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Rituximab in the Treatment of Refractory Anti-HMGCR Immune-mediated Necrotizing Myopathy
Océane Landon-Cardinal1,2,3, Yves Allenbach4,5,6, Antoine Soulages4,5,6
1From 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. o.landoncardinal@gmail.com.
Objective:
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.
Methods:
All patients who had been treated with RTX were retrospectively reviewed to assess features and outcome.
Results:
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.
Conclusion:
One-third of patients with refractory anti-HMGCR had improved strength or other evidence of improved disease activity following RTX treatment.
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