Efficacy of intravenous immunoglobulin therapy in acute phase of myasthenia gravis in children

Abstract

  Objective: We investigated the efficacy of intravenous immunoglobulin (IVIG) therapy in acute phase of myasthenia gravis (MG) in children and the characteristics of patients for whom IVIG was effective. Methods: The study included 17 patients (6 boys) (8 children with ocular and 9 with generalized MG) aged ＜15 years who were diagnosed with MG, and received more than 1g/kg of IVIG therapy in acute phase between April 1, 2010 and March 31, 2022. Children in whom the MG activities of daily living score improved by ≥1 within 14 days after IVIG therapy commencement were considered to show a successful response to therapy Those in whom IVIG therapy was effective underwent post-treatment follow-up. Results: Median age at disease onset was 6.0 (0.9-13.9) years, and IVIG therapy in acute phase was administered at median age 6.9 (1.1-14.1) years. IVIG therapy was effective in 8 patients, with significantly more generalized MG in the effective cases (p=0.02). Among patients in whom IVIG therapy was effective, one patient achieved complete remission without medication for ＞2 years. Complete remission without medication for ＞2 years after treatment completion was achieved in four patients who responded to IVIG therapy in acute phase and received maintenance IVIG therapy. Conclusions: The efficacy of IVIG therapy in acute phase was higher in children with generalized MG than in those with ocular MG. When IVIG therapy in acute phase is effective, the use of steroids may be avoided or reduced by shifting to maintenance IVIG therapy.