Review

.2014 Feb-Mar:48-49:60-5.

doi: 10.1016/j.jaut.2014.01.020. Epub 2014 Feb 1.

International consensus criteria for the diagnosis of Raynaud's phenomenon

Emanual Maverakis¹, Forum Patel², Daniel G Kronenberg², Lorinda Chung³, David Fiorentino⁴, Yannick Allanore⁵, Serena Guiducci⁶, Roger Hesselstrand⁷, Laura K Hummers⁸, Chris Duong², Bashar Kahaleh⁹, Alexander Macgregor¹⁰, Marco Matucci-Cerinic¹¹, Frank A Wollheim⁷, Maureen D Mayes¹², M Eric Gershwin¹³

Affiliations

¹ Department of Dermatology, University of California, Davis, Sacramento, CA 95817, USA; Department of Dermatology, Veterans Affairs Northern California Health Care System, Sacramento, CA 95655, USA. Electronic address: emaverakis@ucdavis.edu.
² Department of Dermatology, University of California, Davis, Sacramento, CA 95817, USA.
³ Department of Internal Medicine and Dermatology, Division of Immunology and Rheumatology, Stanford University and Palo Alto VA Hospital, Palo Alto, CA 94305, USA.
⁴ Department of Internal Medicine and Dermatology, Division of Immunology and Rheumatology, Stanford University and Palo Alto VA Hospital, Palo Alto, CA 94305, USA; Department of Dermatology, Stanford University, Redwood City, CA 94305, USA.
⁵ Department of Rheumatology, Paris Descartes University, Paris, France.
⁶ Department of Rheumatology, University of Florence, Florence, Italy.
⁷ Department of Rheumatology, Lund University, Lund, Sweden.
⁸ Department of Medicine/Rheumatology, Johns Hopkins University, Baltimore, MD 21287, USA.
⁹ Department of Internal Medicine, Division of Rheumatology, University of Toledo, Toledo, OH 43614, USA.
¹⁰ Department of Rheumatology, University of East Anglia, Norwich, Norfolk, United Kingdom.
¹¹ Department of Medicine & Rheumatology, University of Florence, Florence, Italy.
¹² Department of Internal Medicine, Division of Rheumatology and Clinical Immunogenetics, University of Texas-Houston, Houston, TX 77030, USA.
¹³ Department of Internal Medicine, Division of Rheumatology, University of California, Davis, CA 95616, USA.

PMID:24491823
PMCID: PMC4018202
DOI: 10.1016/j.jaut.2014.01.020

Review

International consensus criteria for the diagnosis of Raynaud's phenomenon

Emanual Maverakis et al. J Autoimmun.2014 Feb-Mar.

.2014 Feb-Mar:48-49:60-5.

doi: 10.1016/j.jaut.2014.01.020. Epub 2014 Feb 1.

Authors

Affiliations

¹ Department of Dermatology, University of California, Davis, Sacramento, CA 95817, USA; Department of Dermatology, Veterans Affairs Northern California Health Care System, Sacramento, CA 95655, USA. Electronic address: emaverakis@ucdavis.edu.
² Department of Dermatology, University of California, Davis, Sacramento, CA 95817, USA.
³ Department of Internal Medicine and Dermatology, Division of Immunology and Rheumatology, Stanford University and Palo Alto VA Hospital, Palo Alto, CA 94305, USA.
⁴ Department of Internal Medicine and Dermatology, Division of Immunology and Rheumatology, Stanford University and Palo Alto VA Hospital, Palo Alto, CA 94305, USA; Department of Dermatology, Stanford University, Redwood City, CA 94305, USA.
⁵ Department of Rheumatology, Paris Descartes University, Paris, France.
⁶ Department of Rheumatology, University of Florence, Florence, Italy.
⁷ Department of Rheumatology, Lund University, Lund, Sweden.
⁸ Department of Medicine/Rheumatology, Johns Hopkins University, Baltimore, MD 21287, USA.
⁹ Department of Internal Medicine, Division of Rheumatology, University of Toledo, Toledo, OH 43614, USA.
¹⁰ Department of Rheumatology, University of East Anglia, Norwich, Norfolk, United Kingdom.
¹¹ Department of Medicine & Rheumatology, University of Florence, Florence, Italy.
¹² Department of Internal Medicine, Division of Rheumatology and Clinical Immunogenetics, University of Texas-Houston, Houston, TX 77030, USA.
¹³ Department of Internal Medicine, Division of Rheumatology, University of California, Davis, CA 95616, USA.

PMID:24491823
PMCID: PMC4018202
DOI: 10.1016/j.jaut.2014.01.020

Abstract

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.

Published by Elsevier Ltd.

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Conflict of interest statement

Conflict of interest disclosures: None Reported

Figures

**Figure 1**
Expert panel development process.

**Figure 2**
Three-step outline for newly proposed diagnostic method.

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International consensus criteria for the diagnosis of Raynaud's phenomenon

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International consensus criteria for the diagnosis of Raynaud's phenomenon

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