Cerebral vasculitis in a patient with rheumatoid arthritis
Cerebral vasculitis in a patient with rheumatoid arthritis
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DOI：
10.1016/j.jbspin.2006.08.004
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发表时间：
2007-03-01
期刊：
影响因子：
4.2
通讯作者：
Sellami, Slaheddine
中科院分区：
文献类型：
--
作者：
Mrabet, Dalila;Meddeb, Nihel;Sellami, Slaheddine
中枢神经系统炎性血管炎在类风湿关节炎患者中极为罕见。症状可能会误导。大多数报告的病例发生在患有长期、结节性、破坏性、类风湿因子阳性疾病的男性中。严重的全身症状和突出的关节外表现的血管炎通常存在。我们报告一例脑血管炎的59岁妇女与20年的历史，破坏性类风湿因子阳性的RA是良好的控制甲氨蝶呤。对对症治疗无反应的头痛突然发生，并伴有步态障碍。脑部磁共振成像显示，双侧脑室周围皮质下分布有点状高信号区。磁共振血管造影显示右颈内动脉长而紧的狭窄和左颈内动脉串珠状狭窄提示血管炎。甲基强的松龙（1 g/d，持续3天）和环磷酰胺（1 g）每月一次的脉冲治疗确保了神经系统症状和炎症实验室证据的消退。5个月后末次随访时无复发证据。脑血管炎通常每月使用糖皮质激素和环磷酰胺推注治疗，并与持续糖皮质激素治疗分开，TNF α拮抗剂可能对常规治疗无效的患者有效。然而，必须排除其他血管炎，如巨细胞动脉炎和韦格纳肉芽肿病，因为它们对TNF α拮抗剂治疗是难治的。(C)2007年，Elsevier Masson SAS。All rights reserved.
Inflammatory vasculitis of the central nervous system is exceedingly rare in patients with rheumatoid arthritis (RA). The symptoms may be misleading. Most of the reported cases occurred in males with long-standing, nodular, destructive, rheumatoid factor-positive disease. Severe constitutional symptoms and prominent extraarticular manifestations of vasculitis were usually present. We report a case of cerebral vasculitis in a 59-year-old woman with a 20-year history of destructive rheumatoid factor-positive RA that was well controlled by methotrexate. Headache that was unresponsive to symptomatic treatment developed abruptly, together with gait disorders. Magnetic resonance imaging of the brain showed dot-like areas of high-signal in a periventricular subcortical distribution on both sides. Magnetic resonance angiography visualized a long tight stenosis of the fight internal carotid artery and a string-of-beads stenosis of the left internal carotid artery suggesting vasculitis. Pulse therapy with methylprednisolone (1 g/d for 3 days) and cyclophosphamide (1 g) once a month ensured resolution of the neurological symptoms and laboratory evidence of inflammation. There was no evidence of a relapse at last follow-up after 5 months. Cerebral vasculitis is usually treated with monthly glucocorticoid and cyclophosphamide boluses separated by continuous glucocorticoid therapy, TNF alpha antagonists may be effective in patients who fail to respond to conventional treatment. However, other vasculitides such as giant cell arteritis and Wegener's granulomatosis must be ruled out, as they are refractory to TNFa antagonist therapy. (C) 2007 Elsevier Masson SAS. All rights reserved.