Markus Magerl

WAO Guideline for the Management of Hereditary Angioedema

Hereditary Angioedema (HAE) is a rare disease and for this reason proper diagnosis and appropriate therapy are often unknown or not available for physicians and other health care providers. For this reason we convened a group of specialists that focus upon HAE from around the world to develop not only a consensus on diagnosis and management of HAE, but to also provide evidence based grades, strength of evidence and classification for the consensus. Since both consensus and evidence grading were adhered to the document meets criteria as a guideline. The outcome of the guideline is to improve diagnosis and management of patients with HAE throughout the world and to help initiate uniform care and availability of therapies to all with the diagnosis no matter where the residence of the individual with HAE exists.

Successful treatment of solar urticaria with anti- immunoglobulin E therapy

anaesthesiologists and oncologists), practising doctors treated anaphylactic patients regularly. The average number of treated patients per practice was low, but considering the high number of practising doctors, they deliver a broad number of anaphylactic cases. The reported triggers of anaphylaxis are in line with data from the literature. However, the most common trigger factor of anaphylaxis reported by these doctors, who perform SCIT on a regular basis, was SCIT. This observation points to the necessity of gaining more information on severe reactions during SCIT, which are probably underreported. There is a possibility that the reported anaphylactic reactions were not diagnosed properly, as only 19% of the practitioners were specialized in allergy. A recall-bias also needs consideration. Still, our data clearly show that SCIT seems to be an important cause of anaphylaxis in private practice and are in line with a previous survey from Bernstein et al. (4). In conclusion, anaphylaxis is relevant in private practices and more epidemiological data are urgently needed.

Quality of life in patients with chronic urticaria is differentially impaired and determined by psychiatric comorbidity

Background  Chronic urticaria (CU), one of the most common skin disorders, is characterized by spontaneous recurrent bouts of weals and pruritus and associated with severely impaired quality of life (QoL).

The definition and diagnostic testing of physical and cholinergic urticarias--EAACI/GA2LEN/EDF/UNEV consensus panel recommendations.

The recommendations for the definition and diagnosis presented in this position paper are the result of a panel consensus meeting held in December 2008 in Berlin. This consensus meeting was a joint initiative of EAACI (European Academy of Allergology and Clinical Immunology) Dermatology Section, the EU‐funded network of excellence, GA2LEN (Global Allergy and Asthma European Network), the EDF (European Dermatology Forum) and UNEV (urticaria network e.V.). The aim of these recommendations is to improve the diagnosis and management of patients with physical urticaria or cholinergic urticaria and to promote research and a better understanding of these diseases. Our recommendations used the paper produced by a 1996 expert meeting ( 1 ) and they acknowledge the latest changes in our understanding of physical urticarias and cholinergic urticaria as well as the recent development of novel diagnostic tools. In addition, this consensus paper highlights areas of need for further research.

Hereditary angioedema with normal C1 inhibitor function: consensus of an international expert panel.

A new form of hereditary angioedema (HAE) with normal C1 inhibitor (C1INH) was first described in 2000. The lack of clear diagnostic criteria, the heterogeneity among affected patients, and the varying names given to this disease have led to substantial confusion among both physicians and patients. This study was designed to bring more clarity to the diagnosis and potential treatment of HAE with normal C1INH. An international symposium of experts was convened to review the field and develop consensus opinions that could help clinicians who evaluate and manage these patients. Criteria were developed for the diagnosis of HAE with normal C1INH in patients with recurrent angioedema in the absence of concurrent urticaria. In addition, potential therapeutic strategies are discussed. The consensus criteria developed during this symposium will allow physicians to better diagnose and treat patients with HAE with normal C1INH.

The German version of the Chronic Urticaria Quality-of-Life Questionnaire: factor analysis, validation, and initial clinical findings.

Background:  Chronic urticaria (CU) is a common skin disorder that causes a substantial burden on patients’ quality‐of‐life (QoL). The aim of this work was to generate and validate a German version of the Chronic Urticaria Quality of Life Questionnaire (CU‐Q2oL) and to provide reference assessments of QoL.

Anti-Immunoglobulin E Treatment of Patients with Recalcitrant Physical Urticaria

In physical urticaria, exogenous physical factors such as thermal triggers, solar radiation and mechanic triggers including friction or pressure are responsible for the elicitation of symptoms in the skin of patients. Avoidance of the respective stimulus is usually difficult or impossible, and many patients are not sufficiently treated with standard antihistamines. We report that treatment with omalizumab (Xolair®) of 7 patients with physical urticarias [solar urticaria (n = 2), urticaria factitia/symptomatic dermographism (n = 2), cold urticaria, delayed pressure urticaria and localized heat urticaria] resulted in complete symptom control within days after the first injection in 5 patients. In 1 patient, symptoms improved after increasing the dose of omalizumab, and 1 patient with localized heat urticaria did not respond significantly to treatment. Before anti-immunoglobulin E treatment, all patients had suffered from their physical urticaria for years and had had numerous unsuccessful therapies. The overall excellent responses to omalizumab treatment reported here indicate that anti-immunoglobulin E is a safe and effective treatment for recalcitrant physical urticarias.

New topics in bradykinin research

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.

Development and construct validation of the angioedema quality of life questionnaire

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.

Effects of a pseudoallergen-free diet on chronic spontaneous urticaria: a prospective trial

Background:  Chronic spontaneous urticaria is a skin disorder that is difficult to manage and can last for years. ‘Pseudoallergens’ are substances that induce hypersensitive/intolerance reactions that are similar to true allergic reactions. They include food additives, vasoactive substances such as histamine, and some natural substances in fruits, vegetables and spices. Eliminating pseudoallergens from the diet can reduce symptom severity and improve patient quality of life.