Abstract：
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.
摘要：
我们在此报告了两名抗肌肉特异性激酶（MuSK）抗体阳性的重症肌无力患者，他们经历了无力的快速恶化。尤其是呼吸肌无力，需要无创正压通气（NIPPV），并用efgartigimod治疗。治疗开始后，观察到IgG水平快速降低和临床症状恢复.首次输注efgartigimod后两到三周不再需要NIPPV。这些发现表明，在重症肌无力患者中，使用efgartigimod降低IgG水平是抗MuSK抗体阳性的良好治疗选择，甚至在疾病的急性期。
