What is the prognosis of CREST syndrome?

Updated: Oct 05, 2020
  • Author: Jeanie C Yoon, MD; Chief Editor: Dirk M Elston, MD  more...
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In a large 2003 US study by Mayes et al, [33] the survival rate from time of diagnosis was computed to be 77.9% at 5 years, 55.1% at 10 years, 37.4% at 15 years, and 26.8% at 20 years. The extent of skin involvement is a good predictor of survival in patients with scleroderma.

Limited cutaneous disease (as defined by Medsger [42] in 1997) is associated with a better survival rate than diffuse disease. [13, 12] Similarly, patients with sclerodactyly alone have better survival rates than patients with truncal skin involvement. [8]  Calcinosis cutis may be problematic, even in patients with limited cutaneous disease. [43]

Renal involvement is responsible for half of all scleroderma-related deaths in patients with widespread skin changes, while patients with sclerodactyly alone do not tend to develop any type of renal disease.

The mortality in patients with limited skin involvement results from cardiac, pulmonary, and GI causes.

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