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CSU Learning Zone

Disease Overview

Read time: 45 mins
Last updated:12th Mar 2020
This section of the Learning Zone introduces urticaria and covers the major topics in disease awareness. Choose one of the sections below, or scroll down the page, to learn about the epidemiology and different classifications of urticaria, the pathophysiology and symptoms to look out for, or see diagnosis and assessment tools. Current guidelines and potential treatments are also presented, allowing you to consider the options for your patient.

Added in September 2018 – the Comorbidities section reviews the autoimmune, atopy and psychiatric comorbidities that have been associated with CSU. In addition, real-world data has now been added to the treatment section, providing insights into efficacy and safety outside of a clinical trial setting.

Epidemiology

In this section of the Learning Zone, we explore the prevalence and duration of urticaria, including the risk factors for increased time with the condition. The burden of disease is also explored, with consideration of the impact on quality of life, daily functioning and socioeconomic factors. 

You can also learn more on the epidemiology of chronic urticaria from Professor Marcus Maurer, filmed at EAACI 2019.

 

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Pathophysiology

 

CSU is driven by the activation of mast cells, which release histamines and other immune modulators, although the precise mechanism is not fully known. In this section we present the current understanding of the molecular and immune processes involved in urticaria.

 

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Symptoms

The symptoms of chronic spontaneous urticaria (CSU) include itchy hives (wheals) and angioedema.

 

The symptoms of CSU may appear without warning with a variable intensity (Maurer et al., 2011; Zuberbier et al., 2018) and may profoundly impact patients' day-to-day lives (Maurer et al., 2011; O'Donnell et al., 1997; Kang et al., 2009; Barbosa et al., 2011; Engin et al., 2008). In CSU, itchy hives, angioedema or both, may occur spontaneously every day, or almost daily for 6 weeks or more (Zuberbier et al., 2018).

 

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Diagnosis and Assessment

Since there is no definitive test for chronic spontaneous urticaria, diagnosis is based on a thorough medical history and physical examination as well as diagnostic tests. 

In this section we describe the steps required to diagnose the disease, the diagnostic markers to be aware of and other conditions which can cause hives and angioedema.

 

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Comorbidities

 

Unfortunately for many patients with CSU, the itchy hives and/or angioedema associated with the condition is not all they have to contend with. A substantial number of patients also experience comorbidities associated with the development of CSU.

 

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Treatment

The Aim of Treatment
The aim of treatment for urticaria is quick and complete symptom control (Maurer et al., 2011; Maurer et al., 2015; Zuberbier et al., 2018).

The Recommended Treatment Algorithm

The 2013 EAACI/GA2LEN/EDF/WAO guidelines recommend the following step-wise approach to the treatment of urticaria (Zuberbier et al., 2018).

 

The 2013 EAACI/GA2LEN/EDF/WAO recommended treatment algorithm.

Figure 1. The 2017 EAACI/GA2LEN/EDF/WAO recommended treatment algorithm.
EAACI, European Academy of Allergy and Clinical Immunology: EDF, European Dermatology Forum; GA2LEN, Global Allergy and Asthma European Network; WAO. World Allergy Organisation
A number of additional treatment options are mentioned in the EAACI/GA2LEN/EDF/WAO guidelines, but are not included in the recommended treatment algorithm due to limited supporting evidence.

 

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Guidelines

Best practice guidelines for CSU have been developed and published by a number of national and international groups, and those with major significance are described in this section. Broadly speaking they recommend second generation antihistamines of standard and then increased dose, followed by alternative agents such as anti-inflammatories, immunosuppressants or biologics.

 

CSU presents as wheals and/or angioedema which usually last for less than 24 hours and resolve without leaving a mark.  These symptoms occur for 6 weeks or more (Zuberbier et al., 2018).

 

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Paediatric Urticaria

 

Having explored adult urticaria, in this section of the Learning Zone, we review the available data in paediatric urticaria and establish the impact it has on younger patients and how diagnosis and management compares to the recommendations for adult patients with CSU.

 

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Welcome:

Developed by EPG Health for Medthority in collaboration with Novartis Pharma AG, with some content provided by Novartis Pharma AG. An unrestricted educational grant was provided from Novartis Pharma AG for the development of the e-Learning module within this Learning Zone (Novartis were not involved in the content or development of these modules). Developed by EPG Health for Medthority in collaboration with J. Uriach y Compañía S.A, with some content provided by J. Uriach y Compañía S.A.