Ramchandren Sindhu, Lewis Richard A
Wayne State University, Detroit Medical Center, Department of Neurology, Michigan 48201, USA.
Curr Opin Neurol. 2009 Oct;22(5):480-5. doi: 10.1097/WCO.0b013e32832fd563.
The management of peripheral neuropathy associated with monoclonal gammopathies has been advanced by recent clinical studies. We review the causal association between monoclonal gammopathy and neuropathy, and critically review the recent evidence on treatment.
IgM monoclonal gammopathy of undetermined significance (MGUS) is the most commonly found monoclonal gammopathy associated with neuropathy. Neuropathies associated with specific lymphoproliferative disorders may not respond to treatments aimed at that disorder. Standard immunomodulatory agents including steroids, intravenous immunoglobulin, and plasmapheresis have shown limited efficacy in IgM monoclonal gammopathy of undetermined significance. Newer studies have shown promising results with rituximab, a monoclonal antibody which targets the B cell surface antigen CD20 and results in a rapid and sustained depletion of B cells.
There is a clear association between peripheral neuropathy and IgM MGUS with characteristic clinical, electrophysiology and pathologic features that make the disorder distinct from chronic inflammatory demyelinating polyneuropathy. The IgG and IgA monoclonal gammopathies are rarely associated with specific neuropathies. Long-term studies looking at the association between specific immunologic markers and disease recurrence are needed to ultimately develop targeted therapies.
近期的临床研究推动了与单克隆丙种球蛋白病相关的周围神经病变的管理。我们回顾单克隆丙种球蛋白病与神经病变之间的因果关联，并严格审视近期关于治疗的证据。
意义未明的IgM单克隆丙种球蛋白病（MGUS）是最常发现的与神经病变相关的单克隆丙种球蛋白病。与特定淋巴增殖性疾病相关的神经病变可能对针对该疾病的治疗无反应。包括类固醇、静脉注射免疫球蛋白和血浆置换在内的标准免疫调节药物在意义未明的IgM单克隆丙种球蛋白病中显示出有限的疗效。较新的研究显示，利妥昔单抗取得了有前景的结果，利妥昔单抗是一种靶向B细胞表面抗原CD20的单克隆抗体，可导致B细胞快速且持续的耗竭。
周围神经病变与IgM MGUS之间存在明确关联，其具有特征性的临床、电生理和病理特征，使该疾病有别于慢性炎症性脱髓鞘性多发性神经病。IgG和IgA单克隆丙种球蛋白病很少与特定神经病变相关。最终开发靶向治疗需要开展关于特定免疫标志物与疾病复发之间关联的长期研究。
