Fast-acting treatment of myasthenic crisis with efgartigimod from the perspective of the neonatal intensive care unit.

Abstract

Background: Myasthenic crisis (MC) refers to rapid deterioration of myasthenia gravis (MG), affecting lung and bulbar muscles and causing breathing difficulties. Currently, efgartigimod has shown good therapeutic effects in patients with generalized myasthenia gravis (GMG). This retrospective real-world study explored the effectiveness of efgartigimod in patients with MC.

Method: Reviewing the clinical data of five patients (including four patients with refractory MC) with MC who received efgartigimod at the First Affiliated Hospital of Sun Yat-sen University, all of these patients were admitted from September 2023 to December 2023.

Results: Each patient received 20 mg/kg of efgartigimod on the first and fifth day. After discharge, all patients showed a clinically meaningful decrease in Myasthenia Gravis Activities of Daily Living (MG-ADL) scale (a decrease of ≥ 2 points) and an improvement in their lung function. Additionally, all patients had a decrease in IgG levels (58.59 ± 18.48% after one cycle of efgartigimod). We also explored the ICU stay and mechanical ventilation (MV) duration for these five patients, and found no significant improvement compared to a large sample data. In terms of safety, four patients experienced adverse events (AEs), all of which were mild. At the last follow-up, four patients achieved the minimal symptom expression (MSE) status (an MG-ADL score of 0 or 1) after 6.25 ± 3.30 weeks. Only one patient experienced a worsening of symptoms in the second week after discharge, but she also achieved the MSE status after receiving a second cycle of efgartigimod treatment.

Conclusions: Given the conclusion that intravenous efgartigimod is a non-invasive fast-acting treatment with fewer AEs, this may provide NICU workers with another option for managing patients with MC.