Myasthenia gravis (MG) is a neuromuscular autoimmune disorder characterized by chronic but intermittent fatigue of the eye- and general body muscles. Muscle weakness is caused primarily by the binding of an autoantibody to the acetylcholine receptors, resulting in blockage of normal neuromuscular signal transmission. Inflammation has long been regarded as an important contributing factor for both generalized and ocular MG pathogenesis. The mediators of inflammation, predominantly cytokines and chemokines are released mostly by inflammatory monocytes and macrophages in the affected postsynaptic neuromuscular junction (NMJ) or thymic tissue which then move also into the peripheral circulation. Inflammatory mediators, play a major role in immune modulation. The presence of infiltrated macrophages and monocytes at the neuromuscular junction in MG. Local release of cytokines from these cells further recruits polymorpho neutrophils (PMN) and other immune cells that permeate into the specific site and start the inflammatory cascades for immune activation. Monotherapies targeting various inflammatory mediators (such as IL-6 or IL6-receptors) are currently active in clinical trials. Tocilizumab also known as atlizumab, is a recombinant humanized monoclonal antibody (mAb) against the membrane bound or soluble IL6 receptor. Tocilizumab treatment has shown clinical efficacy in MG patients refractory to rituximab and other autoimmune diseases. Future therapy approaches for MG may also assess the effectiveness of combination therapy targeting both components, autoantibody and inflammation, responsible for MG pathogenesis.
