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杜克重症肌无力临床登记处:I. 描述和人口统计学。

The Duke myasthenia gravis clinic registry: I. Description and demographics.

机构信息

Neuromuscular Division, Department of Neurology, Duke University Medical Center, Durham, North Carolina, USA.

出版信息

Muscle Nerve. 2021 Feb;63(2):209-216. doi: 10.1002/mus.27120. Epub 2020 Dec 5.

DOI:10.1002/mus.27120
PMID:33205437
Abstract

INTRODUCTION

The Duke Myasthenia Gravis (MG) Clinic Registry is a disease-specific database containing physician-derived data from patients seen in the Duke MG Clinic since 1980.

METHODS

Data from 1060 MG patients initially seen between 1980 and 2008 were reviewed.

RESULTS

Fifty-four percent were male. Symptoms began after age 50 in 66% of males and 42% of females. Peak onset age in males was in their 60's; females had no predominant onset age. Onset age for both sexes increased from 1980 to 2008. Thymoma was present in 8.5%. Weakness was limited to ocular muscles for at least 2 y in 22% and became generalized later in 8.3% of these. Acetylcholine receptor antibodies were present in 78% overall, 82% with generalized MG and 52% with ocular MG (OMG). The distribution of MG disease class was similar in males and females, except that a greater proportion of women experienced myasthenic crisis and men were more likely to have OMG.

DISCUSSION

Data in the Registry permit comprehensive and longitudinal analysis of a validated MG population. Analysis of Registry data shows that the frequency of AChR antibody negative MG, ocular MG, and thymoma are similar to other reports, but the onset age and proportion of males have progressively increased compared to studies published more than 20 y ago. These observations demonstrate the value of collecting comprehensive clinical information and comparing historic and contemporary populations. Other potential uses of Registry data include comparison of outcome measures in different disease subgroups and the response to specific treatments.

摘要

简介

杜克重症肌无力(MG)临床注册中心是一个特定疾病的数据库,其中包含自 1980 年以来在杜克 MG 临床中心就诊的患者的医生提供的数据。

方法

对 1980 年至 2008 年间首次就诊的 1060 名 MG 患者的数据进行了回顾。

结果

54%为男性。66%的男性和 42%的女性症状在 50 岁后出现。男性发病高峰年龄在 60 多岁;女性没有明显的发病年龄。男女发病年龄均从 1980 年到 2008 年有所增加。8.5%的患者存在胸腺瘤。至少 2 年局限于眼外肌的肌无力在 22%的患者中存在,其中 8.3%的患者后来出现全身肌无力。乙酰胆碱受体抗体总体阳性率为 78%,全身性 MG 患者为 82%,眼肌型 MG(OMG)患者为 52%。男性和女性的 MG 疾病分类分布相似,只是女性更易发生肌无力危象,而男性更易发生 OMG。

讨论

注册中心的数据允许对经过验证的 MG 人群进行全面和纵向分析。对注册中心数据的分析表明,AChR 抗体阴性 MG、眼肌型 MG 和胸腺瘤的频率与其他报告相似,但与 20 多年前发表的研究相比,发病年龄和男性比例逐渐增加。这些观察结果表明,收集全面的临床信息并比较历史和当代人群是有价值的。注册中心数据的其他潜在用途包括比较不同疾病亚组的结局测量指标以及对特定治疗的反应。

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