Abstract
Idiopathic inflammatory myopathies (IIMs) are a group of autoimmune muscle diseases with significant morbidity and mortality. This review details and updates the pathogenesis and emerging importance of myositis-specific antibodies in the development of IIMs. An increase in the understanding of how these myositis-specific antibodies play a role in IIMs has led to the further categorization of IIMs from the traditional polymyositis versus dermatomyositis, to additional subcategories of IIMs such as necrotizing autoimmune myositis (NAM). The diagnosis of IIMs, including manual muscle testing, laboratory studies, and non-invasive imaging have become important in classifying IIM subtypes and for identifying disease severity. Treatment has evolved from an era where glucocorticoid therapy was the only option to a time now that includes traditional steroid-sparing agents along with immunoglobulin therapy and biologics, such as rituximab.
Highlights
Idiopathic inflammatory myopathies (IIMs) are a group of autoimmune muscle diseases with significant morbidity and mortality if not properly addressed during a clinical evaluation early on in the disease course
Additional attempts have been made by multiple investigative groups to further classify the pathophysiology of IIMs in order to better diagnose and predict which groups of patients have an increased likelihood for the development of malignancy, interstitial lung disease (ILD), and other co-morbidities associated with IIMs
Polymyositis is increasingly being recognized as a bucket term for IIMs that do not have the specific criteria for placement into the other four subcategories or which are negative for myositis-specific antibody
Summary
Idiopathic inflammatory myopathies (IIMs) are a group of autoimmune muscle diseases with significant morbidity and mortality if not properly addressed during a clinical evaluation early on in the disease course. This group of myopathies has long been classified based on clinical criteria created by Bohan and Peter in 1975. Additional attempts have been made by multiple investigative groups to further classify the pathophysiology of IIMs in order to better diagnose and predict which groups of patients have an increased likelihood for the development of malignancy, interstitial lung disease (ILD), and other co-morbidities associated with IIMs. In that respect, the treatment of inflammatory myopathy is changing, once only consisting of treatment with high-dose glucocorticoids to include traditional immunosuppressive steroid-sparing immunotherapy as well as immunoglobulin and biologic agents, such as rituximab infusion. The exciting science associated with uncovering the pathophysiological underpinnings of IIMs has become more intricate, and treatments have expanded over years of research, with the findings of myositis-specific antibodies
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