Schneider Christian, Wassermann Meike K, Fink Gereon R, Lehmann Helmar C
Department of Neurology, Medical Faculty and University Hospital of Cologne, Kerpener Straße 62, D-50937, Cologne, Germany.
Research Center Jülich, Institute of Neuroscience and Medicine (INM-3), Jülich, Germany.
Neurol Res Pract. 2022 Aug 15;4(1):32. doi: 10.1186/s42466-022-00198-5.
No controlled studies for non-systemic vasculitic neuropathy treatment exist (NSVN). We compared the treatment response to induction therapy commonly used in clinical practice in NSVN.
In this retrospective single-center study, 43 patients with biopsy-proven NSVN were analyzed. Patients were subdivided into groups depending on their initial treatment. Relapse rates, changes of motor and sensory symptoms, adverse events, predictors of relapses, and second-line treatment were compared.
Initial treatment regimens were corticosteroid monotherapy, cyclophosphamide monotherapy, pulsed corticosteroid therapy, and combination therapy. Discontinuation due to adverse events occurred in 6 of 43 patients. Clinical data did not differ between treatment groups. Within 12 months, 24.3% of patients relapsed. The median time to relapse was 4 (1.5, 6) months. No relapse occurred in the combination therapy group. However, there was no statistically significant difference in relapse-free survival between treatment groups (p = 0.58). Neither clinical data nor biopsy analysis predicted relapses sufficiently. As a second-line treatment, cyclophosphamide as mono- or combination therapy was used (7 of 9 patients) most frequently. One patient was treated with methotrexate, and one with IVIG.
Induction therapy used in clinical practice is effective and mainly well-tolerated in NSVN. Our data do not support an overall advantage of cyclophosphamide over corticosteroid monotherapy. Controlled trials comparing the effectiveness of induction and maintenance therapy in NSVN are warranted.
目前尚无针对非系统性血管炎性神经病变（NSVN）治疗的对照研究。我们比较了NSVN临床实践中常用诱导治疗的疗效。
在这项回顾性单中心研究中，分析了43例经活检证实为NSVN的患者。根据初始治疗将患者分为不同组。比较了复发率、运动和感觉症状的变化、不良事件、复发的预测因素以及二线治疗情况。
初始治疗方案包括糖皮质激素单药治疗、环磷酰胺单药治疗、脉冲式糖皮质激素治疗和联合治疗。43例患者中有6例因不良事件停药。各治疗组之间的临床数据无差异。12个月内，24.3%的患者复发。复发的中位时间为4（1.5，6）个月。联合治疗组未出现复发。然而，各治疗组之间的无复发生存率无统计学显著差异（p = 0.58）。临床数据和活检分析均不能充分预测复发。作为二线治疗，最常使用环磷酰胺单药或联合治疗（9例患者中的7例）。1例患者接受甲氨蝶呤治疗，1例接受静脉注射免疫球蛋白治疗。
临床实践中使用的诱导治疗在NSVN中有效且耐受性良好。我们的数据不支持环磷酰胺相对于糖皮质激素单药治疗具有总体优势。有必要进行对照试验比较NSVN诱导治疗和维持治疗的有效性。
