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Curr Rheumatol Rep. 2015 Dec;17(12):72. doi: 10.1007/s11926-015-0548-6.

Immune-Mediated Necrotizing Myopathy: Update on Diagnosis and Management.

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Division of Rheumatology, Department of Medicine, The University of Western Ontario, 268 Grosvenor Street, London, ON, Canada, N6A 4V2.
Johns Hopkins Myositis Center, The Johns Hopkins University School of Medicine, 5200 Eastern Avenue, Mason F. Lord Center Tower; Suite 4500, Baltimore, MD, 21224, USA.


The idiopathic inflammatory myopathies (IIMs) comprise a group of autoimmune disorders that target skeletal muscle. They are characterized by typical laboratory and clinical features including muscle weakness, elevated muscle enzymes, characteristic histopathology of muscle biopsies, as well as electromyography abnormalities. The IIMs are divided into polymyositis, dermatomyositis, inclusion body myositis, nonspecific myositis, and immune-mediated necrotizing myopathy (IMNM). IMNM is distinguished by the absence of primary inflammation on muscle biopsy. IMNM may be associated with myositis-specific autoantibodies (i.e., anti-SRP and anti-HMGCR) and malignancy, in association with viral infections (HIV or hepatitis C), or in relation to other connective tissue diseases (i.e., scleroderma). Typical clinical findings such as severe muscle weakness, highly elevated creatine kinase (CK) levels, as well as resistance to conventional immunosuppressive therapy are associated with this subtype of IIM. This review provides an overview of this disease entity and focuses on its diagnosis and treatment.


Immune-mediated necrotizing myopathy; Myositis; Statin-induced myopathy; anti-HMGCR; anti-SRP

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