Grand Rounds
Clinician's Corner
January 12, 2011

Deciphering the Clinical Presentations, Pathogenesis, and Treatment of the Idiopathic Inflammatory Myopathies

Author Affiliations

Author Affiliations: Environmental Autoimmunity Group, Program of Clinical Research, National Institute of Environmental Health Sciences, National Institutes of Health, US Department of Health and Human Services, Bethesda, Maryland.

JAMA. 2011;305(2):183-190. doi:10.1001/jama.2010.1977

The idiopathic inflammatory myopathies or myositis syndromes (the most common forms are polymyositis, dermatomyositis, and inclusion body myositis) are systemic autoimmune diseases defined by chronic muscle weakness and inflammation of unknown etiology and result in significant morbidity and mortality. Research suggests that categorizing heterogeneous myositis syndromes into mutually exclusive and stable phenotypes by using clinical and immune response features is useful for predicting clinical signs and symptoms, associated genetic and environmental risk factors, and responses to therapy and prognosis. Knowledge of myositis phenotypes should enhance clinicians' ability to recognize and manage these rare disorders.