2024;191(5):e8-e25. doi: 10.1093/bjd/ljae263.

Gaudric J, Jayet J, Saadoun D et al. Factors influencing the recurrence of arterial involvement after surgical repair in Behçet disease. J Vasc Surg. 2020;72(5):1761-1769. doi: 10.1016/j.jvs.2020.01.076

Wang Z, Wang X, Liu W et al. Baricitinib for the treatment of refractory vascular Behçet's disease. Clin Immunol. 2023; 250:109298. doi: 10.1016/j.clim.2023.109298.

Hatemi G, Tukek NB, Esatoglu SN et al. Infliximab for vascular involvement in Behçet's syndrome. Clin Immunol. 2023; 253:109682. doi: 10.1016/j.clim.2023.109682

Khitri MY, Bartoli A, Maalouf G et al. Tocilizumab in Behçet disease: a multicenter study of 30 patients. J Rheumatol. 2023;50(7):916-923. doi: 10.3899/jrheum.221106.

23-Mease PJ, Hatemi G, Paris M et al. Apremilast long-term safety up to 5 years from 15 pooled randomized, placebo-controlled studies of psoriasis, psoriatic arthritis, and behçet’s syndrome. Am J Clin Dermatol. 2023;24(5):809-820. doi: 10.1007/s40257-023-00783-7.

Peñuelas Leal R, Labrandero Hoyos C, Peñuelas Ruiz JA et al. Treatment of Behçet disease with oral roflumilast, an observational study. Clin Exp Dermatol. 2024;50(1):62-68. doi: 10.1093/ced/llae284.

Sulu B, Hatemi G. New and future perspectives in Behçet's syndrome. Arch Rheumatol. 2024;39(4):511-521. doi: 10.46497/ArchRheumatol.2024.11049.

Yalcin Kehribar D, Gunaydin S, Ozgen M. Infliximab therapy in parenchymal neuro‐Behçet’s disease: A single‐center experience. Int J Rheum Dis. 2021;24(10):1302-1307. doi: 10.1111/1756-185X.14209.

Mohammed RHA, Woldeamanuel YW. The effectiveness of the anti-tumor necrosis factor therapy infliximab in neuro-Behçet’s disease: a systematic review and meta-analysis. J Int Med Res. 2023;51(5):3000605231169895. doi: 10.1177/03000605231169895.

Zhong Z, Deng D, Gao Y et al. Combinations of immunomodulatory agents for prevention of uveitis relapse in patients with severe Behçet's disease already on corticosteroid therapy: a randomised, open-label, head-to-head trial. Lancet Rheumatol. 2024;6(11):e780-e790. doi: 10.1016/S2665-9913(24)00194-2.

Esatoglu SN, Sonmez O. Ucar D et al. De novo manifestations during adalimumab treatment in Behçet’s syndrome. Rheumatology (Oxford). 2025;64(4):2034-2040. doi: 10.1093/rheumatology/keae416.

Kötter I, Günaydin I, Zierhut M et al. The use of interferon α in Behçet disease: review of the literature. Semin Arthritis Rheum. 2004;33(5):320-35. doi: 10.1016/j.semarthrit.2003.09.010.

Alpsoy E, Durusoy C, Yilmaz E et al. Interferon alfa-2a in the treatment of Behçet disease: a randomized placebo-controlled and double-blind study. Arch Dermatol. 2002;138(4):467-71. doi: 10.1001/archderm.138.4.467.

Davatchi F, Sadeghi Abdollahi B, Shams H et al. Combination of pulse cyclophosphamide and azathioprine in ocular manifestations of Behcet's disease: longitudinal study of up to 10 years. Int J Rheum Dis. 2014;17(4):444-52. doi: 10.1111/1756-185X.12248.

Alpsoy E, Leccese P, Emmi G et al. Treatment of Behçet's disease: an algorithmic multidisciplinary approach. Front Med (Lausanne). 2021; 28:8:624795. doi: 10.3389/fmed.2021.624795.

Liu J, Yan D, Wang Z et al. Tocilizumab in the treatment of severe and refractory parenchymal neuro-Behçet’s syndrome: case series and literature review. Ther Adv Musculoskelet Dis. 2020; 12:1759720X20971908. doi: 10.1177/1759720X20971908.

Ozdede A, Esatoglu SN, Durmaz ES et al. Tocilizumab may not be a good option for vascular involvement due to Behçet's syndrome. Clin Exp Rheumatol. 2024; 42(10):2057-2064. doi: 10.55563/clinexprheumatol/3myixe.

Zou J, Cai JF, Ye JF et al. Tofacitinib as an alternative therapy for refractory intestinal Behçet’s syndrome. Ther Adv Musculoskelet Dis. 2022;14:1759720X221124014. doi: 10.1177/1759720X221124014. eCollection 2022.

Tao T, He D, Peng X et al. Successful remission with upadacitinib in two patients with anti-TNF-refractory macular edema associated with Behçet’s uveitis. Ocul Immunol Inflamm. 2024;32(8):1897-1900. doi: 10.1080/09273948.2023.2263557.

Davatchi F, Shams H, Rezaipoor M et al. Rituximab in intractable ocular lesions of Behcet’s disease; randomized single‐blind control study (pilot study). Int J Rheum Dis. 2010;13(3):246-52. doi: 10.1111/j.1756-185X.2010.01546.x

Cantarini L, Vitale A, Scalini P et al. Anakinra treatment in drug-resistant Behcet’s disease: a case series. Clin Rheumatol. 2015;34(7):1293-301. doi: 10.1007/s10067-013-2443-8.

Mohammad AJ, Smith RM, Chow YW et al. Alemtuzumab as remission induction therapy in Behçet disease: a 20-year experience. J Rheumatol. 2015;42(10):1906-13. doi: 10.3899/jrheum.141344.

Puyade M, Patel A, Lim YJ et al. Autologous hematopoietic stem cell transplantation for behcet’s disease: a retrospective survey of patients treated in europe, on behalf of the Autoimmune Diseases Working Party of the European Society for Blood and Marrow Transplantation. Front Immunol. 2021;12:638709. doi: 10.3389/fimmu.2021.638709.

Seider N, Beiran I, Scharf J et al. Intravenous immunoglobulin therapy for resistant ocular Behcet's disease. Br J Ophthalmol. 2001;85(11):1287-8. doi: 10.1136/bjo.85.11.1287.

Zhao C, Li C, Duan FJ et al. Case Report: Repeated Low-Dose Rituximab Treatment Is Effective in Relapsing Neuro Behçet's Disease. Front Neurol. 2021;12:595984. doi: 10.3389/fneur.2021.595984.

Hirohata S, Kikuchi H, Sawada T et al. Recommendations for the management of neuro-Behçet's disease by the Japanese National Research Committee for Behçet's Disease. Intern Med. 2020;59(19):2359-2367. doi: 10.2169/internalmedicine.4705-20.

Gelecek

21 Ocak 2026

Lisans

Lisans