Myasthenia Gravis

Abstract

Treatment of patients with acquired (autoimmune) myasthenia gravis should rely on evidence-based therapeutic choices, taking into account the individual's needs according to disease severity (mild to severe), extent (ocular or generalized), comorbidities (including other autoimmune diseases, infections, thymoma, and pregnancy), age, iatrogenic factors (the risks and benefits of therapy), patient autonomy and quality of life, financial burden to the patient, and associated health care costs. Therapy is aimed at managing symptoms by improving neuromuscular junction transmission (cholinesterase inhibitors) and/or modifying the underlying immunopathogenetic cause of acquired myasthenia gravis via immunosuppression or immunomodulation. Myasthenic patients with operable thymoma should be referred for surgery and closely followed up for tumor recurrence. A concerted international effort is addressing treatment recommendations for thymectomy in myasthenic patients with no radiologic evidence of thymoma who are positive for circulating acetylcholine receptor antibodies. There is a lack of evidence-based treatment guidelines for both acute and long-term management of ocular myasthenia. Acute management of myasthenic crisis requires intensive monitoring of the patient and institution of an efficient and safe treatment such as plasma exchange. Patient education is essential to a comprehensive long-term treatment plan.

A randomized and controlled study comparing immunoadsorption and plasma exchange in myasthenic crisis

“…In the IA group, one patient experienced a severe tachycardia with atrial fibrillation. In the PE group, six severe adverse advents occurred: bradycardia with shock (2), hematoma (2), tachycardia (1), hypersalivation with worsening of respiratory function (1) and dyspnea (1). In one patient the SAE (haematoma related to the central venous access) led to termination of the study, in all other SAE's symptoms subsequently improved or completely disappeared.…”

“…Myasthenia gravis (MG) is an organ specific, autoimmune disease caused by circulating autoantibodies against components of the nicotinic acetylcholine receptor (AChR) on the postsynaptic membrane at neuromuscular junctions [1]. Failure of neuromuscular transmission resulting in fluctuating and fatigable weakness of the peripheral, bulbar, and ocular muscles with remissions and exacerbations is a hallmark of MG [2,3].…”

“…Long-term treatment consists of acetylcholinesterase inhibitors, steroids, and other immunosuppressive drugs or immunomodulating therapies like intravenous immunoglobulins, and surgical thymectomy [1][2][3]17]. Interventional strategies for the rapid elimination of pathogenic antibodies can be offered by therapeutic apheresis [e.g., plasma exchange (PE) or immunoadsorption (IA)] [17,18].…”

An update on myasthenia gravis, challenging disease for the dental profession

“…Prednisone and other immunosuppressive agents such as azathioprine are widely employed in the treatment of MG, often with good results (61). However, corticosteroids and immunosuppressants may have an unfavorable effect on the immune system's efficiency (61).…”

“…However, corticosteroids and immunosuppressants may have an unfavorable effect on the immune system's efficiency (61). Furthermore, both thymic follicular hyperplasia and thymomas are associated with complex dysregulation of the immune system due to dysfunctional T cells and a cytokinerelated pro-inflammatory environment (62,63).…”