血管性肌肉病:临床特征和长期结果
Marcus V Pinto1, Kenneth J Warrington1, Pannathat Soontrapa1
1From the Department of Neurology (M.V.P., P.S., E.N.), and Division of Rheumatology (K.J.W., M.J.K.), Department of Internal Medicine, Mayo Clinic, Rochester, MN.
Neurology
|November 25, 2024
概括
血管性肌肉病常常呈现为最初的弱点,具有正常的肌酸激酶,但高的阿尔多酶. 大多数患有血管性肌肉病变的患者对免疫疗法反应良好,显示出良好的生存率.
科学领域:
- 神经学 神经学
- 类风湿病学 类风湿病学
- 病理学 病理学 病理学
背景情况:
- 周围神经病变是一种常见的血管炎表现,但血管性肌肉病变的理解较少.
- 关于血管性肌肉病的临床,实验室和病理特征的文献有限.
研究的目的:
- 描述血管性肌肉病变的临床,实验室和病理学特征.
- 为了评估患有血管性肌肉病的患者的治疗结果.
主要方法:
- 对被诊断患有血管炎和肌肉病症的患者 (1980-2022) 的回顾性图表审查.
- 纳入标准:经活检证明的血管性肌肉病变或经活检证明的血管炎与同时出现的活性肌肉病变 (不包括其他原因).
- 肌肉活检审查与免疫组织化学.
主要成果:
- 确定了25名患者;60%是女性,中位数年龄为63. 肌病是80%的初始表现.
- 靠近对称的弱点和疼痛是常见的. 肌酸激酶 (CK) 通常是正常的,阿尔多酶升高. 电磁共振显示了肌病变化和神经病变.
- 肌肉活检显示出周周血管炎症,血管破坏和亡. 免疫疗法导致24/25患者的病情改善,复发率低.
结论:
- 血管性肌病变可能是系统性血管炎或非系统性形式的初始迹象.
- 呈现通常涉及近位弱点,正常的CK和升高的阿尔多酶.
- 患者通常对免疫疗法反应良好,生存概率有利.
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