난치중증근무력증 환자의 치료에 대한 권고합의안

Abstract

Background Despite advances in treatment, 10-15% of the patients with myasthenia gravis (MG) are refractory to convention therapies, have severe disease, and are more likely to experience frequent clinical exacerbation. Although various new treatments have been developed, the evidence regarding their applicability to refractory MG remains unclear. Additionally, most of these new treatments are not yet available in Korea. Thus, we aimed to develop the consensus guidance regarding the management of patients with refractory MG. Methods The Korean Society of Neuroimmunology formed a task force consisting of 11 experts and adopted the RAND/UCLA appropriateness methodology to develop national consensus guidance for management of refractory MG. The standard therapy for MG was first defined. Following a thorough review of the literature, initial guidance statements on five key topics were developed. Two rounds of anonymous voting were conducted to reach consensus on the guidance statements, with modifications made based on panel input. Results Comprehensive guidance statements were developed covering definition, treatment goal and treatment of refractory MG. In addition, specific treatment strategies for acetylcholine receptor antibody-positive MG and muscle-specific tyrosine kinase antibody-positive MG were covered. Conclusion This guidance represents a national consensus of experts and was developed as a valuable resource for clinicians managing Korean adult patients with refractory MG.