Journal of Multiple Sclerosis and Neuroimmunology 12(1):1-5, 2021
Steroid-Unresponsive Immune-Mediated Necrotizing Myopathy Successfully Treated with Rituximab
Jong-Su Kim, MDa , SeonDeuk Kim, MDa , Woohee Ju, MDa , Soo Hyun Park, MD, PhDb,c, Je-Young Shin, MDa , Jung-Joon Sung, MD, PhDa , Seok-Jin Choi, MDa,d
Department of Neurology, Seoul National University Hospital, Seoul, Koreaa Department of Neurology, Inha University Hospital, Incheon, Koreab Department of Critical Care Medicine, Inha University Hospital, Incheon, Koreac Biomedical Research Institute, Seoul National University Hospital, Seoul, Koread
Immune-mediated necrotizing myopathy (IMNM) is a group of inflammatory myopathies
characterized by pathological findings including many necrotic muscle fibers and sparse
infiltration of inflammatory cells. Although about 20% of patients with IMNM remain
seronegative, most IMNMs are associated with anti-signal recognition particle or anti-3-hydroxy3-methylglutaryl-CoA reductase autoantibodies. Therapeutically, the mainstay of treatment
of IMNM is corticosteroid, and intravenous immunoglobulin and rituximab have emerged as
second-line therapy. Herein, we describe a rare case of steroid-resistant IMNM successfully
treated with rituximab.
Journal of Multiple Sclerosis and Neuroimmunology 12(1):1-5, 2021