Department of Neurology, Erasmus MC, University Medical Center Rotterdam, room BA 450, s-Gravendijkwal 230, Rotterdam, The Netherlands.
J Clin Immunol. 2010 May;30 Suppl 1(Suppl 1):S74-8. doi: 10.1007/s10875-010-9407-4.
Guillain-Barré syndrome (GBS) is an acute, immune-mediated polyneuropathy that often leads to severe weakness. Intravenous immunoglobulin (IVIG) is a proven effective treatment for GBS (class 1 evidence). However, about 25% of patients need artificial ventilation and 20% are still unable to walk unaided after 6 months. Important clinical factors associated with poor outcome are age, presence of preceding diarrhea and the severity of disability in the early course of disease. These clinical factors were combined in a clinical prognostic scoring scale, the Erasmus GBS Outcome Scale (EGOS).
GBS patients being unable to walk unaided are currently treated with a standard single IVIg dose (0.4 g/kg bodyweight for 5 days). A recent retrospective study in 174 GBS patients enrolled in one of our randomized controlled clinical trials showed that patients with a minor increase of serum IgG level after standard single IVIg dose recovered significantly slower. Additionally, fewer patients reached the ability to walk unaided at six months after correction for the known clinical prognostic factors (multivariate analysis; P < 0.022).
It is yet unknown why some GBS patients only have a minor increase after standard IVIg treatment. By using the EGOS it is possible to select GBS patients with a poor prognosis. These patients potentially may benefit from a second IVIg dose.
A standard dose of IVIG is not sufficiently effective in many GBS patients. Whether these patients might benefit from a second IVIg dose needs further investigation.
吉兰-巴雷综合征(GBS)是一种急性、免疫介导的多发性神经病,常导致严重的无力。静脉注射免疫球蛋白(IVIG)是治疗 GBS 的有效方法(1 级证据)。然而,约 25%的患者需要人工通气,20%的患者在疾病早期仍无法独立行走。与不良预后相关的重要临床因素包括年龄、有无前驱性腹泻以及疾病早期的残疾严重程度。这些临床因素被结合到一种临床预后评分量表中,即 Erasmus GBS 结局量表(EGOS)。
目前,无法独立行走的 GBS 患者接受标准单剂量 IVIg 治疗(0.4 g/kg 体重,连续 5 天)。最近,对我们的一项随机对照临床试验中 174 例 GBS 患者的回顾性研究显示,标准单剂量 IVIg 治疗后血清 IgG 水平仅轻度升高的患者恢复明显较慢。此外,在对已知临床预后因素进行校正后(多变量分析;P < 0.022),较少的患者在 6 个月时达到独立行走的能力。
目前尚不清楚为什么一些 GBS 患者在接受标准 IVIg 治疗后仅出现轻度升高。通过使用 EGOS,可以选择预后不良的 GBS 患者。这些患者可能从第二剂 IVIg 中受益。
标准剂量的 IVIG 对许多 GBS 患者的疗效并不充分。这些患者是否可能从第二剂 IVIg 中获益,需要进一步研究。