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Understanding Systemic Sclerosis (SSc) and SCOT

Clinical Features of Systemic Sclerosis

Raynaud's Phenomenon

Raynaud's phenomenon occurs in more than 85% of individuals with SSc and is often the first complaint reported. Clinical manifestations include distinctive color changes in the skin of the affected digits. The abnormal vasoconstriction of digital arteries and cutaneous arterioles is thought to be due to a defect in normal vascular responses.

Skin Changes

  • Skin thickening and tightness
  • Atrophy of appendages
  • Reduced range of motion
    • Finger flexion contractures
    • Decreased oral aperture
photo of a hand with manifestation of Raynaud's phenomenon

Photo courtesy Massachusetts Medical Society

photo of a hand with manifestation of Raynaud's phenomenon

Photo courtesy Duke University Medical Center

Interstitial Lung Disease

  • Signs and symptoms:
    • Cough
    • Dyspnea
    • Pleural rub or dry rales
  • Imaging changes
    • Interstitial infiltrates (early)
      • CT: "ground glass" opacities
    • Scarring (late)
      • CT: "honeycombing," traction bronchiectasis
  • Pulmonary function
    • Restrictive pattern
      • Depressed lung volumes (proportionately reduced FEV1)
      • Reduced diffusing capacity (pulmonary hypertension) and reduced MVV (myopathy)

Course of Systemic Sclerosis

The degree of internal organ involvement greatly influences survival in limited and diffuse forms of SSc. The mortality rate for individuals with diffuse disease is approximately 5 to 8 times higher than that of the general population, after adjustment for age and gender. For those with limited skin involvement, the increase in the mortality ratio is approximately twofold. In one U.S. epidemiological study of individuals with SSc with both limited and diffuse disease, renal disease and pulmonary involvement (pulmonary fibrosis and/or pulmonary hypertension) accounted for the most significant mortality.

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