Development of the Cold Urticaria Activity Score

Abstract

Background: Cold urticaria (ColdU) is a form of inducible urticaria where cold induces wheals and/or angioedema. The burden of disease is high and linked to trigger thresholds, exposure, and avoidance. There are presently no validated patient-reported outcome measures (PROMs) to assess and monitor disease activity. Our objective was to develop a disease-specific activity score for ColdU that is easy to administer and evaluate.Methods: A Cold Urticaria Activity Score (ColdUAS) questionnaire was developed, directed by PROM developing guidelines. After the generation of a conceptional framework, the item generation phase included the literature research on ColdU signs and symptoms and on comparable tools for similar diseases and 47 ColdU patient interviews. Subsequently, an impact analysis for content validity was performed. The final selection of items underwent expert review for face validity and cognitive debriefing. Results:The ColdUAS, a self-administered questionnaire for the prospective assessment of disease activity in patients with ColdU, consists of 4 items: 1. the frequency and severity of the signs (wheals and/or angioedema), 2. the frequency and severity of the symptoms (e.g., itch and burn), 3. the exposure to specific triggers, and 4. the avoidance of these triggers. The recall period for each item is the last 24 h. Conclusions:The ColdUAS is the first disease-specific PROM to assess ColdU disease activity. It may help to better assess patients' disease status in routine clinical practice as well as in clinical trials. Anchor-based approaches are currently used to validate the ColdUAS.

Urticaria

“…Similarly, the Angioedema Activity Score (AAS) is a prospective, diary-type tool that enables clinicians to measure angioedema activity in patients with all forms of recurrent angioedema 136 . In CIndU, the Cholinergic Urticaria Activity Score (CholUAS; not yet validated) and the Cold Urticaria Activity Score (ColdUAS), modified versions of the UAS, collect data on a daily basis assessing the intensity of wheals and pruritus as well as the intensity of exposure to specific triggers with a recall period of 24 h 134,137,138 . The Urticaria Control Test (UCT) 139 and the Angioedema Control Test (AECT) 140 are retrospective tools with a recall period of 4 weeks used to assess disease control in all forms of CU.…”

“…• Urticaria Activity Score (UAS) 4, 135 • Angioedema Activity Score (AAS) 136 • Cholinergic Urticaria Activity Score (CholUAS) 138 • Cold Urticaria Activity Score (ColdUAS) 137…”

Inhibition of interleukin‐1 with rilonacept is not effective in cold urticaria—Results of a randomized, placebo‐controlled study

“… 8 In addition, we did not use the Cold Urticaria Activity Score (ColdUAS), a patient‐reported outcome measure to assess ColdU disease activity, as it was not available at the start of our study. 40 …”

“…Also, patients with atypical cold urticaria may present similar clinical features as patients with interleukin‐1‐mediated cold‐induced whealing due to autoinflammatory syndromes 8 . In addition, we did not use the Cold Urticaria Activity Score (ColdUAS), a patient‐reported outcome measure to assess ColdU disease activity, as it was not available at the start of our study 40 …”

A Patient Charter for Chronic Urticaria

“…The Angioedema Activity Score, Angioedema Control Test, and Angioedema Quality of Life Questionnaire can also be used when angioedema is present [44,50]. The only tools specifically for measuring disease activity in the subtypes of CIndU are the Cold Urticaria Activity Score, the Symptomatic Dermographism Activity Score, and the Cholinergic Urticaria Activity Score [51][52][53], although disease control in patients with CIndU in general can be measured with the Urticaria Control Test [44]. In addition, the GA 2 LEN/UCARE network has developed the CRUSE Ò app (https://cruse-control.com) that allows patients to fill out a patient-reported outcome questionnaire daily on their smart device and send the results to their physician [24].…”