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Immune Necrotizing Myopathy

Oct 26, 2020 4:31 pm

A full read review of immune-mediated necrotizing myopathy is portrayed as a distinct subtype of inflammatory myopathies first described in 2004.

This form of myositis has been associated with antibodies to signal recognition particle (anti-SRP) or anti-3-hydroxy-3-methylglutaryl-coA reductase (anti-HMGCR) myositis-specific autoantibodies, yet one in five with IMNM are seronegative. HMGCR and SRP autoantibodies are thought to play a role in the pathophysiology of IMNM and autoantibody-induced muscle damage appears to be complement dependent.

IMNM can be considered a muscle-specific autoimmune disease; patients with anti-SRP-positive IMNM are at an increased risk of myocarditis and patients with seronegative IMNM are at a major risk of associated malignancy.

Necrotizing myopathies are usually severe, rapidly progressive, and have high creatine kinase levels.  Prompt treatment is paramount as failure to treat IMNMs effectively may lead to severe muscle impairment. The article discusses about potential treatments including corticosteroids, immunosuppressants, rituximab, intravenous immunoglobulins, ands novel targeted therapies, such as anticomplement agents.

Disclosures
The author has no conflicts of interest to disclose related to this subject

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