Finsterer J
Krankenanstalt Rudolfstiftung, Vienna, Austria, Europe.
Acta Neurol Belg. 2009 Jun;109(2):100-13.
Vasculitis affecting the peripheral nerves predominantly manifests as subacute, progressive, asymmetric sensorimotor polyneuropathy or mononeuritis multiplex, and more rarely as painful mononeuropathy, pure sensory neuropathy, neuropathy of the cranial nerves, plexopathy, or as autonomic neuropathy. Vasculitic neuropathy may occur isolated or non-isolated (systemic) together with involvement of other organs. Systemic vasculitis with involvement of the peripheral nerves is further subdivided into primary (Takayasu syndrome, giant cell arteritis, classical panarteritis nodosa, thrombangitis obliterans, Kawasaki disease, Churg-Strauss syndrome, Wegener granulomatosis, cryoglobulinemic vasculitis, Behcet disease, microscopic polyangitis, Schoenlein Henoch purpura) or secondary systemic vasculitis (autoimmune connective tissue diseases, vasculitis from infection, sarcoidosis, malignancy, drugs, radiation, or diabetes). In addition to routine laboratory investigations and nerve conduction studies, nerve biopsy is essential for diagnosing the condition and to delineate it from differentials, although its sensitivity is only approximately 60%. Therapy of non-viral vasculitic neuropathy is based on corticosteroids and cyclophosphamide alone or in combination. Additional options include azathioprine, methotrexate, mycophenolate mofetil, or rituximab. In single cases immunoglobulins, immunoadsorbtion, or plasma exchange have been successfully applied. In case of virus-associated vasculitis interferon-alpha plus lamivudine or ribaverin may be beneficial.
主要影响周围神经的血管炎主要表现为亚急性、进行性、不对称性感觉运动性多发性神经病或多发性单神经炎，较少表现为疼痛性单神经病、纯感觉性神经病、颅神经神经病、神经丛病或自主神经病。血管炎性神经病可单独发生，也可与其他器官受累一起发生（系统性）。累及周围神经的系统性血管炎进一步分为原发性（高安综合征、巨细胞动脉炎、经典结节性多动脉炎、闭塞性血栓性脉管炎、川崎病、变应性肉芽肿性血管炎、韦格纳肉芽肿、冷球蛋白血症性血管炎、白塞病、显微镜下多血管炎、过敏性紫癜）或继发性系统性血管炎（自身免疫性结缔组织病、感染性血管炎、结节病、恶性肿瘤、药物、辐射或糖尿病）。除了常规实验室检查和神经传导研究外，神经活检对于诊断该病并与鉴别诊断区分开来至关重要，尽管其敏感性仅约为60%。非病毒性血管炎性神经病治疗基于单独或联合使用皮质类固醇和环磷酰胺。其他选择包括硫唑嘌呤、甲氨蝶呤、霉酚酸酯或利妥昔单抗。在个别情况下，免疫球蛋白、免疫吸附或血浆置换已成功应用。对于病毒相关性血管炎，α干扰素加拉米夫定或利巴韦林可能有益。
