Division of Rheumatology, Department of Medicine, University of California, San Francisco, San Francisco, California, USA.
Curr Opin Rheumatol. 2019 Jan;31(1):40-45. doi: 10.1097/BOR.0000000000000559.
Vasculitis of medium-sized and small vessels commonly affects peripheral nerves and can occur in context of a systemic vasculitis with multiorgan involvement or a nonsystemic vasculitis limited to the peripheral nervous system. This review summarizes the clinical and pathological features of systemic and nonsystemic vasculitis of the peripheral nervous system.
Vasculitis of peripheral nerves is a diffuse process that affects the vasa nervorum along the entire length of affected nerves but appears to cause injury primarily in a zone in the proximal-middle of the nerve that is particularly susceptible to ischemic injury. Nerve biopsy can help establish the diagnosis of a systemic vasculitis, particularly when other organ involvement is not clinically apparent, and is required for diagnosis of nonsystemic vasculitic neuropathy. Observational studies suggest that nonsystemic vasculitic neuropathy responds to immunosuppressive therapy but conclusive data are lacking.
The current review summarizes the clinical and pathological features of both systemic and nonsystemic vasculitis of the peripheral nervous system so that clinicians can better recognize, make a more timely diagnosis, and thus treat this condition more effectively in their patients.
中等及小血管血管炎常累及周围神经,可发生于系统性血管炎伴多器官受累,或局限于周围神经系统的非系统性血管炎。本文总结了系统性和非系统性周围神经系统血管炎的临床和病理特征。
周围神经血管炎是一种弥漫性病变,影响受累神经全长的血管,但似乎主要在神经的近-中段易发生缺血性损伤的区域引起损伤。神经活检有助于确立系统性血管炎的诊断,特别是当其他器官受累在临床上不明显时,并且对非系统性血管炎性神经病的诊断是必需的。观察性研究表明,非系统性血管炎性神经病对免疫抑制治疗有反应,但缺乏确凿的数据。
本文综述了系统性和非系统性周围神经系统血管炎的临床和病理特征,以便临床医生更好地识别、更及时地诊断,并因此更有效地治疗患者的这种疾病。