Department of Neurology, Amsterdam University Medical Center, University of Amsterdam, Amsterdam Neuroscience, Amsterdam, The Netherlands.
Department of Neurology, Erasmus MC, University Medical Center, Rotterdam, The Netherlands.
J Neurol. 2022 Feb;269(2):945-955. doi: 10.1007/s00415-021-10677-5. Epub 2021 Jun 26.
To assess clinical outcome in treatment-naive patients with chronic inflammatory demyelinating polyradiculoneuropathy (CIDP).
We included adult treatment-naive patients participating in the prospective International CIDP Outcome Study (ICOS) that fulfilled the European Federation of Neurological Societies/Peripheral Nerve Society (EFNS/PNS) diagnostic criteria for CIDP. Patients were grouped based on initial treatment with (1) intravenous immunoglobulin (IVIg), (2) corticosteroid monotherapy or (3) IVIg and corticosteroids (combination treatment). Outcome measures included the inflammatory Rasch-built overall disability scale (I-RODS), grip strength, and Medical Research Council (MRC) sum score. Treatment response, treatment status, remissions (improved and untreated), treatment changes, and residual symptoms or deficits were assessed at 1 year.
Forty patients were included of whom 18 (45%) initially received IVIg, 6 (15%) corticosteroids, and 16 (40%) combination treatment. Improvement on ≥ 1 of the outcome measures was seen in 31 (78%) patients. At 1 year, 19 (48%) patients were still treated and fourteen (36%) patients were in remission. Improvement was seen most frequently in patients started on IVIg (94%) and remission in those started on combination treatment (44%). Differences between groups did not reach statistical significance. Residual symptoms or deficits ranged from 25% for neuropathic pain to 96% for any sensory deficit.
Improvement was seen in most patients. One year after the start of treatment, more than half of the patients were untreated and around one-third in remission. Residual symptoms and deficits were common regardless of treatment.
评估初治慢性炎症性脱髓鞘性多发性神经病(CIDP)患者的临床转归。
我们纳入了参加前瞻性国际 CIDP 结局研究(ICOS)的初治成年患者,这些患者符合欧洲神经病学学会/周围神经学会(EFNS/PNS)CIDP 的诊断标准。根据初始治疗分为三组:(1)静脉注射免疫球蛋白(IVIg)、(2)皮质类固醇单药治疗或(3)IVIg 和皮质类固醇(联合治疗)。疗效评估指标包括改良后的炎症 Rasch 整体残疾量表(I-RODS)、握力和运动医学研究委员会(MRC)总分。治疗 1 年后评估疗效、治疗状态、缓解(改善和未治疗)、治疗方案改变以及残留症状或缺陷。
共纳入 40 例患者,其中 18 例(45%)患者初始接受 IVIg 治疗,6 例(15%)患者接受皮质类固醇治疗,16 例(40%)患者接受联合治疗。31 例(78%)患者至少有 1 项疗效评估指标改善。治疗 1 年后,19 例(48%)患者仍在接受治疗,14 例(36%)患者处于缓解状态。IVIg 起始治疗的患者中改善最为常见(94%),联合治疗起始治疗的患者中缓解最为常见(44%)。组间差异无统计学意义。残留症状或缺陷范围从神经病理性疼痛的 25%到任何感觉缺陷的 96%。
大多数患者有改善。治疗开始 1 年后,超过一半的患者未接受治疗,约三分之一的患者处于缓解状态。无论治疗方案如何,残留症状和缺陷都很常见。