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混合连接组织疾病相关的肌肉炎的临床病理特征:一个病例系列

Naohiro Sakamoto^1,2, Rumiko Izumi¹, Naoki Suzuki¹

¹Department of Neurology, Tohoku University Graduate School of Medicine, Sendai, Japan.

|January 29, 2025

概括

患有混合结缔组织疾病 (MCTD) 肌肉炎的患者经常经历非典型的肌肉衰弱,需要密集治疗. 肌肉病理在MCTD肌肉炎是异质的,表明皮肤肌炎和血管病的潜在同时发生.

科学领域:

类风湿病学类风湿病学
神经学神经学
病理学病理学病理学

背景情况:

混合结合组织疾病 (MCTD) 患者经常患有肌肉炎,但详细的临床和病理学数据有限.
之前的研究发现了各种神经病理发现,包括周围筋缩或周围血管炎症.
最近的观察表明,MCTD肌炎病理类似于免疫媒介性死肌病 (IMNM).

研究的目的:

描述MCTD患者肌肉炎的临床和肌病学特征.

主要方法:

对9名患有U1-RNP抗体,符合MCTD诊断标准的患者的临床和肌病学数据的分析.

主要成果:

8名患者表现出近端肢体的弱点;6名患者表现出非典型的弱点 (脸部,部,手腕,手指).
四名患者需要密集治疗 (IVIg或甲基prednisolone),反应良好,没有死亡.
肌肉活检显示了轻微的肌源性变化,死/再生纤维的增加和周围血管炎症透;两个被归类为皮肤肌炎,七个是不可分类的.

结论:

MCTD肌炎通常表现为轴向或非典型的弱点,需要密集治疗.
肌肉病理在MCTD肌肉炎是异质的,表明潜在的共存皮肤肌肉炎和血管病变.

关键词:

一个U1-RNP抗体.皮肤肌肉炎 (dermatomyositis) 是一种疾病.混合结合组织疾病 (MCTD)血管病变是一种血管病变.

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