Expert Clinical Perspectives: Management of immune-mediated necrotizing myopathy-Article

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The article discusses the management of immune-mediated necrotizing myopathy (IMNM), a rare autoimmune myopathy characterized by proximal muscle weakness and elevated creatine kinase levels. Different subtypes of IMNM are associated with specific autoantibodies and triggers such as statin use or cancer. Immunotherapy is the main treatment approach, although no therapies are currently FDA-approved for IMNM. Observational studies suggest that certain therapies may be more effective for different serological subtypes. HMGCR IMNM often responds well to intravenous immunoglobulin (IVIG), while SRP and seronegative IMNM typically require combination immunotherapy. Treatment strategies may involve corticosteroids, immunosuppressants, IVIG, and rituximab.<br /><br />Patients with HMGCR IMNM should discontinue statin therapy and consider IVIG as first-line treatment. For SRP IMNM, aggressive therapy with corticosteroids, IVIG, and immunosuppressants is usually initiated, with rituximab added for severe cases. Patients with seronegative IMNM may also require combination therapy and monitoring for potential malignancy. Long-term immunosuppression management includes PJP prophylaxis and osteoporosis prevention.<br /><br />The article emphasizes the importance of early treatment to prevent irreversible muscle damage, as well as the need for further research to guide optimal management strategies for IMNM. It provides insights into the clinical features, treatment approaches, and considerations for patients on long-term immunosuppression. Additional therapies such as physical therapy and speech language pathology are also highlighted for supportive care in managing IMNM.

Keywords

immune-mediated necrotizing myopathy

IMNM

autoimmune myopathy

proximal muscle weakness

elevated creatine kinase levels

specific autoantibodies

statin use

cancer

immunotherapy

HMGCR IMNM