Department of Neurology, Graduate School of Medicine, Chiba University, 1-8-1, Inohana, Chuo-ku, Chiba, 260-8670, Japan.
Department of Neurology, Graduate School of Medical Science, Kyoto Prefectural University of Medicine, Kyoto, Japan.
J Neurol. 2021 Oct;268(10):3781-3788. doi: 10.1007/s00415-021-10520-x. Epub 2021 Mar 27.
This study aimed to investigate the timing of meeting the criteria for a status of "minimal manifestation (MM) or better" and the factors that influenced whether "MM or better status" or "MM or better status with an oral prednisolone (PSL) dose of 5 mg/day or less (5-mg MM)" was met in patients with acetylcholine receptor (AChR) antibody-positive generalized myasthenia gravis (MG).
We performed a retrospective study in 93 patients with AChR antibody-positive generalized MG who were followed for 3 years after the start of immunotherapy. We reviewed clinical data, such as MG-related symptoms, the MG activities of daily living profile (MGADL) score, immunotherapy including the dose of PSL, and achievement of the status of MM or better at baseline and 3, 6, 12, 24, and 36 months after treatment.
An MM or better status was achieved in 60% of the patients 3 months and in 90% of the patients 2 years after initiating immunotherapy. At 2 years, 60% of the patients had achieved the treatment goal, which was an "5-mg MM". More frequent plasmapheresis and higher dose of PSL within 3 months after immunotherapy initiation were associated with difficulty in achieving the 5-mg MM status at 2 years.
Approximately 60% of the MG patients achieved the treatment goal within 2 years after treatment. PSL dose and the cumulative number of plasmapheresis procedures at 3 months after immunotherapy initiation may help identify treatment-resistant patients with MG.
本研究旨在探讨符合“微小表现（MM）或更好”标准的时间以及影响乙酰胆碱受体（AChR）抗体阳性全身型重症肌无力（MG）患者达到“MM 或更好状态”或“MM 或更好状态且泼尼松龙（PSL）剂量为 5mg/日或更低（5mgMM）”的因素。
我们对 93 例 AChR 抗体阳性全身型 MG 患者进行了回顾性研究，这些患者在免疫治疗开始后随访了 3 年。我们回顾了临床数据，如 MG 相关症状、MG 日常生活活动量表（MGADL）评分、免疫治疗包括 PSL 剂量以及在基线和治疗后 3、6、12、24 和 36 个月时达到 MM 或更好状态的情况。
60%的患者在治疗 3 个月时达到 MM 或更好状态，90%的患者在 2 年内达到 MM 或更好状态。2 年内，60%的患者达到了治疗目标，即“5mgMM”。免疫治疗开始后 3 个月内更频繁的血浆置换和更高剂量的 PSL 与 2 年内难以达到 5mgMM 状态相关。
大约 60%的 MG 患者在治疗后 2 年内达到了治疗目标。PSL 剂量和免疫治疗开始后 3 个月内的血浆置换累积次数可能有助于识别治疗抵抗的 MG 患者。
