International consensus criteria for the diagnosis of Raynaud's phenomenon.
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.
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.
Emanual
E
Maverakis
Department of Dermatology, University of California, Davis, Sacramento, CA 95817, USA.
Forum
F
Patel
Department of Dermatology, University of California, Davis, Sacramento, CA 95817, USA.
Daniel G
DG
Kronenberg
Department of Internal Medicine and Dermatology, Division of Immunology and Rheumatology, Stanford University and Palo Alto VA Hospital, Palo Alto, CA 94305, USA.
Lorinda
L
Chung
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.
David
D
Fiorentino
Department of Rheumatology, Paris Descartes University, Paris, France.
Yannick
Y
Allanore
Department of Rheumatology, University of Florence, Florence, Italy.
Serena
S
Guiducci
Department of Rheumatology, Lund University, Lund, Sweden.
Roger
R
Hesselstrand
Department of Medicine/Rheumatology, Johns Hopkins University, Baltimore, MD 21287, USA.
Laura K
LK
Hummers
Department of Dermatology, University of California, Davis, Sacramento, CA 95817, USA.
Chris
C
Duong
Department of Internal Medicine, Division of Rheumatology, University of Toledo, Toledo, OH 43614, USA.
Bashar
B
Kahaleh
Department of Rheumatology, University of East Anglia, Norwich, Norfolk, United Kingdom.
Alexander
A
Macgregor
Department of Medicine & Rheumatology, University of Florence, Florence, Italy.
Marco
M
Matucci-Cerinic
Department of Rheumatology, Lund University, Lund, Sweden.
Frank A
FA
Wollheim
Department of Internal Medicine, Division of Rheumatology and Clinical Immunogenetics, University of Texas-Houston, Houston, TX 77030, USA.
Maureen D
MD
Mayes
Department of Internal Medicine, Division of Rheumatology, University of California, Davis, CA 95616, USA.
M Eric
ME
Gershwin
D001172:Arthritis, Rheumatoid / Q000175:diagnosis; D001327:Autoimmune Diseases / Q000175:diagnosis; D003625:Data Collection; D003882:Dermatomyositis / Q000175:diagnosis; D003937:Diagnosis, Differential; D007391:International Cooperation; D008180:Lupus Erythematosus, Systemic / Q000175:diagnosis; D008947:Mixed Connective Tissue Disease / Q000175:diagnosis; D011928:Raynaud Disease / Q000175:diagnosis*; D012595:Scleroderma, Systemic / Q000175:diagnosis; D012859:Sjogren's Syndrome / Q000175:diagnosis
Diagnostic criteria; Primary Raynaud's; Raynaud's phenomenon; Secondary Raynaud's