Grand Rounds | Clinician's Corner

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

Lisa G. Rider, MD; Frederick W. Miller, MD, PhD
JAMA. 2011;305(2):183-190. doi:10.1001/jama.2010.1977.
Text Size: A A A
Published online

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.

Figures in this Article

Sign In to Access Full Content

Don't have Access?

Register and get free email Table of Contents alerts, saved searches, PowerPoint downloads, CME quizzes, and more

Subscribe for full-text access to content from 1998 forward and a host of useful features

Activate your current subscription (AMA members and current subscribers)

Purchase Online Access to this article for 24 hours


Place holder to copy figure label and caption
Figure 1. Photosensitive Facial Rash in Case Patient
Graphic Jump Location

Heliotrope, malar rash, and facial erythema in pattern of photosensitivity in an 11-year-old patient with juvenile dermatomyositis and anti-p155 autoantibodies.

Place holder to copy figure label and caption
Figure 2. Characteristic Findings Associated With Clinical and Autoantibody Phenotypes in Myositis
Graphic Jump Location

A, Interstitial lung disease, defined by high-resolution computed tomographic (CT) scanning, is seen frequently in patients with polymyositis, as well as with the antisynthetase autoantibody phenotype. More recently, rapidly progressive interstitial lung disease has been associated with the anti–CADM-140 autoantibody. The chest CT scan depicted is from a 55-year-old female patient with anti–Jo-1 autoantibodies and interstitial lung disease. B, Dystrophic calcification around the elbow and forearm of a 10.5-year-old girl with juvenile dermatomyositis and anti-MJ autoantibodies. Anti-MJ autoantibodies are seen in up to 25% of patients with juvenile-onset dermatomyositis and less frequently in patients with adult-onset disease. Calcinosis is present in more than 60% of patients with the anti–MJ autoantibody phenotype. C, Lipodystrophy (loss of subcutaneous fat with frequent insulin resistance, diabetes, and hypertriglyceridemia) is associated with juvenile-onset dermatomyositis. Generalized lipodystrophy (a widespread loss of fat from the trunk and extremities), has been associated with anti-p155 autoantibodies. A loss of fat from the arm is seen in this 20-year-old woman with juvenile-onset dermatomyositis since age 8 years, who has anti-p155 autoantibodies and lipodystrophy.




You need to register in order to view this quiz.
Citing articles are presented as examples only. In non-demo SCM6 implementation, integration with CrossRef’s "Cited By" API will populate this tab (http://www.crossref.org/citedby.html).


Some tools below are only available to our subscribers or users with an online account.

Web of Science® Times Cited: 16

Sign In to Access Full Content

Related Content

Customize your page view by dragging & repositioning the boxes below.

Articles Related By Topic
Related Topics
PubMed Articles