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HAE international home therapy consensus document

Hilary J Longhurst1*, Henriette Farkas2, Timothy Craig3, Emel Aygören-Pürsün4, Claire Bethune5, Janne Bjorkander6, Konrad Bork7, Laurence Bouillet8, Henrik Boysen9, Anette Bygum10, Teresa Caballero11, Marco Cicardi12, John Dempster13, Mark Gompels14, Jimmy Gooi15, Sofia Grigoriadou16, Ursula Huffer17, Wolfhart Kreuz18, Marcel M Levi19, Janet Long20, Inmaculada Martinez-Saguer21, Michel Raguet22, Avner Reshef23, Tom Bowen24 and Bruce Zuraw25

Author Affiliations

1 Department of Immunology, Barts and the London NHS Trust, London, UK

2 3rd Department of Internal Medicine, Faculty of Medicine, Semmelweis University, Budapest, Hungary

3 Departments of Medicine and Pediatrics, Penn State University, Hershey, Pennsylvania, USA

4 Johann Wolfgang Goethe University, Frankfurt/Main, Germany

5 Department of Immunology, Plymouth Hospitals NHS Trust, UK

6 Dept of Internal Medicin, Ryhov County Hospital, SE-55185 Jönköping, Sweden

7 Department of Dermatology, University Hospital of the Johannes Gutenberg-University of Mainz, Mainz, Germany

8 Department of Medicine, CHU de Grenoble, Grenoble, France

9 Executive Director, HAE International, Denmark

10 Department of Dermatology and Allergy Centre, Odense University Hospital, Denmark

11 Hospital La Paz Health Research Institute, Madrid, Spain

12 Department of Internal Medicine, Universita degli Studi di Milano, Ospedale L. Sacco, Milan, Italy

13 Department of Immunology, Barts and the London NHS Trust, London, UK

14 Department of Immunology, Southmead Hospital, Bristol, UK

15 Department of Immunology, St James' Hospital, Leeds, UK

16 Department of Immunology, Barts and the London NHS Trust, London, UK

17 HAE association, Germany

18 Johann Wolfgang Goethe University, Frankfurt/Main, Germany

19 Academic Medical Center, University of Amsterdam, Amsterdam, Netherlands

20 US HAEA Executive Vice President; US HAEA Patient Registry, USA

21 Johann Wolfgang Goethe University, Frankfurt/Main, Germany

22 HAE Association, France

23 Tel Hashomer, and Sackler Faculty of Medicine, Tel Aviv University, Ramat Aviv, Israel

24 Departments of Medicine and Paediatrics, University of Calgary, Calgary, Alberta, Canada

25 University of California, San Diego, San Diego, California, USA

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Allergy, Asthma & Clinical Immunology 2010, 6:22  doi:10.1186/1710-1492-6-22

Published: 28 July 2010


Hereditary angioedema (C1 inhibitor deficiency, HAE) is associated with intermittent swellings which are disabling and may be fatal. Effective treatments are available and these are most useful when given early in the course of the swelling. The requirement to attend a medical facility for parenteral treatment results in delays. Home therapy offers the possibility of earlier treatment and better symptom control, enabling patients to live more healthy, productive lives. This paper examines the evidence for patient-controlled home treatment of acute attacks ('self or assisted administration') and suggests a framework for patients and physicians interested in participating in home or self-administration programmes. It represents the opinion of the authors who have a wide range of expert experience in the management of HAE.