Department of Pediatrics, Seoul National University College of Medicine, Pediatric Clinical Neuroscience Center, Seoul National University Children's Hospital, Seoul, Republic of Korea.
Department of Pediatrics, Seoul National University Bundang Hospital, Seoul, Republic of Korea.
Neuromuscul Disord. 2019 Sep;29(9):684-692. doi: 10.1016/j.nmd.2019.06.010. Epub 2019 Jun 19.
Childhood chronic inflammatory demyelinating polyradiculoneuropathy (CIDP) is a rare condition, and the optimal treatment strategy is not well established, especially in refractory cases. We analyzed the clinical features and treatment outcomes of 14 cases of childhood CIDP with more than 12 months of follow-up. Of the 14 cases, 10 cases were considered refractory to the conventional first-line treatment. In the monophasic group (n = 6), plasmapheresis resulted in a better treatment response than did IVIG. Monophasic refractory cases (n = 4) were especially responsive to plasmapheresis. In the polyphasic group (n = 8), IVIG and plasmapheresis had comparable effects. Among them six polyphasic patients were refractory to the first-line treatment options and received additional immunosuppressants. Four treatment-refractory polyphasic patients received cyclosporine and achieved successful disease control. With regard to the long-term outcomes, six patients showed minimal symptoms and no relapse within 6 months. Our results suggest that early administration of plasmapheresis in a monophasic course and cyclosporine in a polyphasic course may be effective treatment options for refractory childhood CIDP.
儿童慢性炎症性脱髓鞘性多发神经病（CIDP）较为罕见，其最佳治疗策略尚未明确，尤其是对于难治性病例。我们分析了 14 例随访时间超过 12 个月的儿童 CIDP 病例的临床特征和治疗结局。14 例病例中，10 例被认为对常规一线治疗耐药。单相组（n=6）中，血浆置换的治疗反应优于静脉注射免疫球蛋白（IVIG）。单相耐药病例（n=4）对血浆置换反应尤其敏感。在多相组（n=8）中，IVIG 和血浆置换的效果相当。其中 6 例多相患者对一线治疗方案耐药，接受了额外的免疫抑制剂。4 例治疗耐药的多相患者接受环孢素治疗后成功控制了疾病。关于长期结局，6 例患者在 6 个月内症状轻微且无复发。我们的结果表明，在单相病程中早期给予血浆置换，在多相病程中给予环孢素可能是治疗难治性儿童 CIDP 的有效选择。
