自身免疫性结节病诊疗进展

Progress in the Diagnosis and Treatment of Autoimmune Nodopathies

既往在自身免疫性神经病患者中发现针对结旁蛋白的特异性自身抗体。这些特定抗体与特定的临床病理特征相关,不同于经典的慢性炎症性脱髓鞘多神经根神经病。这些患者目前被单独归类为自身免疫性结节病(Autoimmune Nodopathies, AN)。儿童自身免疫性结节病作为从慢性炎症性脱髓鞘多神经病变演变出的独立新型疾病概念,对其认识尚不足。本研究旨在归纳自身免疫性结节病的临床特征及治疗方案选择,以提高对其疾病的认识。

Specific autoantibodies against paraprotein have previously been found in patients with autoimmune neuropathies. These specific antibodies have been associated with specific clinicopathological features, distinct from the classical chronic inflammatory demyelinating polyradiculoneuropathy. These patients are now classified separately as autoimmune nodopathies (AN). Autoimmune nodopathies in children are poorly understood as a separate novel disease concept evolving from chronic inflammatory demyelinating polyneuropathy. This study aimed to summarize the clinical features of autoimmune nodopathies and the choice of a therapeutic regimen to improve the understanding of their disease.