Harada Yohei, Herrmann David N, Logigian Eric L
Department of Neurology, University of Rochester School of Medicine and Dentistry, Rochester, NY.
J Clin Neuromuscul Dis. 2017 Dec;19(2):57-65. doi: 10.1097/CND.0000000000000179.
Childhood chronic inflammatory demyelinating polyradiculoneuropathy (CIDP) responds favorably to immunomodulatory treatment. However, the optimal sequencing and selection of immunotherapy is uncertain.
Using accepted diagnostic criteria, pediatric patients with CIDP seen at our center from 1999 to 2015 were identified retrospectively through medical record review. Clinical details and treatment responses were tabulated.
Ten patients (age 4-16, 6 women) with definite (N = 8) or possible (N = 2) CIDP met criteria. All were initially treated with IVIg; 6 responded but 4 did not. All 4 IVIG nonresponders improved with twice-weekly high-dose oral prednisone, as did 1 IVIg responder who was also treated with twice-weekly oral prednisone when IVIg was discontinued. Pulse steroids were well tolerated.
Pulse oral corticosteroid therapy holds promise as an alternative treatment to IVIG in pediatric CIDP. Future multicenter studies are warranted to determine the comparative efficacy and safety of weekly pulse oral corticosteroids versus IVIg in pediatric CIDP.
儿童慢性炎性脱髓鞘性多发性神经根神经病（CIDP）对免疫调节治疗反应良好。然而，免疫治疗的最佳顺序和选择尚不确定。
采用公认的诊断标准，通过病历回顾对1999年至2015年在本中心就诊的CIDP儿科患者进行回顾性识别。将临床细节和治疗反应制成表格。
10例确诊（N = 8）或可能（N = 2）CIDP患者符合标准。所有患者最初均接受静脉注射免疫球蛋白（IVIg）治疗；6例有反应，但4例无反应。所有4例IVIg无反应者经每周两次高剂量口服泼尼松治疗后病情改善，1例IVIg有反应者在停用IVIg后也接受每周两次口服泼尼松治疗，病情同样改善。脉冲类固醇耐受性良好。
脉冲口服皮质类固醇疗法有望成为儿科CIDP中IVIg的替代治疗方法。未来有必要开展多中心研究，以确定每周脉冲口服皮质类固醇与IVIg在儿科CIDP中的比较疗效和安全性。
