Chronic spontaneous urticaria (CSU) is a debilitating condition affecting 0.5–1% of the population worldwide. It typically presents with transient hives (wheals) that last for at least 6 weeks, often with concomitant angioedema (Maurer et al, 2011; Zuberbier, 2012). Available evidence indicates that CSU can substantially affect patients' quality of life (QoL), their ability to perform daily tasks and their mental health (O'Donnell et al, 1997; Kang et al, 2009; Vietri et al, 2015; Balp et al, 2017; Mendelson et al, 2017).
International urticaria guidelines on the management of CSU have been jointly developed by the European Academy of Allergy and Clinical Immunology (EAACI), the Global Allergy and Asthma European Network (GA2LEN), the European Dermatology Forum (EDF) and the World Allergy Organization (WAO) (Zuberbier et al, 2018). The guidelines state that the goal of treatment is symptom control or remission. The therapeutic approach to CSU often involves the identification and elimination of underlying causes, the avoidance of eliciting factors and the use of pharmacological treatment. Treatment should follow the treat-to-target strategy of treating as much as needed and as little as possible, which may mean stepping up or stepping down in the treatment algorithm according to the course of CSU. Re-evaluating the need for continued or alternative drug treatment is recommended because the severity of urticaria may fluctuate and spontaneous remission may occur.
However, assessing CSU activity and severity in patients can be difficult because of the common fluctuation in symptoms from one day to another. This can often mean that the clinical picture of a patient at the time of presentation to the treating health professional is rarely representative of the actual current status of their condition. As such, it is important that the identification and assessment of CSU is performed accurately, to enable the implementation of an adequate treatment approach according to treatment guidelines. This article will highlight the patient-reported outcome (PRO) tools recommended in the assessment of CSU.
Assessment of CSU control and activity
As CSU activity frequently changes, the overall activity and control are best measured by advising patients to document their symptoms using validated tools. The EAACI/GA2LEN/EDF/WAO international urticaria guidelines (Zuberbier et al, 2018) recommend assessing activity, impact and control of CSU at the first and every follow-up visit. However, it is acknowledged that while some tools allow retrospective assessment by patients, others may only be used prospectively. Ensuring the correct tools are used is essential in monitoring CSU activity and status to determine optimal treatment. The following tools are the ones most frequently used to assess control and activity in CSU (see Table 1).
| UCT | UAS7 | AAS | DLQI | CU-Q2oL | |
|---|---|---|---|---|---|
| Assessment components | Urticaria symptom control |
Urticaria symptom severity | Angioedema activity |
Dermatology-specific QoL | Urticaria-specific QoL |
| Assessment type | Retrospective: condition over the previous 4 weeks | Prospective: daily questionnaire over 7 consecutive days | Prospective: daily questionnaire over 4 weeks | Retrospective: condition over the 7 days | Retrospective: condition over the previous 2 weeks |
| Angioedema assessment? | Yes | No | Yes | No | No |
| Number of items | 4 | 2 | 5 | 10 | 23 |
| When to use |
|
|
|
|
|
| Scoring | Urticaria activity is: |
Urticaria activity is: |
Angioedema activity is: |
Impact on QoL: |
Higher scores indicate a greater impact on QoL |
| Format | Paper version |
Paper version |
Paper version |
Paper version |
Paper version |
UCT: Urticaria Control Test; UAS7: Urticaria Activity Score; AAS: Angioedema Activity Score; DLQI: Dermatology Life Quality Index; CU-Q2oL: Chronic Urticaria Quality of Life Questionnaire; QoL: quality of life
Urticaria Control Test
The Urticaria Control Test (UCT) is the first validated questionnaire for assessing symptom control in patients with all forms of CSU and has been found to exhibit high levels of validity and reliability (Weller et al, 2014).
Scoring
The UCT is a single questionnaire consisting of four questions, designed to evaluate the physical symptoms of CSU, the impact on health-related QoL and the effectiveness of treatment over the previous 4 weeks. Each question requires assigning a rating from 0 to 4, with 0 indicating ‘very much’ and 4 indicating ‘not at all’. This results in a total score ranging from 0 to 16, with a score of 0 indicating no symptom control, ≤11 indicating poor symptom control, ≥12 denoting well-controlled CSU symptoms, and the highest possible score of 16 representing complete symptom control. It should also be noted that the UCT score comprises itch (pruritus), wheals and/or angioedema.
Clinical practice
The scoring method of the UCT enables a clearly defined cut off for patients with ‘well controlled’ versus ‘poorly controlled’ CSU. The high cut-off value ensures a high degree of sensitivity, making the UCT suitable for the screening of patients and determining their CSU status and control, which is essential for ascertaining whether the patient's current treatment is sufficient or needs to be intensified. As well as serving as a useful tool for the detection of patients with poorly controlled CSU symptoms, the UCT may also be used to monitor CSU status at each visit, and to guide further treatment/referral decisions.
The retrospective and short format of the UCT means patients can complete the assessment while in the waiting room, enabling it to be easily implemented in clinical practice with minimal impact on clinic resources. It is also available as an electronic document so may be sent to the patient for completion before the assessment visit.
Given its retrospective nature, ease of use and easy implementation into clinical practice, the UCT is recommended for use at the initial screening of patients and at every follow-up visit. The recently updated EAACI/GA2LEN/EDF/WAO international urticaria guidelines recommend using the UCT for the evaluation of CSU control and monitoring of CSU status in routine clinical practice (Zuberbier et al, 2018).
Urticaria Activity Score
The Urticaria Activity Score over 7 days (UAS7) questionnaire is a validated daily measure of assessing urticaria severity and monitoring treatment outcomes. It comprises two questions that evaluate the severity of itching (the itch severity score) and number of hives (daily hives score); scores are recorded every day for 7 days to give an overall composite score (Młynek et al, 2008).
Scoring
The daily UAS equals the sum of the daily itch severity score (range: 0-3; with 0 indicating ‘none’ and 3 indicating ‘intense’) and the daily hives score (range: 0-3; 0 indicating ‘none’ and 3 indicating ‘>50 hives’), resulting in a total UAS7 ranging from 0 to 42.
A score of 0 indicates that the patient is itch- and hives-free, while a score ranging from 1 to 6 has been proposed to reflect well-controlled CSU. Scores ranging from 7 to 15, 16 to 27 and 28 to 42 are proposed to indicate mild, moderate and severe CSU activity, respectively.
Clinical practice
The scoring method of the UAS7 allows the severity of patients' CSU to be clearly defined. This makes the test suitable for monitoring patients' current symptom severity and determining whether the current treatment is sufficient or needs to be intensified, guiding further treatment and referral decisions.
Additionally, in many health services, evidence of poorly controlled CSU symptoms is essential when considering stepping up from antihistamines in the treatment algorithm. Monitoring patients' symptom severity using the UAS7 ensures accurate documentation and the collection of evidence, enabling optimal treatment decisions to be made efficiently.
Furthermore, the prospective, short format of the UAS7 and its electronic availability means that following initial screening, patients can complete the assessment between visits. This maximises the information gathered because patients can record symptom severity daily, and it has a minimal impact on clinic resources.
Given the ease of use, straightforward implementation into clinical practice, depth of data that can be collected and the ability to assess symptoms daily, the UAS7 is recommended for use at every follow-up visit. The EAACI/GA2LEN/EDF/WAO international urticaria guidelines recommend the evaluation of CSU activity, severity and response to treatment in routine clinical practice using the UAS7 (Zuberbier et al, 2018).
A limitation of the UAS7 is that it does not include a component relating to angioedema; over 50% of patients with CSU have associated angioedema at baseline, and the presence of angioedema has been shown to impair patient QoL more than hives alone (Maurer et al, 2011).
Angioedema Activity Score
The Angioedema Activity Score (AAS) questionnaire is a validated daily measure of angioedema activity in patients with recurrent angioedema (Weller et al, 2013).
Scoring
The AAS is a daily questionnaire comprising five questions evaluating the severity of CSU symptoms and angioedema.
The daily AAS equals the sum of five key factors relating to patients' symptoms, rated 0 to 3 (giving a daily score ranging from 0 to 15), which can then be added up to give 7-day scores (AAS7), 4-week scores (AAS28) and 12-week scores (AAS84).
An AAS7 score of 0-24 has been proposed to reflect mild symptom severity, while AAS7 scores ranging from 25 to 59 and 60 or over are proposed to indicate moderate and severe symptom severity, respectively.
Clinical practice
As the AAS7 indicates the symptom severity of patients with angioedema, it is suitable for monitoring current symptom severity and can be used instead of the UAS7 if angioedema is present. The AAS7 is recommended for use in conjunction with assessment of bloods and family history, and referral to an allergy and immunology clinic is often advised after repeated episodes to exclude hereditary causes.
As with the UAS7, the AAS7 ensures accurate documentation and evidence of symptom severity, allowing optimal treatment escalations to be made if necessary. Similarly, the AAS7's prospective and short format and electronic availability mean that, following initial screening, patients can complete the assessment between visits. As such, the AAS7 is recommended for use at every follow-up visit in place of the UAS7 for patients with angioedema alone.
The EAACI/GA2LEN/EDF/WAO international urticaria guidelines recommend evaluation of angioedema activity, severity and response to treatment in routine clinical practice using the AAS7 (Zuberbier et al, 2018).
Assessment of quality of life
In addition to assessing symptom severity and control, the assessment of patient QoL is also essential to ensuring patients are receiving optimal treatment and care. Importantly, QoL should always be assessed independently from CSU activity as the psychosocial impact of urticaria may be high, despite low symptom severity (Koti et al, 2013). The following tools are recommended in the assessment of QoL in patients with CSU.
Dermatology Life Quality Index
The Dermatology Life Quality Index (DLQI) is a validated and widely used dermatology-specific patient questionnaire for evaluating health-related QoL in patients with a variety of skin conditions, including CSU (Finlay and Khan, 1994; Młynek et al, 2008).
Scoring
The DLQI is a daily questionnaire comprising 10 questions concerning symptoms and their impact on a patient's feelings, daily activities, leisure, work/school, personal relationships and treatment over the previous 7 days. Each question is scored from 0 to 3, with 0 indicating ‘no impact’ and 3 indicating ‘very much’. The scores are then added up, giving a range from 0 (no impairment on QoL) to 30 (maximum impairment on QoL). A DLQI score of 0-1 indicates no impact on QoL, while scores of 2-5, 6-10 and 11-20 indicate a small, moderate and very large impact of CSU symptoms on QoL, respectively. Scores of 21-30 indicate that CSU symptoms have an extremely large impact on QoL.
Clinical practice
Assessment of QoL is essential to ensuring patients are receiving the most appropriate treatment. As such, the DLQI or another measure of QoL is recommended for use at each patient visit to monitor QoL over time and to guide further treatment and referral decisions. This is supported by guidelines by the EAACI/GA2LEN/EDF/WAO, the National Institute for Health and Care Excellence (NICE) and the British Society for Allergy & Clinical Immunology (BSACI), which all recommend the use of the DLQI in monitoring the QoL of patients with CSU (Powell et al, 2015; NICE, 2018; Zuberbier et al, 2018).
Similar to the UCT, the DLQI's retrospective nature and availability as an electronic version mean patients can complete the assessment while sitting in the clinic waiting room. This allows the assessment to be easily incorporated into clinical practice with minimal impact on clinic resources.
Chronic Urticaria Quality of Life Questionnaire
The Chronic Urticaria Quality of Life Questionnaire (CU-Q2oL) is a validated, self-administered questionnaire assessing QoL in patients with CSU (Baiardini et al, 2005). Although the assessment is longer than the DLQI, the CU-Q2oL was developed to assess QoL in patients with CSU specifically, and is thus advantageous in outlining the impact CSU has on patients.
Scoring
The CU-Q2oL is a daily questionnaire comprising 23 questions aimed at finding out to what extent patients have been troubled by each problem over the preceding 2 weeks. Each question requires assigning a score from 1 (indicating ‘not at all’) to 5 (indicating ‘very much’). The scores are then added up, giving a range from 0 to 115, with a higher composite score indicating greater impact of CSU on patient QoL.
Clinical practice
The CU-Q2oL or another measure of QoL is recommended for use at each patient visit to monitor patient QoL over time and to guide further treatment and referral decisions. The CU-Q2oL can be used instead of the DLQI, with the EAACI/GA2LEN/EDF/WAO, NICE and BSACI guidelines also listing it as a validated and robust measure of monitoring QoL in patients with urticaria (Powell et al, 2015; NICE, 2018; Zuberbier et al, 2018).
As with the DLQI, the CU-Q2oL's retrospective nature and availability as an electronic version mean patients can complete the assessment while sitting in the clinic waiting room or before their visit, allowing the assessment to be easily incorporated into clinical practice with minimal impact on clinic resources.
Discussion
The clinical picture of a patient at the time of presentation to the treating health professional is rarely representative of actual current CSU status. The accurate identification and assessment of CSU is essential to enable the implementation of the optimal treatment approach, according to treatment guidelines.
There are several guidelines on the management of CSU (Zuberbier et al, 2018). In particular, the EAACI/GA2LEN/EDF/WAO international urticaria guidelines recommend aiming for complete symptom control (treat-to-target) using pharmacological treatment as much as needed and as little as possible. This can often mean that treatment selection changes throughout the course of CSU, with patients stepping up or stepping down in the treatment algorithm and the constant re-evaluation of the need for continued or alternative drug treatment.
To assist in the accurate evaluation of symptom severity and the management of patients with CSU, the EAACI/GA2LEN/EDF/WAO guidelines recommend the use of PRO tools in clinical care to help ascertain treatment needs, highlighting that as urticaria activity frequently changes, the overall activity is best measured by advising patients to document self-evaluation scores (Zuberbier et al, 2018). In line with these recommendations, the NICE and BSACI treatment guidelines for CSU also recommend the accurate assessment of CSU status and QoL to ensure patients can be moved up or down in the treatment algorithm accordingly (Powell et al, 2015; NICE, 2018).
The PRO methods described in this article are easy-to-use, effective and robust measures of symptom severity and patient QoL, and are recommended by the EAACI/GA2LEN/EDF/WAO guidelines. As the PRO tools are self-administered, CSU symptom severity and impact on patients' QoL can be recorded outside clinic visits and monitored with minimal impact on overall clinic costs.
Furthermore, the use of PRO tools in daily clinical practice will enable the accurate monitoring of CSU activity; it will allow health professionals to ensure patients are receiving the correct treatment and that documentation and evidence of treatment needs are recorded. This may be particularly valuable when considering moving the patient up in the treatment algorithm.
Taking this into account, it is recommended that the UCT be performed at every assessment. In secondary care, the UAS7 should be performed at each visit and is necessary when considering stepping up in the treatment algorithm from antihistamines or biological treatment options. For patients with angioedema, the AAS should be used in place of the UAS7. Additionally, an assessment of QoL, which should include the DLQI at a minimum, should be made at each visit. Including these PRO methods in the treatment pathway will help standardise care provided to different patients and facilitate the treatment of patients in line with recommended guidelines, which require evidence of symptom severity before the provision of some treatments.
Providing patients with access to these self-administered questionnaires before appointments will aid in their incorporation into clinical practice, and the further development of standardised care pathways indicating when PRO tools should be used in the treatment pathway would be of great benefit when introducing them into everyday clinical practice. Creating a gold standard for care in CSU will aid in achieving the treat-to-target strategy, subsequently increasing patient wellbeing and reducing healthcare costs through the optimal treatment and management of patients with CSU. This will not only ensure adherence to treatment guidelines, but may also provide benefits in the future training of nurses in CSU.
Conclusions
The use of PRO tools ensures patients receive the most appropriate treatment, resulting in improved QoL and psychosocial well-being. Incorporating PRO measures into clinical practice will help enable symptom control and normalisation of patients' QoL, while standardising care and enabling efficient data collection to help ensure health professionals adhere to treatment guidelines. Introducing such a gold standard for care in CSU will also provide cost benefits by allowing the efficient and optimal management of patients.