Provost T T, Vasily D, Alexander E
Neurol Clin. 1987 Aug;5(3):405-26.
The studies recounted in this review have demonstrated that cutaneous vasculitis is a frequent extraglandular manifestation of primary Sjögren's syndrome. Two histopathologic types of vasculitis have been detected. One type, a leukocytoclastic angiitis, is found in association with high-titer anti-Ro(SS-A) antibodies, rheumatoid factor, hypergammaglobulinemia, and hypocomplementemia. The second type, a mononuclear inflammatory vasculopathy, in sharp contrast, is found in association with low-titer Ro(SS-A) antibodies, normocomplementemia, and absence of hypergammaglobulinemia and rheumatoid factor. Both types of vasculitis are found in association with peripheral nervous system and CNS disease. The peripheral nervous system and CNS disease involves the entire neuroaxis and preliminary data indicate that a vasculopathy is the cause of the peripheral nervous system and CNS disease. Evoked sensory response testing, CSF analysis, and MRI have proved to be very valuable techniques in investigating these patients with Sjögren's syndrome. Preliminary data suggest that high doses of prednisone or immunosuppressive agents are effective in treating these patients.
本综述中提及的研究表明，皮肤血管炎是原发性干燥综合征常见的腺外表现。已检测到两种组织病理学类型的血管炎。一种类型是白细胞破碎性血管炎，与高滴度抗Ro(SS - A)抗体、类风湿因子、高球蛋白血症和低补体血症相关。与之形成鲜明对比的是，第二种类型是单核细胞炎性血管病，与低滴度Ro(SS - A)抗体、补体正常、无高球蛋白血症和类风湿因子相关。两种类型的血管炎均与外周神经系统和中枢神经系统疾病相关。外周神经系统和中枢神经系统疾病累及整个神经轴，初步数据表明血管病是外周神经系统和中枢神经系统疾病的病因。诱发性感觉反应测试、脑脊液分析和磁共振成像已被证明是研究这些干燥综合征患者非常有价值的技术。初步数据表明，高剂量泼尼松或免疫抑制剂对治疗这些患者有效。
