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Expert Clinical Perspectives: Management of immune-mediated necrotizing myopathy
Expert Clinical Perspectives: Management of immune-mediated necrotizing myopathy
Abstract: 
The immune-mediated necrotizing myopathies (IMNM) are autoimmune myositides clinically characterized by proximal predominant weakness and elevated creatine kinase (CK). They may be associated with autoantibodies (anti-HMGCR, anti-SRP), triggered by statin use (e.g., anti-HMGCR myopathy), associated with cancer, or may be idiopathic. Immunotherapy is required to improve strength and decrease the CK level, but no therapies are currently approved by the U.S. Food and Drug Administration for the treatment of IMNM. The optimal treatment strategy for IMNM is currently unknown and wide practice variation exists in the management of this condition. However, observational studies and expert opinion suggest that certain therapies may be more effective for the different serological subtypes of IMNM. HMGCR IMNM often responds favorably to intravenous immunoglobulin (IVIG) even as monotherapy. Signal recognition peptide and seronegative IMNM typically require combination immunotherapy, most often consisting of an oral immunosuppressant, corticosteroids, and IVIG or rituximab. Patients often remain on immunotherapy for years and relapse is common during tapering of immunotherapy. Further studies are needed to guide the optimal management of these patients.

The objectives of this activity are to: 1) Be able to initiate first and second-line treatments for HMGCR immune-mediated necrotizing myopathy; 2) Be able to initiate first and second-line treatments for SRP immune-mediated necrotizing myopathy; 3) Be able to initiate first and second-line treatments for seronegative immune-mediated necrotizing myopathy.


ACCREDITATION STATEMENT
The AANEM is accredited by the Accreditation Council for Continuing Medical Education (ACCME) to provide continuing medical education for physicians.

DISCLOSURE INFORMATION
Joome Suh: Advisory boards for Argenx, Alexion, UCB, research support from Alexion. Anthony A. Amato: Medical Advisory Boards/
Consultant for Argenx, Ra Pharmaceuticals, Horizon Therapeutics, OnoPharma, Alexion, EMD Serono, Takeda, Johnson & Johnson
(COVID-19 vaccination program). Conflicts have been resolved according to ACCME guidelines. 

CREDIT DESIGNATION
The AANEM is accredited by the American Council for Continuing Medical Education (ACCME) to providing continuing education for physicians. AANEM designates this Journal-based CME activity for a maximum of 1.0 AMA PRA Category 1 Credit™. Physicians should claim only the credit commensurate with the extent of their participation in the activity.  
Author
Joome Suh, MD; Anthony A. Amato, MD.
Summary
Availability: On-Demand
Expires on May 24, 2027
Cost: Member: $0.00
Non-Member: $15.00
Credit Offered:
1 CME Credit
1 CEU Credit


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