Department of Neurology, Washington University School of Medicine, St. Louis, Missouri, USA.
Department of Pediatrics, Neurology Division, Nationwide Children's Hospital, Ohio State University, Columbus, Ohio, USA.
Muscle Nerve. 2020 Dec;62(6):705-709. doi: 10.1002/mus.27058. Epub 2020 Oct 2.
Childhood onset chronic inflammatory demyelinating polyneuropathy (CIDP) often requires long-term immunomodulatory therapy. We report a comprehensive review of our treatment of pediatric CIDP with a focus on high-dose weekly corticosteroids ("pulse oral corticosteroids"), a treatment method that is not commonly reported. We retrospectively reviewed medical records of pediatric patients with CIDP treated at our center between 2000 and 2018 for whom we had at least 12 mo follow-up. Here, we describe the demographics, disease course, treatment regimens, and long-term outcomes of these patients. Twenty-five patients were identified for analysis. Pulse oral corticosteroid monotherapy was the predominant maintenance treatment in 56% of patients. Patients were followed for a median of 4 y. Side effects were seen in a minority of patients. The probability of a normal exam or being off treatment at last follow-up was similar regardless of predominant maintenance therapy. Pulse oral corticosteroid therapy is a safe and effective long-term treatment option in children with CIDP.
儿童起病的慢性炎症性脱髓鞘性多发性神经病（CIDP）常需要长期免疫调节治疗。我们报告了对儿科 CIDP 进行的全面治疗回顾，重点关注高剂量每周皮质类固醇（“脉冲口服皮质类固醇”），这是一种不常见的治疗方法。我们回顾性分析了 2000 年至 2018 年在我们中心接受治疗且至少随访 12 个月的儿科 CIDP 患者的病历。在此，我们描述了这些患者的人口统计学、疾病过程、治疗方案和长期结果。确定了 25 名患者进行分析。脉冲口服皮质类固醇单药治疗是 56%患者的主要维持治疗。患者中位随访 4 年。少数患者出现副作用。无论主要维持治疗如何，最后一次随访时正常检查或停药的可能性相似。脉冲口服皮质类固醇治疗是儿童 CIDP 的一种安全有效的长期治疗选择。
