First line treatment with subcutaneous efgartigimod in impending myasthenic crisis: a case report

Abstract

In acetylcholine receptor (AChR) antibody-positive generalized myasthenia gravis (gMG), neonatal Fc-receptor (FcRn) inhibition has broadened the therapeutic spectrum. Myasthenic crisis (MC), heralded by an impending myasthenic crisis (iMC), is a critical condition requiring treatments with rapid onset and sustained efficacy. Currently treatments used for iMC, including intravenous immunoglobulins and plasma exchange/immunoadsorption, have limitations, such as delayed onset of action and potential side effects. So far, there is limited data on the use of FcRn inhibitors in the management of impending or manifest MC (mMC). Here, we present a case of AChR antibody-positive gMG with iMC, where subcutaneous administration of the FcRn inhibitor efgartigimod resulted in rapid clinical remission. Within 24 h of administration, the patient exhibited significant improvement in respiratory and bulbar muscle function, preventing progression to manifest MC and the need for mechanical ventilation. This rapid response was accompanied by a marked reduction in AChR antibody level by 89.8% within 4 weeks. This case supports the potential of efgartigimod as a fast-acting and effective treatment option for managing iMC, offering an alternative to existing therapies.

Keywords: FcRn inhibition; case report; efgartigimod; myasthenia gravis; myasthenic crisis.

Figure 1.

Time course after efgartigimod subcutaneous administration. (a) Clinical course: myasthenia Gravis-Quality of Life 15, MG-QoL15; quantitative myasthenia gravis score, QMG; myasthenia gravis-specific activities of daily living scale, MG-ADL and (b) Serum levels of acetylcholine receptor (AChR)-antibodies and immunoglobuline G (IgG).