International consensus criteria for the diagnosis of Raynaud's phenomenon

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.

ESVM guidelines – the diagnosis and management of Raynaud’s phenomenon

“…However, this classical triphasic colour change is not always present (occurring in about one-third of primary RP patients and two thirds of secondary RP associated with systemic sclerosis [5]) but blanching must be a feature in order for the diagnosis of RP to be made. As cyanosis and rubor are not always present, it should be remembered that blanching alone can also allow for the diagnosis of RP to be made.…”

Nailfold capillaroscopic findings in patients with Raynaud’s phenomenon: Preliminary report

“…The diagnostic criteria for primary RP include normal NFC findings. 9,10 Rest of those with RP is likely to have an underlying cause. 8 Connective tissue disorders are associated with and often present with RP.…”

Raynaud’s phenomenon — the clinical picture, treatment and diagnostics

“…The most common risk factor that can lead to RP is exposure to cold or a sudden change in temperature (for example entering an air-conditioned room) [3]. Moreover, factors such as emotional stress, exposure to vibrations, excessive activities requiring precise movements of the fingers, smoking [7], as well as therapy with beta-blockers may also provoke the paroxysmal vasospasm of small vessels [3,8].…”

“…Moreover, factors such as emotional stress, exposure to vibrations, excessive activities requiring precise movements of the fingers, smoking [7], as well as therapy with beta-blockers may also provoke the paroxysmal vasospasm of small vessels [3,8].…”

“…Clinically, two types of RP can be distinguishedprimary RP (of a relatively benign course) and secondary RP, which occurs secondarily to other medical conditions such as connective tissue disorders [3,4]. Primary RP, if idiopathic and not associated with other diseases, it is referred to as Raynaud's disease (80% of RP cases).…”