Journal of Clinical Neuromuscular Disease. 15(4):167–178, JUNE 2014
DOI: 10.1097/CND.0000000000000034
,
PMID: 24872217
Issn Print: 1522-0443
Publication Date: June 2014
Treatment-Refractory Myasthenia Gravis
Nicholas Silvestri;Gil Wolfe;
+ Author Information
Department of Neurology, State University of New York, Buffalo School of Medicine and Biomedical Sciences, Buffalo General Medical Center, Buffalo, NY.
Abstract
Myasthenia gravis (MG) is the most common disorder of neuromuscular transmission and is a prototypical autoimmune disorder. Most patients with MG are successfully treated with acetylcholinesterase inhibitors, corticosteroids, and/or steroid sparing agents such as azathioprine and mycophenolate mofetil. There is a small subset of patients, however, with treatment-refractory disease. In these cases, medications such as rituximab, high-dose cyclophosphamide, and eculizumab may be used. Thymectomy (in some cases repeat thymectomy) is another option in selected patients. Studies evaluating these and other forms of therapy in treatment-refractory MG are reviewed.