What are the classification criteria for CREST syndrome proposed by Nadashkevich et al?

Updated: Oct 05, 2020
  • Author: Jeanie C Yoon, MD; Chief Editor: Dirk M Elston, MD  more...
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In 2004, Nadashkevich et al [14] proposed the classification criteria (1) autoantibodies to centromere proteins, Scl-70 (topo I) and fibrillarin; (2) bibasilar pulmonary fibrosis; (3) contractures of the digital joints or the prayer sign; (4) dermal thickening proximal to the wrists; (5) calcinosis cutis; (6) Raynaud phenomenon (at least a 2-phase color change); (7) esophageal distal hypomotility or reflux esophagitis; (8) sclerodactyly or nonpitting digital edema; and (9) telangiectasias, which can be remembered by the abbreviation ABCDCREST. Fulfilling 3 or more criteria indicates definite systemic scleroderma with a sensitivity and specificity as high as 99% and 100%, respectively.

Also in 2004, Maricq and Valter [15] had a complex but potentially very useful proposal for classifying the scleroderma spectrum disorders; however, in 2005, Wollheim [16] reported that without substantial independent confirmatory work, this classification system may not gain widespread acceptance in its present form.

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