Haas Johannes-Peter, Metzler Markus, Ruder Hans, Waldherr Rüdiger, Böswald Michael, Rupprecht Thomas
Department of Pediatrics, Friedrich-Alexander University, Erlangen, Germany.
Pediatr Neurol. 2002 Jul;27(1):71-4. doi: 10.1016/s0887-8994(02)00392-2.
We report a female who was diagnosed with Wegener's granulomatosis at 4 years of age with life-threatening intracranial bleeding. The patient's serum was positive for c-antineutrophilic cytoplasmic antibodies, and histologic analysis of the lung biopsy revealed evidence of granulomatous vasculitis. Initial treatment with steroids and cyclophosphamide was successfully converted to a long-term medication regimen consisting of azathioprine, trimethoprim, and sulfamethoxazole. Thereafter the patient showed no signs of disease activity for more than 3 years and manifested only a low-grade neurologic handicap. In February 2001, 5 years after the initial diagnosis, she presented with altered consciousness and myoclonic multifocal seizures. Subsequent diagnostic studies confirmed the diagnosis of disseminated cerebral vasculitis unresponsive to steroid treatment. Acute neurologic symptoms relented immediately after cyclophosphamide pulse therapy. Magnetic resonance imaging of the brain demonstrated complete remission within 8 weeks. Her current treatment includes steroids and monthly pulses of cyclophosphamide.
我们报告了一名4岁时被诊断为韦格纳肉芽肿病并伴有危及生命的颅内出血的女性患者。患者血清抗中性粒细胞胞浆抗体呈阳性，肺活检的组织学分析显示有肉芽肿性血管炎的证据。最初使用类固醇和环磷酰胺治疗成功转换为长期用药方案，包括硫唑嘌呤、甲氧苄啶和磺胺甲恶唑。此后，患者超过3年没有疾病活动迹象，仅表现出轻度神经功能障碍。在初次诊断5年后的2001年2月，她出现意识改变和肌阵挛性多灶性癫痫发作。随后的诊断研究证实为对类固醇治疗无反应的播散性脑血管炎。环磷酰胺脉冲治疗后急性神经症状立即缓解。脑部磁共振成像显示8周内完全缓解。她目前的治疗包括类固醇和每月一次的环磷酰胺脉冲治疗。
