Department of Neurology, Yashoda Hospital, Hyderabad, Telangana, India.
Department of Neurology, National Institute of Mental Health and Neurosciences, Bengaluru, Karnataka, India.
Neurol India. 2019 Jan-Feb;67(Supplement):S62-S70. doi: 10.4103/0028-3886.250709.
To describe the clinical and laboratory findings of patients with biopsy proven vasculitic neuropathy.
Peripheral neuropathies form one of the most common disorders of the nervous system. However, more than 50% of them are labelled as 'idiopathic' and, therefore, treatment options become less. In this study, we tried to evaluate the phenotypic as well as laboratory characteristics and outcome of patients with biopsy proven vasculitic neuropathy.
Review of biopsy proven definite or probable vasculitic neuropathy was done.
The cohort consisted of 67 subjects. There were 21 patients of systemic vasculitis (SVS) and 46 of non-systemic vasculitic neuropathy (NSVN). The nerve biopsy revealed definite vasculitis in 37 and probable vasculitis in 30 patients. The symptoms at onset were paraesthesia (68.7%), and paraesthesia and weakness (28.4%). Diffuse polyneuropathy occurred in 70.1% patients. The course was chronic in the majority (80.59%) of patients. Electrophysiology revealed mononeuritis multiplex in 32.84%, and polyneuropathy in 67.16% of patients. Pure sensory neuropathy was present in 16.42%. Among the patients who had undergone bilateral nerve conduction studies, the majority (71.05%) of patients had an asymmetric neuropathy. An elevated erythrocyte sedimentation rate (ESR) was observed in 80.59% (mean 71.57 ± 30.81 mm/1hr [in SVS] and 35.24 ± 21.62mm/1 hr in NSVN) of patients. The treatment included steroids, other immunomodulators, and symptomatic medications. The mean follow up was 10.98 ± 9.58 months. The outcome was good in 73.46% (43.8% with SVS and 87.88% with NSVN) patients, with those having a NSVN having a significantly better outcome.
Vasculitis is a potentially treatable cause of peripheral neuropathy. The clinical features, electrophysiology, laboratory results and nerve biopsy may help in the diagnosis and categorization of patients into non-systemic and systemic vasculitic neuropathies. The long-term outcome is better in patients with NSVN compared to those with systemic vasculitis.
描述经活检证实的血管炎性神经病患者的临床和实验室发现。
周围神经病是神经系统最常见的疾病之一。然而，其中超过 50%被标记为“特发性”，因此治疗选择减少。在这项研究中，我们试图评估经活检证实的血管炎性神经病患者的表型以及实验室特征和结果。
对经活检证实的明确或可能的血管炎性神经病进行了回顾。
该队列包括 67 名患者。其中 21 例为系统性血管炎（SVS），46 例为非系统性血管炎性神经病（NSVN）。神经活检显示 37 例为明确血管炎，30 例为可能血管炎。发病时的症状为感觉异常（68.7%），感觉异常伴无力（28.4%）。弥漫性多神经病发生在 70.1%的患者中。大多数患者（80.59%）的病程为慢性。电生理学显示 32.84%的患者为单神经病多发性神经病，67.16%的患者为多神经病。16.42%的患者为单纯感觉神经病。在接受双侧神经传导研究的患者中，大多数（71.05%）患者存在不对称性神经病。80.59%（平均 71.57 ± 30.81mm/1hr[SVS]和 35.24 ± 21.62mm/1hr 在 NSVN）的患者出现红细胞沉降率升高。治疗包括类固醇、其他免疫调节剂和对症药物。平均随访时间为 10.98 ± 9.58 个月。73.46%（43.8%的 SVS 和 87.88%的 NSVN）的患者预后良好，NSVN 患者的预后明显更好。
血管炎是一种潜在可治疗的周围神经病病因。临床特征、电生理学、实验室结果和神经活检有助于诊断和将患者分类为非系统性和系统性血管炎性神经病。与系统性血管炎相比，NSVN 患者的长期预后更好。
