Hereditary Angioedema (HAE) is a rare and disabling disease. Early diagnosis and appropriate therapy are essential. This update and revision of the global guideline for HAE provides up‐to‐date ...consensus recommendations for the management of HAE. In the development of this update and revision of the guideline, an international expert panel reviewed the existing evidence and developed 20 recommendations that were discussed, finalized and consented during the guideline consensus conference in June 2016 in Vienna. The final version of this update and revision of the guideline incorporates the contributions of a board of expert reviewers and the endorsing societies. The goal of this guideline update and revision is to provide clinicians and their patients with guidance that will assist them in making rational decisions in the management of HAE with deficient C1‐inhibitor (type 1) and HAE with dysfunctional C1‐inhibitor (type 2). The key clinical questions covered by these recommendations are: (1) How should HAE‐1/2 be defined and classified?, (2) How should HAE‐1/2 be diagnosed?, (3) Should HAE‐1/2 patients receive prophylactic and/or on‐demand treatment and what treatment options should be used?, (4) Should HAE‐1/2 management be different for special HAE‐1/2 patient groups such as pregnant/lactating women or children?, and (5) Should HAE‐1/2 management incorporate self‐administration of therapies and patient support measures?
Background
Recurrent angioedema is a frequent clinical problem characterized by unpredictably and rapidly occurring cutaneous and mucosal swellings. These swellings may be painful and/or disfiguring. ...Upper airway involvement can also lead to dyspnea and suffocation. Although the disease burden is high, there is currently no specific instrument to measure health‐related quality of life (QoL) impairment.
Objective
To develop and validate the first symptom‐specific tool to assess QoL impairment in recurrent angioedema patients, adhering to established methodological recommendations.
Methods
During the development phase, 29 questions (items) were generated. Subsequently, item reduction was performed by means of impact analysis and factor analysis as well as by checking for content and face validity. As a result, 17 items were selected and included in the final instrument, the Angioedema QoL Questionnaire (AE‐QoL). AE‐QoL was then tested for its validity, reliability, and influence factors.
Results
One hundred and ten angioedema patients took part in the validation of AE‐QoL. AE‐QoL was found to have a four‐dimensional structure as well as a valid total score. All of its four domains (functioning, fatigue/mood, fears/shame, food) showed good levels of internal consistency with Cronbach's alpha > 0.8. Test–retesting revealed a good reliability of the instruments total score and domain scores. Gender as well as the patients' self‐rated disease activity was found to be predictors of the AE‐QoL total score.
Conclusions
Angioedema Quality of Life Questionnaire is the first angioedema‐specific QoL questionnaire. It is a short, valid and reliable instrument that may serve as a valuable tool in future clinical studies and in routine patient care.
This evidence‐ and consensus‐based guideline was developed following the methods recommended by Cochrane and the Grading of Recommendations Assessment, Development and Evaluation (GRADE) working ...group. The conference was held on 1 December 2016. It is a joint initiative of the Dermatology Section of the European Academy of Allergology and Clinical Immunology (EAACI), the EU‐founded network of excellence, the Global Allergy and Asthma European Network (GA²LEN), the European Dermatology Forum (EDF) and the World Allergy Organization (WAO) with the participation of 48 delegates of 42 national and international societies. This guideline was acknowledged and accepted by the European Union of Medical Specialists (UEMS). Urticaria is a frequent, mast cell‐driven disease, presenting with wheals, angioedema, or both. The lifetime prevalence for acute urticaria is approximately 20%. Chronic spontaneous urticaria and other chronic forms of urticaria are disabling, impair quality of life and affect performance at work and school. This guideline covers the definition and classification of urticaria, taking into account the recent progress in identifying its causes, eliciting factors and pathomechanisms. In addition, it outlines evidence‐based diagnostic and therapeutic approaches for the different subtypes of urticaria.
Background
Recurrent angioedema (RecA) is a frequent clinical problem characterized by suddenly occurring cutaneous and/or mucosal swellings. Depending on their location, RecA may be painful, ...hindering, disfiguring, or even life‐threatening. The assessment of disease activity in affected patients is important to guide treatment decisions. Currently, however, there is no standardized and validated outcome measure available to do so.
Objective
To develop and validate the first specific patient‐reported outcome instrument to assess disease activity in RecA patients, the Angioedema Activity Score (AAS).
Methods
After a set of potential AAS items was developed, item evaluation and reduction were performed by means of impact analysis, factor analysis, regression analysis, and by checking for face validity. In addition, the items of the final AAS questionnaire were tested for their validity and reliability during a 12‐week validation study.
Results
In total, data from 110 and 80 RecA patients were used during the AAS item evaluation and validation phase, respectively. The resulting AAS consisted of five items and was found to have a one‐dimensional structure and excellent internal consistency. It correlated well with other measures of disease activity and quality‐of‐life impairment, thus demonstrating its convergent validity. In addition, the known‐groups validity and test–retest reliability of the AAS were found to be good.
Conclusions
The AAS is the first validated and reliable tool to determine disease activity in RecA patients, and it may serve as a valuable instrument in future clinical studies and routine patient care.
These recommendations for the definition, diagnosis and management of chronic inducible urticaria (CIndU) extend, revise and update our previous consensus report on physical urticarias and ...cholinergic urticaria (Allergy, 2009). The aim of these recommendations is to improve the diagnosis and management of patients with CIndU. Our recommendations acknowledge the latest changes in our understanding of CIndU, and the available therapeutic options, as well as the development of novel diagnostic tools.
New topics in bradykinin research Maurer, M.; Bader, M.; Bas, M. ...
Allergy,
November 2011, Letnik:
66, Številka:
11
Journal Article
Recenzirano
Odprti dostop
To cite this article: Maurer M, Bader M, Bas M, Bossi F, Cicardi M, Cugno M, Howarth P, Kaplan A, Kojda G, Leeb‐Lundberg F, Lötvall J, Magerl M. New topics in bradykinin research. Allergy 2011; 66: ...1397–1406.
Bradykinin has been implicated to contribute to allergic inflammation and the pathogenesis of allergic conditions. It binds to endothelial B1 and B2 receptors and exerts potent pharmacological and physiological effects, notably, decreased blood pressure, increased vascular permeability and the promotion of classical symptoms of inflammation such as vasodilation, hyperthermia, oedema and pain. Towards potential clinical benefit, bradykinin has also been shown to exert potent antithrombogenic, antiproliferative and antifibrogenic effects. The development of pharmacologically active substances, such as bradykinin receptor blockers, opens up new therapeutic options that require further research into bradykinin. This review presents current understanding surrounding the role of bradykinin in nonallergic angioedema and other conditions seen by allergists and emergency physicians, and its potential role as a therapeutic target.
GA²LEN, the Global Allergy and Asthma European Network, has recently launched a program for the development, interaction, and accreditation of centers of reference and excellence in special areas of ...allergy embedded in its overall quality management of allergy centers of excellence. The first area chosen is urticaria. Urticaria is a common and debilitating condition and can be a challenge for both patients and treating physicians, especially when chronic. Centers of reference and excellence in urticaria (UCAREs) can help to improve the management of hard-to-treat conditions such as urticaria.
Here, we describe the aims, the requirements and deliverables, the application process, and the audit and accreditation protocol for GA²LEN UCAREs.
The main aims of GA²LEN UCAREs are to provide excellence in urticaria management, to increase the knowledge of urticaria by research and education, and to promote the awareness of urticaria by advocacy activities. To become a certified GA²LEN UCARE, urticaria centers have to apply and fulfill 32 requirements, defined by specific deliverables that are assessed during an audit visit.
The GA²LEN UCARE program will result in a strong network of urticaria specialists, promote urticaria research, and harmonize and improve urticaria management globally.
Background
Autoimmune processes are considered to play a major role in the pathogenesis of chronic spontaneous urticaria (CSU). Very recently, interleukin 24 (IL‐24) has been identified as an ...immunoglobulin E (IgE) autoantigen in CSU. Some studies revealed that notably autologous serum skin test (ASST)‐positive CSU patients may benefit from autohemotherapy; however, the mechanisms of action remain unknown. We aimed to investigate the immunological effects of autologous serum injections in ASST‐positive CSU patients.
Methods
Sixty‐six ASST‐positive CSU patients were treated with weekly intramuscular autologous serum injections for 8 weeks and followed up for 12 weeks. Urticaria activity score (UAS7) and Dermatology Life Quality Index (DLQI) were assessed. The ASST was done at baseline, week 9 and week 21. Serum samples (baseline, weeks 9, 13 and/or 21) were analysed for the levels of IgE‐anti‐IL‐24 and immunoglobulin G (IgG)‐anti‐IL‐24 via ELISA and their ability to release histamine in basophils basophil histamine release assay (BHRA).
Results
Autologous serum therapy resulted in a substantial improvement in disease activity and quality of life after 8 and 20 weeks. Twenty‐eight percent and 34% of patients turned ASST‐negative in weeks 9 and 21, respectively, but there was no link between their response to treatment and changes of ASST results. Also, no significant or relevant changes in BHRA were observed. In contrast, autologous serum therapy significantly decreased IgE‐anti‐IL‐24 serum levels, but not IgG‐anti‐IL‐24 serum levels, in responders but not in non‐responders.
Conclusions
Our findings suggest that the immunological effects of autologous serum therapy include a reduction in IgE‐anti‐IL24 autoantibodies, which may contribute to the pathogenesis of CSU.
Background
Cold‐ and heat‐induced urticaria are chronic physical urticaria conditions in which wheals, angioedema or both are evoked by skin exposure to cold and heat respectively. The diagnostic ...work up of both conditions should include skin provocation tests and accurate determination of critical temperature thresholds (CTT) for producing symptoms in order to be able to predict the potential risk that each individual patient faces and how this may be ameliorated by therapy.
Objective
To develop and validate TempTest® 4, a simple and relatively inexpensive instrument for the accurate determination of CTT which may be used in clinical practice.
Methods
TempTest® 4 has a single 2 mm wide 350 mm U‐shaped Peltier element generating a temperature gradient from 4 °C to 44 °C along its length. Using a clear plastic guide placed over the skin after provocation, CTT values may be determined with an accuracy of ±1 °C. Here, TempTest® 4 was compared with its much more expensive predecessor, TempTest® 3, in inducing wheals in 30 cold urticaria patients.
Results
Both TempTest® 4 and TempTest® 3 induced wheals in all 30 patients between 8 ° and 28 °C. There was a highly significant (P < 0.0001) correlation between the instruments in the CTT values in individual patients.
Conclusion
The TempTest® 4 is a simple, easy to use, licensed, commercially available and affordable instrument for the determination of CTTs in both cold‐ and heat‐induced urticaria.
Background
Chronic urticaria is a common disorder characterized by recurrent wheals, angioedema, or both. Several differential diagnoses need to be considered in patients presenting with wheals ...and/or angioedema. These include rare diseases such as autoinflammatory syndromes and urticarial vasculitis in patients with recurrent wheals and bradykinin‐mediated angioedema in patients with recurrent swellings.
Aim and Result
In order to not miss these conditions, we have developed a symptom‐based diagnostic algorithm for the management of patients with wheals and/or angioedema.
Discussion and Conclusion
By asking the right questions and performing a limited diagnostic workup as suggested here, this algorithm may help to establish the right diagnosis and treat patients early and more effectively.
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