Idiopathic inflammatory myopathies (IIM) are a heterogeneous group of subacute, chronic or acute systemic immune-mediated diseases of the skeletal muscles. Recently, research has shown the utility of adding new classifications of myopathies, mainly based on their pathology and the presence of specific autoantibodies. In addition, developments in genetics have helped reveal the genetic background of IIM. The human leukocyte antigen genomic region has been consistently shown to be the strongest genetic risk factor for IIM. The present review summarizes the previous and current literature on human leukocyte antigen analysis of IIM, as well as future directions for research.
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