From the Department of Internal Medicine and Clinical Immunology and Inflammation-Immunopathology-Biotherapy Department (I2B), Pitié-Salpêtrière University Hospital, AP-HP, East Paris Neuromuscular Diseases Reference Center, Inserm U974, Sorbonne Université, Paris, France; Center of Reference for Neuromuscular Disorders AOC, Department of Neurology, Bordeaux University Hospital, Bordeaux, France; Polyvalent and Oncologic Radiology Department, Musculoskeletal Unit, Pitié-Salpêtrière University Hospital, AP-HP, Paris, France.
Dr. Landon-Cardinal is the recipient of Clinical Fellowship awards from the Université de Montréal Rheumatology Program, Abbvie Educational Grant and the Association des médecins rhumatologues du Québec, Visithan-Khy Educational Grant.
J Rheumatol. 2019 Jun;46(6):623-627. doi: 10.3899/jrheum.171495. Epub 2018 Dec 15.
A pathogenic role of anti-3-hydroxy-3-methylglutaryl-coenzyme A reductase (anti-HMGCR) antibodies has been proposed. Our objective was to assess efficacy of rituximab (RTX) in anti-HMGCR immune-mediated necrotizing myopathy.
All patients who had been treated with RTX were retrospectively reviewed to assess features and outcome.
Three of 9 patients demonstrated stable or improved muscle strength ± decline in creatine kinase levels, or T2/short-tau inversion recovery hypersignal decrease on magnetic resonance imaging following RTX treatment. RTX permitted intravenous immunoglobulin discontinuation and corticosteroid reduction to low dose in 2 patients.
One-third of patients with refractory anti-HMGCR had improved strength or other evidence of improved disease activity following RTX treatment.
有人提出了抗 3-羟基-3-甲基戊二酰辅酶 A 还原酶（抗-HMGCR）抗体的致病作用。我们的目的是评估利妥昔单抗（RTX）在抗-HMGCR 免疫介导的坏死性肌病中的疗效。
回顾性分析了所有接受 RTX 治疗的患者，以评估其特征和结局。
9 例患者中有 3 例在 RTX 治疗后肌肉力量稳定或改善±肌酸激酶水平下降，或磁共振成像上 T2/短 tau 反转恢复高信号减少。RTX 使 2 例患者能够停用静脉注射免疫球蛋白和减少皮质类固醇用量至低剂量。
三分之一的难治性抗-HMGCR 患者在接受 RTX 治疗后肌肉力量增强或其他疾病活动改善的证据。
