Efgartigimod for patients with thymoma associated generalized myasthenia gravis during the perioperative period: a four-case report.

Abstract

Background: Thymectomy is one of the main treatments for thymoma associated myasthenia gravis, but there is a risk of acute exacerbation of myasthenia gravis symptoms during the perioperative period of thymoma. Therefore, perioperative management is very important. We reported four cases of thymoma associated generalized myasthenia gravis who were treated with efgartigimod before and after the perioperative period. The myasthenic symptoms of the patients were quickly controlled before surgery, and the patients successfully completed the surgery without experiencing myasthenic crisis or respiratory failure after surgery.

Case report: Four patients diagnosed with thymoma associated generalized myasthenia gravis were all acetylcholine receptor(AChR) antibody-positive, with case 2 also positive for Titin and anti-RyR antibodies. Prior to surgery, all patients exhibited varying degrees of myasthenic symptoms, with case 2 experiencing a myasthenic crisis induced by glucocorticoid pulse therapy before the operation. Admitted to our multidisciplinary treatment group for myasthenia gravis, all four patients received 1 to 2 treatments with efgartigimod before thymoma surgery, which led to an improvement in myasthenia symptoms and a significant reduction in MG-ADL and QMG scores. All four patients underwent video-assisted thoracoscopic mediastinal tumor resection under general anesthesia, followed by the 3rd to 4th treatments with efgartigimod postoperatively, with no perioperative myasthenic crisis occurring in any of the patients. Apart from case 1, who developed a new pulmonary infection postoperatively and experienced a temporary fluctuation in myasthenia symptoms, the other three patients had no severe complications. Follow-up visits at one month and three months postoperatively showed that three of the patients achieved minimum symptom expression (MG-ADL score ≤1).

Discussion: Efgartigimod provides new insights into the perioperative management of thymomas in patients with thymoma associated generalized myasthenia gravis.