Recent advances in the understanding and therapy of myasthenia gravis

Abstract

Myasthenia gravis (MG) is a T-cell dependent autoimmune disease mediated by autoantibodies, which mainly target muscle nicotinic acetylcholine receptors (AChR) and cause loss of functional AChRs in the neuromuscular junction. Both MG and its major autoantigen are studied extensively, yet the etiology of the disease remains unclear, although it is known to be associated with the thymus. A genetic predisposition, combined with several unidentified environmental stimuli, likely creates a favorable milieu in which the disease can appear. Current research focusses on elucidating the cellular and molecular pathways of immune dysregulation, which underly MG outburst and progression. Considerable progress has been made concerning the involvement of the thymus, the identification of impaired mechanisms of immune control and the B–T-cell interaction in MG pathogenesis, while the role of chemokines arises as an intriguing new puzzle. Recent findings fueled the development of novel therapeutic approaches with some encouraging, although preliminary, results. This review summarizes recent achievements in the fields of both basic research and therapeutics.