Abstract

A new subtype of myositis syndrome, immune-mediated necrotizing myopathy (IMNM), was initially explained in 2004. It manifests as symmetrical proximal muscular weakness in the extremities fairly quickly, with no obvious inflammatory infiltrate and significant muscle fiber necrosis and regeneration. According to the specific autoantibodies in patients, Anti-HMGCR+, anti-SRP+, and antibody-negative IMNM are the three subtypes of IMNM. which are characterized by subtle differences in pathogenesis, clinical manifestations, and therapeutic approaches. The aim of this review is to sort out most of the current studies on IMNM, to provide detailed explanations of their pathogenesis, diagnostic techniques and therapeutic approaches, and to summarize some of the protein molecules and structures that may play a key role in myopathogenesis. We hope that this will help to deepen the understanding of IMNM pathogenesis and optimize therapeutic strategies.

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