Curr Treat Options in Rheum (2017) 3:267–274 DOI 10.1007/s40674-017-0080-0 Other CTD: Inflammatory Myopathies and Sjogren's (P Basharat, Section Editor) Autoimmune Necrotizing Myopathies: Subtypes and Advances in Treatment Jason Liebowitz, MD 1 Lisa Christopher-Stine, MD, MPH 2,* Address 1 Johns Hopkins Hospital/Bayview Medical Center, 5200 Eastern Avenue Mason F. Lord Center Tower; Suite 4500, Baltimore, MD, 21224, USA *,2 Johns Hopkins Myositis Center, 5200 Eastern Avenue Mason F. Lord Center Tower; Suite 4500, Baltimore, MD, 21224, USA Email: lchrist4@jhmi.edu Published online: 7 November 2017 * Springer International Publishing AG 2017 This article is part of the Topical Collection on Other CTD: Inflammatory Myopathies and Sjogren’s Keywords Immune-mediated necrotizing myopathy I Autoimmune myopathy I Signal recognition particle (SRP) I 3-Hydroxy-3-methylglutaryl coenzyme A reductase (HMGCR) Opinion statement Purpose of Review Necrotizing autoimmune myopathies (NAM), or immune-mediated necrotizing myopathies (IMNM), are considered as a unique category of autoimmune myopathy given their distinctive histologic, serologic, and clinical features. The goals of this article are to review the subtypes of IMNM and to discuss treatment options that apply to these conditions. Recent findings Two thirds of cases of IMNM are associated with either autoantibodies recognizing the signal recognition particle (SRP) or with autoantibodies recognizing 3- hydroxy-3-methylglutaryl coenzyme A reductase (HMGCR). Corticosteroids remain first- line therapy in combination with other immunosuppressive treatments, including meth- otrexate, azathioprine, mycophenolate mofetil, cyclosporine, intravenous immunoglobu- lins, rituximab, cyclophosphamide, and plasmapheresis. Summary Though a great deal has been learned about IMNM, there are no standardized treatment protocols yet developed for these conditions and future advances in the field will benefit from randomized clinical trials in which the efficacies of selected therapies are tested in a rigorous manner. Introduction The concept of autoimmune necrotizing myopathies, or immune-mediated necrotizing myopathies, as a distinct category of disease is a relatively new concept. Using Bohan and Peter criteria from their landmark papers in