Idiopathic inflammatory myopathies (IIM) are a group of rare autoimmune disorders characterized by muscle inflammation and progressive weakness. The cause of IIM is unclear but it is believed that disease expression may be triggered by unknown factors in genetically predisposed individuals. Diagnosis is based on a combination of clinical, laboratory and electromyography findings. Muscle biopsy is the definitive diagnostic test. Research into IIM has been limited by the rarity of the disease, a somewhat insidious onset, difficulties with classification and diagnostic methods and heterogeneous study populations making cross-study evaluations difficult. This paper reviews the diagnostic and classification criteria of the IIM and examines epidemiological studies that have been performed, focusing on demographics.
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This 30-color panel was developed to enable the enumeration and purification of distinct circulating immune cell subsets implicated in the pathogenesis of systemic autoimmune diseases including rheumatoid arthritis (RA), systemic lupus erythematosus (SLE), systemic sclerosis (SSc; scleroderma), Sjögren's disease (SjD), idiopathic inflammatory myopathy (IIM), and others. While designed for application to peripheral blood mononuclear cells, the inclusion of CD45 coupled with the ability to extract cellular autofluorescence spectral signatures enables the application of this panel to other tissue types. Of the 30 total markers, this panel employs 18 markers to profile T cell subsets consisting of different memory subsets and T helper polarities, > 10 markers to profile B cell subsets including double-negative B cells, and a total of 8 lineage markers to identify immune lineages including monocyte and natural killer cell subsets, conventional dendritic cells, plasmacytoid dendritic cells, and basophils.
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