Tanaka Koya, Yoshikawa Masaaki, Inoue Yukako, Tsumura Keisuke, Hoshino Yuki, Shichijo Chika, Ide Toshihiro, Suzuyama Kohei, Koike Haruki
Division of Neurology, Department of Internal Medicine, Faculty of Medicine, Saga University, Japan.
Intern Med. 2025 Mar 15;64(6):925-929. doi: 10.2169/internalmedicine.3726-24. Epub 2024 Aug 8.
We herein report two patients with anti-muscle-specific kinase (MuSK) antibody-positive myasthenia gravis who experienced rapid deterioration of weakness, particularly respiratory muscle weakness, necessitating non-invasive positive pressure ventilation (NIPPV) and were treated with efgartigimod. After treatment initiation, a rapid reduction in IgG levels and recovery from clinical symptoms were observed. NIPPV was no longer required two to three weeks after the first infusion of efgartigimod. These findings suggest that the reduction of IgG levels using efgartigimod is a good treatment option in patients with myasthenia gravis positive for anti-MuSK antibodies, even during the acute phase of the disease.
我们在此报告两名抗肌肉特异性激酶(MuSK)抗体阳性的重症肌无力患者,他们出现了肌无力的快速恶化,尤其是呼吸肌无力,需要无创正压通气(NIPPV),并接受了艾加莫德治疗。开始治疗后,观察到IgG水平迅速降低且临床症状有所恢复。首次输注艾加莫德两到三周后,不再需要NIPPV。这些发现表明,即使在疾病急性期,使用艾加莫德降低IgG水平对于抗MuSK抗体阳性的重症肌无力患者也是一种很好的治疗选择。