Activity Score Helps Cold Urticaria Patients Monitor Their Signs, Symptoms, and Encounters With Cold

Lorraine L. Janeczko, MPH

May 04, 2022

A newly developed rating scale called the Cold Urticaria Activity Score (ColdUAS) can help patients with cold urticaria (ColdU) monitor their signs, symptoms, exposure to cold, and avoidance of cold by answering four simple questions, according to a new report.

"The final 4-item ColdUAS is the first PROM [patient-reported outcome measure] for the assessment of disease activity in ColdU patients," the authors write in the journal Allergy. "With its simple and user-friendly application for adolescents aged 12 years and older, the ColdUAS provides a quick overview of disease activity and status. Furthermore, it can be applied for documentation in daily patient care and clinical trials."

For patients with this rare, chronic condition, exposure to cold — from weather, food, drink, or objects touching their skin — provokes wheals, angioedema, headache, and in some cases, anaphylaxis. Existing tools that assess ColdU activity, such as the Urticaria Activity Score (UAS) for chronic spontaneous urticaria (CSU), aren't useful for ColdU patients because although they may have high disease activity, they tend to avoid exposing themselves to cold, so their signs and symptoms may be mild.

ColdUAS Questionnaire Available in German and English

To develop the ColdUAS questionnaire in German and English, senior author Dorothea Terhorst-Molawi, MD, of Charité University Hospital in Berlin, Germany, and colleagues began with a search of PubMed for relevant information in both languages, including similar instruments and activity scores related to cold urticaria.

For the German version, they interviewed 47 patients about their condition at one urticaria treatment center in Berlin between July 2019 and July 2020. Participants ranged in age from 14 to 82 years and had active ColdU. Of these, 34 had typical ColdU and 13 had atypical ColdU; 33 were women, and 3 were younger than 18.

All participants also underwent two cold provocation tests: a standardized ice cube test (ICT) and a standardized assessment of critical temperature threshold (CTT). Those with typical ColdU developed wheals from the ICT, the CTT, or both, while those with atypical cold urticaria were diagnosed based on modified provocation tests, history, and photo documentation, or both.

After reviewing the literature search and the transcribed patient interviews, the researchers developed the participants' common main symptoms, triggers, and avoidance behavior into the tool's four questions.

They created the American English translation after testing it on 4 female and 5 male American English native-speaking ColdU patients between age 22-49 years.

The first three questions ask patients whether they experienced (1) wheals or swelling; (2) itching, burning, pain, or feeling hot because of cold exposure; and (3) whether they've been exposed to cold temperatures that usually cause cold urticaria symptoms. Patients are asked to rate those factors, if present, as mild, moderate, or severe. The fourth question asks whether the patient has avoided cold temperatures because of their symptoms, and the patient is able to answer "no," "partially avoided," or "completely avoided."

Dr Amin Kanani

Amin Kanani, MDCM, a clinical associate professor in the Faculty of Medicine and the head of the Division of Allergy and Clinical Immunology at the University of British Columbia in Vancouver, Canada, welcomed ColdUAS.

"Patient-reported outcomes such as the ColdUAS are important for understanding whether treatments for this condition are impacting a patient's health and quality of life," Kanani told Medscape Medical News in an email.

"The ColdUAS gives an understanding of the effectiveness of care from the patient's perspective," added Kanani, who was not involved in the project. "This tool will be valuable in managing patients with this disorder."

Marc A. Riedl, MD, a professor of medicine and the clinical director of the US Hereditary Angioedema Association (HAEA) Angioedema Center at UC San Diego Health System, California, said that this tool may be very useful both in clinical practice and in clinical studies of cold urticaria.

Dr Marc Riedl

"This work is an effort to better measure and quantify the activity of the condition as well as the impact on patients' lives," said Riedl in an email. "The scoring tool accurately represents what we aim to cover clinically during patient diagnosis and follow-up and it reflects what is often most important to patients in terms of how the condition affects their lives." 

"The tool may allow clinicians to better monitor the daily impact of the condition on patients' lives and activities as well as better evaluate the efficacy of treatment plans," added Riedl, who also was not involved in the project.

"It will be interesting to see application and validation studies across other, more diverse populations, and in younger patients," he said.

The authors write that strengths of the project include the wide age range of participants, and participation by both males and females as well as patients with typical and atypical ColdU of various severity levels.

They also acknowledged its limitations, including the single study site, the small number of participants, many of them being White, and female-only participation in some stages of the process.

"A larger and more diverse study population from different geographic areas could have resulted in a different outcome," they note.

The authors add that COVID-19 might also have affected their results. ColdUAS was developed in part during the pandemic, when many people changed their habits, spent less leisure time outdoors, and may have changed their perceptions of the disease.

The research team is developing a ColdUAS scoring system and plans to do further research to establish the tool's validity, reliability, sensitivity to change, minimal clinical important difference, and recommended observation period.

The research was partially funded by sanofi-aventis US LLC and Celldex Pharmaceuticals. Terhorst-Molawi and several co-authors declare financial involvements with pharmaceutical companies. The full list can be found with the original article. Reidl and Kanani have disclosed no relevant financial relationships.

Allergy. Published online April 11, 2022. Full text

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