Franques J
Hôpital européen, 6, rue Désirée-Clary, 13003 Marseille, France; Hôpital La Casamance, 33, boulevard des Farigoules, 13400 Aubagne, France.
Rev Med Interne. 2019 Dec;40(12):808-815. doi: 10.1016/j.revmed.2019.07.007. Epub 2019 Oct 31.
Chronic inflammatory demyelinating polyradiculoneuropathies are acquired demyelinating neuropathies belonging to the group of autoimmune neuropathies. Since specific biological markers are present in less than 10% of cases, the diagnosis is based on the clinical and electrophysiological analysis of each patient. Furthermore, a decision-making algorithm ranking all other available paraclinical tools will guide the physician to the diagnosis of atypical forms. In nearly 80% of cases, these dysimmune neuropathies are responsive to first-line treatments, namely intravenous immunoglobulins, corticosteroids and plasma exchanges. A second line treatment may be proposed in case of no response, intolerance or inaccessibility to the three reference treatments. While some immunosuppressants or monoclonal antibodies can sometimes be very effective, there is currently no predictive marker or recommendation available to determine which treatment will be most appropriate for which patient.
慢性炎症性脱髓鞘性多发性神经根神经病是属于自身免疫性神经病组的获得性脱髓鞘性神经病。由于不到10%的病例存在特异性生物标志物,诊断基于对每位患者的临床和电生理分析。此外,一种对所有其他可用辅助临床工具进行排序的决策算法将指导医生诊断非典型形式。在近80%的病例中,这些免疫失调性神经病对一线治疗有反应,即静脉注射免疫球蛋白、皮质类固醇和血浆置换。如果对三种参考治疗无反应、不耐受或无法进行,则可提出二线治疗。虽然一些免疫抑制剂或单克隆抗体有时可能非常有效,但目前没有预测标志物或建议可用于确定哪种治疗最适合哪位患者。