INTRODUCTION: Myasthenia gravis, an autoimmune disorder of neuromuscular transmission, is treated by an array of immunomodulating therapies. A variable response is observed with certain patients being medically refractory. METHODS: We report the results of 14 refractory generalized myasthenia gravis patients (6 AChR+; 8 MuSK+) treated with rituximab. RESULTS: Sustained clinical improvement was observed in all patients as well as a reduction of conventional immunotherapies. Prednisone dose decreased a mean of 65.1%, 85.7%, and 93.8% after cycle 1, 2, and 3 of rituximab therapy, respectively. A statistically significant reduction in plasma exchange sessions was seen after cycle 1 with all patients being off of plasma exchange after cycle 3. Acetylcholine receptor antibody titers decreased a mean of 52.1% (p = 0.0046) post-cycle 2. CONCLUSION: Our results support the hypothesis that rituximab is beneficial and well tolerated in managing refractory myasthenia gravis and nearly doubles published cases. We propose that B-cell-directed therapies may become an attractive option and suggest pursuit of a prospective trial.
INTRODUCTION:Myasthenia gravis, an autoimmune disorder of neuromuscular transmission, is treated by an array of immunomodulating therapies. A variable response is observed with certain patients being medically refractory. METHODS: We report the results of 14 refractory generalized myasthenia gravispatients (6 AChR+; 8 MuSK+) treated with rituximab. RESULTS: Sustained clinical improvement was observed in all patients as well as a reduction of conventional immunotherapies. Prednisone dose decreased a mean of 65.1%, 85.7%, and 93.8% after cycle 1, 2, and 3 of rituximab therapy, respectively. A statistically significant reduction in plasma exchange sessions was seen after cycle 1 with all patients being off of plasma exchange after cycle 3. Acetylcholine receptor antibody titers decreased a mean of 52.1% (p = 0.0046) post-cycle 2. CONCLUSION: Our results support the hypothesis that rituximab is beneficial and well tolerated in managing refractory myasthenia gravis and nearly doubles published cases. We propose that B-cell-directed therapies may become an attractive option and suggest pursuit of a prospective trial.
Authors: Jonathan C W Edwards; Leszek Szczepanski; Jacek Szechinski; Anna Filipowicz-Sosnowska; Paul Emery; David R Close; Randall M Stevens; Tim Shaw Journal: N Engl J Med Date: 2004-06-17 Impact factor: 91.245
Authors: Jason A Vander Heiden; Panos Stathopoulos; Julian Q Zhou; Luan Chen; Tamara J Gilbert; Christopher R Bolen; Richard J Barohn; Mazen M Dimachkie; Emma Ciafaloni; Teresa J Broering; Francois Vigneault; Richard J Nowak; Steven H Kleinstein; Kevin C O'Connor Journal: J Immunol Date: 2017-01-13 Impact factor: 5.422
Authors: John S Yi; Jeffrey T Guptill; Panos Stathopoulos; Richard J Nowak; Kevin C O'Connor Journal: Muscle Nerve Date: 2017-09-30 Impact factor: 3.217
Authors: Raffi Topakian; Fritz Zimprich; Stephan Iglseder; Norbert Embacher; Michael Guger; Karl Stieglbauer; Dieter Langenscheidt; Jakob Rath; Stefan Quasthoff; Philipp Simschitz; Julia Wanschitz; David Windisch; Petra Müller; Dierk Oel; Günther Schustereder; Stefan Einsiedler; Christian Eggers; Wolfgang Löscher Journal: J Neurol Date: 2019-01-16 Impact factor: 4.849
Authors: Ruoyi Jiang; Kenneth B Hoehn; Casey S Lee; Minh C Pham; Robert J Homer; Frank C Detterbeck; Inmaculada Aban; Leslie Jacobson; Angela Vincent; Richard J Nowak; Henry J Kaminski; Steven H Kleinstein; Kevin C O'Connor Journal: Proc Natl Acad Sci U S A Date: 2020-11-16 Impact factor: 11.205