Department of Neurology, Istanbul Faculty of Medicine, Istanbul University, Turkey.
Clin Exp Rheumatol. 2011 Jul-Aug;29(4 Suppl 67):S64-7. Epub 2011 Sep 27.
Behçet's disease is a multisystemic, relapsing, inflammatory disorder of unknown origin. Among Turkish cohorts, 5-15% of patients show involvement of the central nervous system (CNS) at some time during their disease. There are mainly two types of clinical presentation: parenchymal CNS inflammation manifesting mainly as meningoencephalitis of the brainstem, or dural sinus thrombosis. Several drugs like high-dose steroids or immunosuppressive agents, mainly azathioprine, are used in the treatment. For patients who do not respond sufficiently to these agents or are not able tolerate them, other options are needed.
We are presenting 4 cases with parenchymal neuro-Behçet's disease, where commonly used immunosuppressive drugs could not be continued due to intolerance or inefficacy. However, the patients benefited well from mycophenolate mofetil. The benefit was sustained during 3-7 years of follow-up (median 6.5 years).
Mycophenolate mofetil seems to be an alternative drug in parenchymal neuro-Behçet's disease; however, large controlled studies should be performed for verification of our results.
白塞病是一种多系统、反复发作、病因不明的炎症性疾病。在土耳其队列中,5-15%的患者在疾病的某个阶段会出现中枢神经系统(CNS)受累。主要有两种临床表现类型:实质 CNS 炎症,主要表现为脑干脑膜脑炎,或硬脑膜窦血栓形成。治疗中主要使用高剂量类固醇或免疫抑制剂,主要是硫唑嘌呤。对于对这些药物反应不佳或不能耐受的患者,需要其他选择。
我们报告了 4 例实质神经白塞病患者,由于不耐受或无效,不能继续使用常用的免疫抑制剂。然而,这些患者从吗替麦考酚酯中获益良好。在 3-7 年的随访中(中位数 6.5 年),疗效持续。
吗替麦考酚酯似乎是实质神经白塞病的一种替代药物;然而,需要进行大规模的对照研究来验证我们的结果。