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Necrotizing Autoimmune Myopathy

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Acquired Neuromuscular Disorders

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Abstract

Necrotizing autoimmune myopathy (NAM) is categorized as one of the idiopathic immune-mediated myopathies. It is characterized by subacute predominantly proximal weakness, elevated serum creatine kinase, and a muscle biopsy showing predominantly necrotic and regenerating fibers with absent or minimal inflammation. In over 50 % of cases, the weakness is severe, and about 40 % of patients have distal muscle weakness. Dyspnea and respiratory muscle weakness are common and can lead to some patients developing respiratory failure requiring intubation. Risk factors associated with NAM include the use of statin medications, neoplasm, and connective tissue disease. A subset of NAM patients have SRP-IgG and/or HMGCR-IgG antibodies, although neither of these antibodies are 100 % sensitive or specific. NAM patients are felt to be pharmacologically more treatment refractory than other immune-mediated myopathies and often require treatment with more than two immunotherapeutic agents. Commonly, patients are treated with a combination of corticosteroids, IVIG, and an oral steroid-sparing immunosuppressant agent (such as methotrexate or mycophenolate mofetil). Despite the severe weakness, the outcome can be favorable in over 50 % of cases, although many will have some residual weakness.

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Authors and Affiliations

  1. Division of Neurology, University of Toronto, St. Michael’s Hospital, 30 Bond Street, Toronto, ON, M5B 1W8, Canada

    Charles Kassardjian

  2. Department of Neurology, Mayo Clinic, 200 First Street SW, Rochester, MN, 55905, USA

    Margherita Milone

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  1. Charles Kassardjian
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  2. Margherita Milone
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Correspondence to Margherita Milone .

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Editors and Affiliations

  1. Neuromuscular Center, IRCCS Fondazione San Camillo Hospit, Venice, Italy

    Corrado Angelini

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Kassardjian, C., Milone, M. (2016). Necrotizing Autoimmune Myopathy. In: Angelini, C. (eds) Acquired Neuromuscular Disorders. Springer, Cham. https://doi.org/10.1007/978-3-319-29514-5_7

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  • DOI: https://doi.org/10.1007/978-3-319-29514-5_7

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