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眼肌型重症肌无力的发病年龄及影响治疗反应的因素。

Age of onset and factors affecting treatment responses in ocular myasthenia gravis.

机构信息

Department of Ophthalmology, Rangsit University, Rajavithi Hospital, 2 Phaya Thai Rd, Thung Phaya Thai, Ratchathewi, Bangkok, 10400, Thailand.

Faculty of Medicine Rajavithi Hospital, Rangsit University, 2 Phaya Thai Rd, Thung Phaya Thai, Ratchathewi, Bangkok, 10400, Thailand.

出版信息

Int Ophthalmol. 2023 Aug;43(8):2777-2785. doi: 10.1007/s10792-023-02676-4. Epub 2023 Mar 7.

DOI:10.1007/s10792-023-02676-4
PMID:36879110
Abstract

BACKGROUND

Ocular myasthenia gravis (OMG) is an autoimmune disease which causes ptosis, diplopia, or both. It can be categorized as early or late onset, with differing presenting characteristics and prognoses. Currently, there is limited information available to compare characteristics and outcomes in onset groups in Thailand.

OBJECTIVE

To describe and compare baseline characteristics and outcomes in OMG patients classified by onset groups and to investigate the factors associated with the disease, especially in terms of treatment responses classified according to the MGFA Post-Intervention Status (MGFA-PIS).

METHODS

OMG patients diagnosed between January 2014 and March 2021 at Rajavithi Hospital, Thailand, were categorized into 2 groups based on age of onset, and baseline characteristics were analyzed and compared. The treatment responses of each group in terms of time to achievement of minimal manifestations (MM) were analyzed.

RESULTS

Eighty-one patients (38 with early and 43 with late onset) were included, and the mean (SD) follow-up time was 35.85 months (17.25). There was no significant difference between the baseline characteristics of the two groups. A low dose of pyridostigmine was more commonly used in the early-onset group (p = 0.01), while the mean dose of corticosteroids was significantly lower in the late-onset patients (p < 0.001). We found that seropositivity of acetylcholine receptor antibody decreased the odds ratio of achievement of MM (OR 0.185, 95% CI 0.043-0.789, p = 0.023) and receiving a high dose of pyridostigmine (≥ 120 mg/day) increased the odds ratio of achieving it (OR 8.296, 95% CI 2.136-32.226, p = 0.002).

CONCLUSIONS

A higher dose of pyridostigmine may be necessary for achievement of favorable treatment response. AChRAb seropositivity is a predictor for unfavorable treatment response in Thai populations.

摘要

背景

眼肌型重症肌无力(OMG)是一种自身免疫性疾病,可导致上睑下垂、复视或两者兼有。它可以分为早发型或晚发型,具有不同的表现特征和预后。目前,泰国关于发病组之间的特征和结果比较,信息有限。

目的

描述和比较按发病组分类的 OMG 患者的基线特征和结果,并研究与疾病相关的因素,特别是根据 MGFA 干预后状态(MGFA-PIS)分类的治疗反应。

方法

2014 年 1 月至 2021 年 3 月在泰国 Rajavithi 医院诊断为 OMG 的患者根据发病年龄分为 2 组,分析并比较其基线特征。分析每组在达到最小表现(MM)时间方面的治疗反应。

结果

共纳入 81 例患者(早发型 38 例,晚发型 43 例),平均(SD)随访时间为 35.85 个月(17.25)。两组间基线特征无显著性差异。早发型组更常使用低剂量吡啶斯的明(p = 0.01),而晚发型患者的皮质类固醇平均剂量明显较低(p < 0.001)。我们发现乙酰胆碱受体抗体阳性的患者,达到 MM 的可能性降低(OR 0.185,95%CI 0.043-0.789,p = 0.023),而使用高剂量吡啶斯的明(≥120mg/天)的可能性增加(OR 8.296,95%CI 2.136-32.226,p = 0.002)。

结论

达到良好治疗反应可能需要更高剂量的吡啶斯的明。在泰国人群中,AChRAb 阳性是治疗反应不良的预测因素。

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