About the Authors

Marcus Maurer

Marcus.Maurer@charite.de

Affiliation Department of Dermatology and Allergy, Allergie-Centrum-Charité, Charité - Universitätsmedizin Berlin, Berlin, Germany

Werner Aberer

Contributed equally to this work with: Werner Aberer, Laurence Bouillet, Teresa Caballero, Vincent Fabien, Gisèle Kanny, Hilary Longhurst, Andrea Zanichelli

Affiliation Department of Dermatology and Venerology, Medical University of Graz, Graz, Austria

Laurence Bouillet

Contributed equally to this work with: Werner Aberer, Laurence Bouillet, Teresa Caballero, Vincent Fabien, Gisèle Kanny, Hilary Longhurst, Andrea Zanichelli

Affiliation National Reference Centre for Angioedema, Internal Medicine Department, Grenoble University Hospital, Grenoble, France

Teresa Caballero

Contributed equally to this work with: Werner Aberer, Laurence Bouillet, Teresa Caballero, Vincent Fabien, Gisèle Kanny, Hilary Longhurst, Andrea Zanichelli

Affiliation Hospital La Paz Health Research Institute (IdiPaz), Biomedical Research Network on Rare Diseases (CIBERER, U754), Madrid, Spain

Vincent Fabien

Contributed equally to this work with: Werner Aberer, Laurence Bouillet, Teresa Caballero, Vincent Fabien, Gisèle Kanny, Hilary Longhurst, Andrea Zanichelli

Affiliation Shire Human Genetic Therapies, Eysins, Switzerland

Gisèle Kanny

Contributed equally to this work with: Werner Aberer, Laurence Bouillet, Teresa Caballero, Vincent Fabien, Gisèle Kanny, Hilary Longhurst, Andrea Zanichelli

Affiliation EA3999 ‘Allergic Disease: Diagnosis and Therapeutic’, University of Lorraine, Faculty of Medicine, Vandoeuvre-les-Nancy, France

Allen Kaplan

Affiliation Medical University of South Carolina, Charleston, South Carolina, United States of America

Hilary Longhurst

Contributed equally to this work with: Werner Aberer, Laurence Bouillet, Teresa Caballero, Vincent Fabien, Gisèle Kanny, Hilary Longhurst, Andrea Zanichelli

Affiliation Department of Immunology, Bart’s and The London Hospital, London, United Kingdom

Andrea Zanichelli

Contributed equally to this work with: Werner Aberer, Laurence Bouillet, Teresa Caballero, Vincent Fabien, Gisèle Kanny, Hilary Longhurst, Andrea Zanichelli

Affiliation Department of Clinical Sciences, Luigi Sacco University of Milan - Ospedale Luigi Sacco Milano, Milan, Italy

on behalf of I. O. S. Investigators

Competing Interests

Marcus Maurer has received speaker/consultancy fees from Biocryst, Shire HGT, Inc./Jerini AG and ViroPharma. Werner Aberer has acted as a medical advisor and speaker for Shire HGT, Inc. and CSL Behring; received funding to attend conferences and other educational events; received donations to his departmental fund, and participated in clinical trials for Shire HGT, Inc. Laurence Bouillet has received honoraria from Shire HGT, Inc., CSL Behring, Pharming and ViroPharma. Teresa Caballero has received speaker fees from Shire HGT, Inc./Jerini AG and ViroPharma; consultancy fees from Shire HGT, Inc./Jerini AG, ViroPharma and CSL Behring; funding for travel and meeting attendance from CSL Behring and Shire HGT, Inc.; and has participated in clinical trials for Dyax, Pharming, CSL Behring and Shire HGT, Inc./Jerini AG. Vincent Fabien is an employee of Shire Human Genetic Therapies, Eysins, Switzerland. Gisèle Kanny has received donations to her Research Unit and participated in clinical trials for Shire HGT, Inc. Allen Kaplan has received consultancy fees from CSL Behring and Dyax; research grants from Shire HGT, Inc. and Dyax; and has received payment for lectures including service on speakers bureaus from ViroPharma and CSL Behring. Hilary Longhurst has received funding for research and staff support from CSL Behring, Shire HGT, Inc., and Pharming; is a consultant for CSL Behring, Shire HGT Inc. and Swedish Orphan; and a speaker for CSL Behring, Shire HGT, Inc., Swedish Orphan and ViroPharma. Andrea Zanichelli is an advisor for Shire HGT, Inc. and has been an invited speaker for CSL Behring and Shire HGT, Inc. The authors confirm that the above Conflicts of Interest do not alter their adherence to all the PLOS ONE policies on sharing data and materials. The funders had no role in study design, data collection and analysis, decision to publish, or preparation of the manuscript.

Author Contributions

Drafted the manuscript and revised it critically for important intellectual content and final approval of the version to be published: MM WA LB TC VF GK AK HL AZ. Responsible for the decision to publish: MM WA LB TC VF GK AK HL AZ. Performed the experiments: MM WA LB TC GK HL AZ. Analyzed the data: MM WA LB TC VF GK HL AZ.