International consensus criteria for the diagnosis of Raynaud's phenomenon

J Autoimmun. Feb-Mar 2014;48-49:60-5. doi: 10.1016/j.jaut.2014.01.020. Epub 2014 Feb 1.


Vasoconstriction accompanied by changes in skin color is a normal physiologic response to cold. The distinction between this normal physiology and Raynaud's phenomenon (RP) has yet to be well characterized. In anticipation of the 9th International Congress on Autoimmunity, a panel of 12 RP experts from 9 different institutes and four different countries were assembled for a Delphi exercise to establish new diagnostic criteria for RP. Relevant investigators with highly cited manuscripts in Raynaud's-related research were identified using the Web of Science and invited to participate. Surveys at each stage were administered to participants via the on-line SurveyMonkey software tool. The participants evaluated the level of appropriateness of statements using a scale of 1 (extremely inappropriate) through 9 (extremely appropriate). In the second stage, panel participants were asked to rank rewritten items from the first round that were scored as "uncertain" for the diagnosis of RP, items with significant disagreement (Disagreement Index > 1), and new items suggested by the panel. Results were analyzed using the Interpercentile Range Adjusted for Symmetry (IPRAS) method. A 3-Step Approach to diagnose RP was then developed using items the panelists "agreed" were "appropriate" diagnostic criteria. In the final stage, the panel was presented with the newly developed diagnostic criteria and asked to rate them against previous models. Following the first two iterations of the Delphi exercise, the panel of 12 experts agreed that 36 of the items were "appropriate", 12 items had "uncertain" appropriateness, and 13 items were "inappropriate" to use in the diagnostic criteria of RP. Using an expert committee, we developed a 3-Step Approach for the diagnosis of RP and 5 additional criteria for the diagnosis of primary RP. The committee came to an agreement that the proposed criteria were "appropriate and accurate" for use by physicians to diagnose patients with RP.

Keywords: Diagnostic criteria; Primary Raynaud's; Raynaud's phenomenon; Secondary Raynaud's.

Publication types

  • Research Support, N.I.H., Extramural
  • Research Support, Non-U.S. Gov't
  • Review

MeSH terms

  • Arthritis, Rheumatoid / diagnosis
  • Autoimmune Diseases / diagnosis
  • Consensus
  • Data Collection
  • Dermatomyositis / diagnosis
  • Diagnosis, Differential
  • International Cooperation
  • Lupus Erythematosus, Systemic / diagnosis
  • Mixed Connective Tissue Disease / diagnosis
  • Raynaud Disease / diagnosis*
  • Scleroderma, Systemic / diagnosis
  • Sjogren's Syndrome / diagnosis