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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.
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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.

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Figure 1. Photosensitive Facial Rash in Case Patient
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Heliotrope, malar rash, and facial erythema in pattern of photosensitivity in an 11-year-old patient with juvenile dermatomyositis and anti-p155 autoantibodies.

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Figure 2. Characteristic Findings Associated With Clinical and Autoantibody Phenotypes in Myositis
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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.

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