Kieseier Bernd C, Hartung Hans-Peter
Department of Neurology, Heinrich-Heine-University, Moorenstrasse 5, 40225 Düsseldorf, Germany.
Semin Neurol. 2003 Jun;23(2):159-68. doi: 10.1055/s-2003-41132.
The Guillain-Barré syndrome (GBS) represents the prototypic immune-mediated peripheral neuropathy, which is now recognized as a group of conditions with diverse pathology and pathogenesis. The observation that plasma exchange and intravenous immunoglobulin result in clinical improvement, the presence of circulating antibodies targeting structures on peripheral nerve tissue in sera from GBS patients, and the deposition of immunoglobulins and complement demonstrated on myelinated fibers in nerve biopsies from affected patients point to a critical role of the humoral immune response in the pathogenesis of GBS. However, the observation of inflammatory infiltrates in nerve and the critical pathogenic role of neuritogenic T lymphocytes as demonstrated in the animal model support the concept that a disordered cellular immunity is also of critical importance in the pathogenesis of this neuropathy. Current treatment strategies are aimed at mitigating the harmful effects of the immune system on peripheral nerve. This review will address the rationale for immunotherapy in GBS based on experimental and immunologic studies of the pathogenesis of this disease.
吉兰-巴雷综合征(GBS)是典型的免疫介导性周围神经病,目前被认为是一组具有不同病理和发病机制的病症。血浆置换和静脉注射免疫球蛋白可使临床症状改善,GBS患者血清中存在针对周围神经组织结构的循环抗体,以及在受累患者的神经活检中显示有免疫球蛋白和补体在有髓纤维上沉积,这些都表明体液免疫反应在GBS发病机制中起关键作用。然而,在神经中观察到炎症浸润以及动物模型中显示的致神经炎T淋巴细胞的关键致病作用支持了这样一种观点,即紊乱的细胞免疫在这种神经病的发病机制中也至关重要。当前的治疗策略旨在减轻免疫系统对周围神经的有害影响。本综述将基于对该疾病发病机制的实验和免疫学研究,探讨GBS免疫治疗的理论依据。