Roggenbuck Johannes J, Boucraut Joseph, Delmont Emilien, Conrad Karsten, Roggenbuck Dirk
Medical Faculty, Technical University Dresden, Dresden, Germany.
Institut de Neurosciences de la Timone, Medicine Faculty, Aix Marseille University, Marseille, France.
Ann Transl Med. 2018 Sep;6(17):337. doi: 10.21037/atm.2018.07.34.
Chronic inflammatory demyelinating polyneuropathy (CIDP) is a rare immune-mediated neuropathy with demyelination of nerve fibers as leading morphological feature. The course of disease can be chronic progressive or remitting relapsing. Whereas for acute immune-mediated neuropathies several serological markers have been identified and used successfully in clinical routine, the serological diagnosis of chronic variants such as CIDP has not yet been evolved satisfactory. The typical CIDP and its various atypical variants are characterized by a certain diversity of clinical phenotype and response to treatment. Thus, diagnostic markers could aid in the differential diagnosis of CIDP variants and stratification of patients for a better treatment response. Most patients respond well to a causal therapy including steroids, intravenous immunoglobulins and plasmapheresis. Apart from electrophysiological and morphological markers, several autoantibodies have been reported as candidate markers for CIDP, including antibodies against glycolipids or paranodal/nodal molecules. The present review provides a summary of the progress in autoantibody testing in CIDP and its possible implication on the stratification of the CIDP variants and treatment response.
慢性炎症性脱髓鞘性多发性神经病(CIDP)是一种罕见的免疫介导性神经病,以神经纤维脱髓鞘为主要形态学特征。病程可为慢性进行性或缓解复发型。虽然对于急性免疫介导性神经病,已经确定了几种血清学标志物并成功应用于临床常规检查,但像CIDP这样的慢性变异型的血清学诊断尚未得到令人满意的发展。典型的CIDP及其各种非典型变异型具有临床表型和治疗反应的一定多样性。因此,诊断标志物有助于CIDP变异型的鉴别诊断和患者分层,以获得更好的治疗反应。大多数患者对包括类固醇、静脉注射免疫球蛋白和血浆置换在内的病因治疗反应良好。除了电生理和形态学标志物外,几种自身抗体已被报道为CIDP的候选标志物,包括抗糖脂或结旁/结区分子的抗体。本综述总结了CIDP自身抗体检测的进展及其对CIDP变异型分层和治疗反应的可能影响。