Briani Chiara, Cocito Dario, Campagnolo Marta, Doneddu Pietro Emiliano, Nobile-Orazio Eduardo
Neurology Unit, Department of Neuroscience, University of Padova, Via Giustiniani, 5, 35128, Padova, Italy.
Istituti Clinici Scientifici Maugeri, Torino, Italy.
Neurol Sci. 2022 Dec;43(Suppl 2):605-614. doi: 10.1007/s10072-020-04998-y. Epub 2021 Jan 16.
Chronic immune-mediated neuropathies, including chronic inflammatory demyelinating polyradiculoneuropathy (CIDP), neuropathies associated with monoclonal gammopathy, and multifocal motor neuropathy (MMN), are a group of disorders deemed to be caused by an immune response against peripheral nerve antigens. Several immune therapies have been reported to be variably effective in these neuropathies including steroids, plasma exchange, and high-dose intravenous (IVIg) or subcutaneous (SCIg) immunoglobulins. These therapies are however far from being invariably effective and may be associated with a number of side effects leading to the use of immunosuppressive agents whose efficacy has not been so far confirmed in randomized trials. More recently, new biological agents, such as rituximab, have proved to be effective in patients with neuropathy associated with IgM monoclonal gammopathy and are currently tested in CIDP.
慢性免疫介导性神经病，包括慢性炎性脱髓鞘性多发性神经根神经病（CIDP）、与单克隆丙种球蛋白病相关的神经病以及多灶性运动神经病（MMN），是一组被认为由针对周围神经抗原的免疫反应引起的疾病。据报道，几种免疫疗法在这些神经病中具有不同程度的疗效，包括类固醇、血浆置换以及大剂量静脉注射（IVIg）或皮下注射（SCIg）免疫球蛋白。然而，这些疗法远非总是有效，并且可能与许多副作用相关，这导致使用免疫抑制剂，而其疗效迄今尚未在随机试验中得到证实。最近，新型生物制剂，如利妥昔单抗，已被证明对与IgM单克隆丙种球蛋白病相关的神经病患者有效，目前正在CIDP中进行试验。
