Franques J, Azulay J-P, Pouget J, Attarian S
Centre de référence pour la prise en charge des maladies neuromusculaires et de la SLA, hôpital de la Timone, CHU de Marseille, 264, rue Saint-Pierre, 13385 Marseille, France.
Rev Med Interne. 2010 Jun;31(6):411-6. doi: 10.1016/j.revmed.2009.08.006. Epub 2010 Apr 14.
Chronic inflammatory demyelinating polyradiculoneuropathy (CIDP) is a demyelinating chronic neuropathy of immune origin whose diagnosis is based upon clinical, biological and electrophysiological data; previously critical to the diagnosis the nerve biopsy is now restricted to the rare situations where accurate diagnosis cannot be reached using these data alone. CIDP are mainly idiopathic, but a few associated diseases must be sought for as they require specific attention. Such associated diseases must particularly be discussed when the manifestations are severe or resistant to immunomodulating or immunosuppressive agents. Indeed, idiopathic CIDP are usually responsive to these treatments. The effectiveness of these treatments is limited by the importance of the secondary axonal loss. The dependence or the resistance may sometimes justify the association of several immunomodulating treatments. A single randomized controlled trial support the use of cytotoxic drugs and none with rituximab.
慢性炎性脱髓鞘性多发性神经根神经病(CIDP)是一种免疫源性的脱髓鞘性慢性神经病,其诊断基于临床、生物学和电生理数据;以前对诊断至关重要的神经活检现在仅限于仅使用这些数据无法得出准确诊断的罕见情况。CIDP主要是特发性的,但必须寻找一些相关疾病,因为它们需要特别关注。当表现严重或对免疫调节或免疫抑制药物耐药时,尤其必须讨论此类相关疾病。事实上,特发性CIDP通常对这些治疗有反应。这些治疗的有效性受到继发性轴突丢失程度的限制。依赖性或耐药性有时可能证明联合使用几种免疫调节治疗是合理的。一项随机对照试验支持使用细胞毒性药物,而没有试验支持使用利妥昔单抗。