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New Classification Criteria for Systemic Sclerosis

2013 criteria outperform 1980 measures (October 3)

New classification criteria for systemic sclerosis have been developed by a joint committee commissioned by the American College of Rheumatology (ACR) and the European League Against Rheumatism (EULAR). The new criteria, published in the ACR journal Arthritis & Rheumatism, supersede 1980 measures.

Systemic sclerosis, also known as scleroderma, is a connective-tissue disease that is characterized by sclerodermatous skin changes (hardening of tissue due to increased collagen deposits); by Reynaud’s phenomenon (spasms of small blood vessels in response to cold or stress that can lead to vasculopathy and tissue death); and by internal organ fibrosis. The ACR estimates that systemic sclerosis affects 49,000 U.S. adults.

Based on the new criteria, a patient with thickening of the skin in the middle part of the fingers (from the proximal to the metacarpophalangeal joints) would be classified as having systemic sclerosis, regardless of other features. If this criterion was not met, however, then seven items with varying weights would need to be assessed in order to obtain a classification of scleroderma:

  • Skin thickening of the fingers
  • Fingertip lesions
  • Telangiectasia
  • Abnormal nailfold capillaries
  • Pulmonary arterial hypertension and/or interstitial lung disease
  • Raynaud’s phenomenon
  • Systemic sclerosis-related antibodies

The results of validation testing showed that sensitivity and specificity were both greater than 90% for the 2013 systemic sclerosis classification criteria compared with 75% for the 1980 ACR criteria.

Source: Wiley; October 3, 2013.

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