Steroid-Unresponsive Immune-Mediated Necrotizing Myopathy Successfully Treated with Rituximab

Kim, Jong-Su; Kim, SeonDeuk; Ju, Woohee; Park, Soo Hyun; Shin, Je-Young; Sung, Jung-Joon; Choi, Seok-Jin

J Mult Scler Neuroimmunol > Volume 12(1); 2021 > Article

Case Report

Journal of Multiple Sclerosis and Neuroimmunology 2021;12(1):1-5.
Published online January 1, 2021.

리툭시맙 투여 후 호전된 스테로이드 불응성 면역매개괴사근육병 증례

김종수^a, 김선득^a, 주우희^a, 박수현^b,c, 신제영^a, 성정준^a, 최석진^a,d

^a서울대학교병원 신경과
^b인하대학교의과대학부속병원 신경과
^c인하대학교의과대학부속병원 중환자의학과

Steroid-Unresponsive Immune-Mediated Necrotizing Myopathy Successfully Treated with Rituximab

Jong-Su Kim^a, SeonDeuk Kim^a, Woohee Ju^a, Soo Hyun Park^b,c, Je-Young Shin^a, Jung-Joon Sung^a, Seok-Jin Choi^a,d

^aDepartment of Neurology, Seoul National University Hospital, Seoul, Korea
^bDepartment of Neurology, Inha University Hospital, Incheon, Korea
^cDepartment of Critical Care Medicine, Inha University Hospital, Incheon, Korea

Abstract

"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"

Key Words: Key Words: Myositis, Steroids, Rituximab