Julien, S.; Challier, I.; Malleter, M.; Jouen, F.; Drouot, L.; Boyer, O. Immune-Mediated Necrotizing Myopathy (IMNM): A Story of Antibodies. Antibodies2024, 13, 12.
Julien, S.; Challier, I.; Malleter, M.; Jouen, F.; Drouot, L.; Boyer, O. Immune-Mediated Necrotizing Myopathy (IMNM): A Story of Antibodies. Antibodies 2024, 13, 12.
Julien, S.; Challier, I.; Malleter, M.; Jouen, F.; Drouot, L.; Boyer, O. Immune-Mediated Necrotizing Myopathy (IMNM): A Story of Antibodies. Antibodies2024, 13, 12.
Julien, S.; Challier, I.; Malleter, M.; Jouen, F.; Drouot, L.; Boyer, O. Immune-Mediated Necrotizing Myopathy (IMNM): A Story of Antibodies. Antibodies 2024, 13, 12.
Abstract
Immune-mediated necrotizing myopathy (IMNM) is a rare and severe disease that corresponds to a specific entity of idiopathic inflammatory myopathy. Patients with IMNM suffer from proximal muscle weakness, and present high levels of creatine kinase and necrotic myofibers. Anti-Signal Recognition Particle (SRP) and anti-3-hydroxy-3-methylglutaryl-coenzyme A reductase autoantibodies (HMGCR) have recently been identified in two thirds of patients with IMNM and are used as a hallmark of the disease. In this review, we provide a detailed description of these antibodies and the tests used to detect them in the serum of patients. Based on in vitro studies and mouse models of IMNM, we will discuss the role of autoantibodies in the pathogenesis of the disease. Finally, in the light of the latest knowledge, we will conclude with a review of recent therapeutic approaches.
Copyright:
This is an open access article distributed under the Creative Commons Attribution License which permits unrestricted use, distribution, and reproduction in any medium, provided the original work is properly cited.
