Behçet's syndrome
Behçet's syndrome is characterized by recurrent oral and genital ulcers, along with various systemic manifestations. Neurological issues are rare but can affect the brain tissue or dural sinuses. Treatment depends on severity, with immunosuppressive drugs like azathioprine used for severe cases. Dural sinus thrombosis may require anticoagulation therapy.
Introduction to Behçet Syndrome
Behçet syndrome is characterized by recurrent oral aphthae and various systemic manifestations like genital aphthae, ocular disease, skin lesions, gastrointestinal involvement, neurological disease, vascular disease, or arthritis. Neurological problems occur in less than 10% of patients, manifesting either as parenchymal or non-parenchymal disease. The syndrome typically follows a relapsing and remitting course. (1, 2, 3)
Treatment of Parenchymal Behçet Syndrome
The goal is to promptly suppress inflammatory exacerbations and prevent irreversible organ damage. A coordinated multidisciplinary approach is necessary. Treatment choices depend on severity, glucocorticoid responsiveness, prior neurological disease, disease course, and other features of Behçet syndrome. For severe parenchymal disease, azathioprine (2 mg/kg/day), mycophenolate mofetil (500 mg twice daily), or methotrexate (5 - 15 mg once weekly) are used. High doses of glucocorticoids (methylprednisolone 1 g daily for 3 days, followed by prednisolone tapering) in combination with an immunosuppressive agent are indicated for life-threatening manifestations like focal parenchymal lesions, encephalitis, and medium-vessel vasculitis. (4, 5, 6)
Duration of Immunosuppressive Treatment
The optimal duration of treatment isn't clear. In severe cases, therapy usually continues for at least 18 months, during which glucocorticoids are tapered as tolerated. Refractory cases may benefit from TNF-alpha inhibitors like adalimumab (40 mg every other week) with or without azathioprine. (7, 8)
Treatment of Dural Sinus Thrombosis
The best approach for dural sinus thrombosis in Behçet syndrome is still uncertain. Treatment may include initial therapeutic LMWH (Innohep 175 IU/Kg) followed by long-term or possibly lifelong warfarin. (9)
References
1- Kidd D, Steuer A, Denman AM, Rudge P. Neurological complications of Behcet’s syndrome. Brain. 1999;122:2183–2194. doi: 10.1093/brain/122.11.2183.
2- Al-Fahad S, Al-Araji A. Neuro-Behcet’s disease in Iraq: a study of 40 patients. J Neurol Sci. 1999;170:105–111.
3- Al-Araji A, Kidd DP. Neuro-Behçet’s disease: epidemiology, clinical characteristics, and management. Lancet Neurol. 2009;8:192–204. doi: 10.1016/S1474-4422(09)70015-8.
4-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; 77:808.
5- azici H, Fresko I, Yurdakul S. Behcet’s syndrome: disease manifestations, management, and advances in treatment. Nat Clin Pract Rheumatol. 2007;3:148–155.
6- Hatemi G, Silman A, Bang D, et al. Management of Behcet’s disease: a systematic literature review for the EULAR evidence based recommendations for the management of Behcet’s disease. Ann Rheum Dis. 2008;67:1656–1662.
7- Kalra S, Silman A, Akman-Demir G, et al. Diagnosis and management of Neuro-Behçet's disease: international consensus recommendations. J Neurol 2014; 261:1662.
8- 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(4 Suppl 67):S54–S57.
9- Tayer-Shifman OE , Seyahi E, Nowatzky J, Ben-Chetrit E: Major vessel thrombosis in Behçet's disease: the dilemma of anticoagulant therapy - the approach of rheumatologists from different countries. Clin Exp Rheumatol. Sep-Oct 2012;30(5):735-40.
