PDF(Pubmed)
Abstract：
Immune axonal neuropathies are a particular group of immune-mediated neuropathies that occasionally accompany systemic autoimmune rheumatic diseases such as connective tissue dissorders and primary systemic vasculitides. Apart from vasculitis of vasa nervorum, various other mechanisms are involved in their pathogenesis, with possible therapeutic implications. Immune axonal neuropathies have highly heterogeneous clinical presentation and course, ranging from mild chronic distal sensorimotor polyneuropathy to severe subacute mononeuritis multiplex with rapid progression and constitutional symptoms such as fever, malaise, weight loss and night sweats, underpinning a vasculitic process. Sensory neuronopathy (ganglionopathy), small fiber neuropathy (sensory and/or autonomic), axonal variants of Guillain-Barré syndrome and cranial neuropathies have also been reported. In contrast to demyelinating neuropathies, immune axonal neuropathies show absent or reduced nerve amplitudes with normal latencies and conduction velocities on nerve conduction studies. Diagnosis and initiation of treatment are often delayed, leading to accumulating disability. Considering the lack of validated diagnostic criteria and evidence-based treatment protocols for immune axonal neuropathies, this review offers a comprehensive perspective on etiopathogenesis, clinical and paraclinical findings as well as therapy guidance for assisting the clinician in approaching these patients. High quality clinical research is required in order to provide indications and follow up rules for treatment in immune axonal neuropathies related to systemic autoimmune rheumatic diseases.
摘要：
免疫轴索神经病是一组特定的免疫介导的神经病，偶尔伴有全身性自身免疫性风湿性疾病，例如结缔组织疾病和原发性全身性血管炎。除了神经血管的血管炎,各种其他机制涉及其发病机理，具有可能的治疗意义。免疫性轴索神经病具有高度异质性的临床表现和病程，从轻度慢性远端感觉运动性多发性神经病到严重的亚急性多发性单神经炎，并伴有快速进展和发热等全身症状，萎靡不振,减肥和盗汗,支持血管化过程。感觉神经病（神经节病），小纤维神经病（感觉和/或自主神经），还报道了格林-巴利综合征和颅神经病变的轴突变异。与脱髓鞘神经病相反，在神经传导研究中，免疫轴索神经病变显示神经振幅缺失或降低，潜伏期和传导速度正常。诊断和治疗的开始往往被延迟，导致残疾累积。考虑到缺乏有效的诊断标准和循证治疗方案的免疫性轴索神经病，这篇综述提供了一个全面的病因，临床和副临床发现以及治疗指导，以协助临床医生接近这些患者。需要高质量的临床研究，以便为与全身性自身免疫性风湿性疾病相关的免疫性轴索神经病的治疗提供适应症和随访规则。
