Collins M P, Periquet M I, Mendell J R, Sahenk Z, Nagaraja H N, Kissel J T
Neurosciences Department, Marshfield Clinic, Marshfield, WI 54449, USA.
Neurology. 2003 Sep 9;61(5):623-30. doi: 10.1212/01.wnl.0000082715.48844.3e.
Nonsystemic vasculitic neuropathy (NSVN) is an uncommon disorder. Few series with small numbers of patients have been reported. The prognosis and treatment of patients presenting with NSVN remain uninvestigated. The authors sought to address these issues by assembling a large retrospective cohort with extended follow-up.
All nerve biopsies performed over 20 years were reviewed; cases with definite, probable, or possible vasculitis were segregated for clinical correlation. Patients satisfying clinical criteria for NSVN at presentation were selected. Clinicopathologic, treatment, and outcome measures were analyzed in patients followed for > or = 6 months.
A total of 48 patients (30 women, 18 men) with a median of 63 months of follow-up were identified. Most patients (85%) had extensive, overlapping involvement of multiple nerves. Only one had a symmetric polyneuropathy. Most neuropathies (96%) were painful. In 96%, nerve damage was distally accentuated, but most had concurrent proximal weakness. Diagnostic sensitivity was 58% for superficial peroneal nerve/peroneus brevis muscle biopsy and 47% for sural nerve biopsy. Combination corticosteroid/cytotoxic therapy was more effective than corticosteroid monotherapy in inducing remission and improving disability, with trends toward reduced relapses and chronic pain. Treatment with cyclophosphamide for >6 months decreased the relapse rate, which was 46% for all patients. Disease/treatment-related mortality was 10%. Six percent developed cutaneous involvement. Although chronic pain persisted in 60% of survivors, 80% had good outcomes.
NSVN nearly always presents as an asymmetric, distally accentuated, painful, sensorimotor polyneuropathy. Risks for systemic spread and death are small, and, aside from pain, neurologic prognosis is unexpectedly good. Although this was not a randomized controlled trial, combination therapy produced the best outcome in this cohort.
非系统性血管炎性神经病（NSVN）是一种罕见疾病。仅有少数报告涉及少量患者的系列研究。NSVN患者的预后及治疗仍未得到研究。作者试图通过组建一个具有长期随访的大型回顾性队列来解决这些问题。
回顾了20年间所做的所有神经活检；将确诊、很可能或可能患有血管炎的病例进行分类以进行临床关联分析。选取初诊时符合NSVN临床标准的患者。对随访时间≥6个月的患者的临床病理、治疗及转归指标进行分析。
共确定了48例患者（30例女性，18例男性），中位随访时间为63个月。大多数患者（85%）有多条神经广泛、重叠受累。仅1例有对称性多发性神经病。大多数神经病（96%）有疼痛症状。96%的患者神经损害以远端为主，但大多数同时伴有近端肌无力。腓浅神经/短腓骨肌活检的诊断敏感性为58%，腓肠神经活检为47%。联合使用皮质类固醇/细胞毒性药物治疗在诱导缓解及改善残疾方面比单纯皮质类固醇治疗更有效，且有减少复发及慢性疼痛的趋势。使用环磷酰胺治疗>6个月可降低复发率，所有患者的复发率为46%。疾病/治疗相关死亡率为10%。6%的患者出现皮肤受累。尽管60%的幸存者仍有慢性疼痛，但80%的患者预后良好。
NSVN几乎总是表现为不对称、以远端为主、疼痛性的感觉运动性多发性神经病。全身播散及死亡风险较小，除疼痛外，神经学预后出人意料地良好。尽管这不是一项随机对照试验，但联合治疗在该队列中产生了最佳结果。
