Conventional and emerging treatments and controversies in myasthenia gravis

Abstract

Introduction: Myasthenia gravis (MG) is caused by IgG antibodies against different proteins at the neuromuscular junction. Anti-acetylcholine receptor (AChR) Abs are detected in the great majority of patients. MG management consists of long-term immunotherapy, based on steroids and immunosuppressants, short-term treatments and therapeutic thymectomy. Targeted immunotherapies that reduce B cell survival, inhibit complement activation, and decrease serum IgG concentration have been evaluated in trials and have entered clinical practice.

Areas covered: Herein, the efficacy and safety data of conventional and novel therapeutic options are reviewed and their indications in the disease subtypes are discussed.

Expert opinion: Even though conventional treatment is generally effective, 10-15% of patients have refractory disease and there are safety concerns related to long-term immunosuppression. Novel therapeutic options offer several advantages but also have limitations. Safety data based on long-term treatment are not yet available for some of these agents. The mechanisms of action of new drugs and the immunopathogenesis of different MG subtypes must be considered in therapy decision making. Integrating new agents in the treatment scenario of MG can significantly improve disease management.

Keywords: B cell targeting agents; FcRn blockers; Myasthenia gravis; complement inhibitors; immunosuppressive therapy.