What is the Maricq and Valter classification for CREST syndrome?

Updated: Oct 05, 2020
  • Author: Jeanie C Yoon, MD; Chief Editor: Dirk M Elston, MD  more...
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Answer

The Maricq and Valter [15] proposed classification for scleroderma spectrum disease is as follows:

  • Type I - Diffuse skin involvement proximal to elbows/knees; includes trunk

  • Type II - Intermediate skin involvement proximal to the metacarpal phalangeal/metatarsal phalangeal joints, distal to the elbows/knees; trunk not involved

  • Type III - Digital sclerodactyly only (meets American College of Rheumatology minor criteria but excludes those without skin involvement)

  • Type IV - Scleroderma sine scleroderma (capillary pattern or pitting scars and visceral involvement; no anticentromere antibodies; no telangiectasia)

  • Type V - Undifferentiated connective-tissue disease with 2 of 3 of the following scleroderma features: sclerodactyly, pitting scars, or scleroderma capillary pattern; or one of these features along with one of the following: Raynaud phenomenon, pulmonary fibrosis, or visceral involvement (esophagus, heart, kidney); but do not meet the criteria for groups III and IV; no anticentromere antibodies; no telangiectasia

  • Type VI - CREST; no skin involvement, or sclerodactyly only, telangiectasia is required with one or more other acronyms; or anticentromere antibodies are required with any 2 or more acronyms


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