Rozanolixizumab as rescue therapy in triple-seronegative refractory generalized myasthenia gravis

Myasthenia gravis (MG) is an autoimmune disease affecting the neuromuscular junction, most commonly associated with anti-acetylcholine receptor antibodies, less frequently with muscle-specific kinase antibodies, and occasionally with low-density lipoprotein receptor-related protein 4 antibodies. However, a subset of patients with MG tests negative for all of these three antibodies - termed triple-seronegative MG - posing significant diagnostic and therapeutic challenges. Fast-acting therapies, such as intravenous methylprednisolone (IVMP), intravenous immunoglobulin (IVIG) and plasma exchange (PLEX), are typically recommended during disease exacerbation. However, some patients are refractory to these interventions. For refractory patients with triple-seronegative MG, the efficacy of a recent monoclonal antibody therapy as rescue therapy remains uncertain. We report a case of triple-seronegative MG refractory to repeated IVIG, IVMP and PLEX, in which clinical improvement was achieved following treatment with rozanolixizumab. This case, with long-term follow-up, suggests the potential utility of rozanolixizumab as rescue therapy in a patient subgroup which is typically excluded from randomized controlled trials. Larger studies specifically including patients with triple-seronegative MG are warranted.