Vrancken Alexander F J E, Said Gérard
Department of Neurology, Rudolf Magnus Institute of Neuroscience, University Medical Centre, Utrecht, The Netherlands.
Handb Clin Neurol. 2013;115:463-83. doi: 10.1016/B978-0-444-52902-2.00026-6.
Vasculitis is a primary phenomenon in autoimmune diseases such as polyarteritis nodosa, Wegener's granulomatosis, Churg-Strauss syndrome, microscopic polyangiitis, and essential mixed cryoglobulinemia. As a secondary feature vasculitis may complicate, for example, connective tissue diseases, infections, malignancies, and diabetes. Vasculitic neuropathy is a consequence of destruction of the vessel wall and occlusion of the vessel lumen of small epineurial arteries. Sometimes patients present with nonsystemic vasculitic neuropathy, i.e., vasculitis limited to peripheral nerves and muscles with no evidence of further systemic involvement. Treatment with corticosteroids, sometimes in combination with other immunosuppressants, is required to control the inflammatory process and prevent further ischemic nerve damage.
血管炎是结节性多动脉炎、韦格纳肉芽肿病、变应性肉芽肿性血管炎、显微镜下多血管炎和原发性混合性冷球蛋白血症等自身免疫性疾病中的主要现象。作为一种次要特征,血管炎可能会使结缔组织疾病、感染、恶性肿瘤和糖尿病等疾病复杂化。血管炎性神经病变是小神经外膜动脉血管壁破坏和管腔闭塞的结果。有时患者表现为非系统性血管炎性神经病变,即血管炎仅限于周围神经和肌肉,无进一步系统性受累的证据。需要使用皮质类固醇进行治疗,有时联合其他免疫抑制剂,以控制炎症过程并防止进一步的缺血性神经损伤。