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MuSK 阳性重症肌无力妊娠病例系列:单中心研究。

Pregnancy in MuSK-positive myasthenia gravis: A single-center case series.

机构信息

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

Division of Neurology, Centra Neurology, Lynchburg, Virginia, USA.

出版信息

Muscle Nerve. 2023 Jul;68(1):85-90. doi: 10.1002/mus.27839. Epub 2023 May 7.

Abstract

INTRODUCTION/AIMS: Myasthenia gravis (MG) with muscle-specific tyrosine kinase (MuSK) antibodies (MMG) is predominantly seen in women of childbearing age. Our objective in this study was to describe the course of MMG during pregnancy and within 6 months postpartum, and to document any effect on fetal health.

METHODS

A retrospective review was performed of medical records of patients with MMG seen in the Duke Myasthenia Gravis Clinic from 2003 to 2022. MMG patients with onset of MMG symptoms before or during pregnancy as well as within 6 months postpartum were reviewed.

RESULTS

A total of 14 pregnancies in 10 patients were included in our study cohort. Initial MG symptoms developed during pregnancy or within 6 months postpartum in six patients. Four patients had two pregnancies, three of whom developed MG during their first pregnancy. In the patients diagnosed before pregnancy, MG symptoms increased in five of eight patients during pregnancy or postpartum. Four patients required rescue therapy with plasma exchange or intravenous immunoglobulin during pregnancy or postpartum. One patient had a cesarean section after prolonged labor due to failure of progression. There were no other complications of pregnancy or delivery, and all infants were healthy at delivery.

DISCUSSION

As in non-MuSK MG, women with MMG may also have worsening or may develop initial MG symptoms during pregnancy or within 6 months postpartum. More aggressive medical therapy may be required for pregnant patients with MMG. Further study is needed to identify the mechanism and risk of worsening of MMG during pregnancy or postpartum.

摘要

简介/目的:伴肌肉特异性酪氨酸激酶(MuSK)抗体的重症肌无力(MG)主要见于育龄期女性。我们本研究的目的是描述 MuSK 抗体阳性的重症肌无力(MMG)患者在怀孕期间及产后 6 个月内的病程,并记录对胎儿健康的任何影响。

方法

回顾性分析了 2003 年至 2022 年在杜克重症肌无力诊所就诊的 MMG 患者的病历。纳入了 MMG 症状在妊娠前或妊娠期间以及产后 6 个月内发生的 MMG 患者。

结果

我们的研究队列共包括 10 名患者的 14 次妊娠。6 名患者最初的 MG 症状发生在妊娠期间或产后 6 个月内。4 名患者有两次妊娠,其中 3 名在第一次妊娠期间发生 MG。在妊娠前诊断的患者中,8 名患者中有 5 名在妊娠或产后期间 MG 症状加重。4 名患者在妊娠或产后需要血浆置换或静脉注射免疫球蛋白进行抢救治疗。1 名患者因产程延长导致进展失败而行剖宫产。妊娠和分娩无其他并发症,所有婴儿均在分娩时健康。

讨论

与非 MuSK MG 一样,MMG 女性也可能在妊娠期间或产后 6 个月内出现病情加重或首次出现 MG 症状。可能需要对 MMG 妊娠患者进行更积极的医学治疗。需要进一步研究以确定 MMG 在妊娠或产后恶化的机制和风险。

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