I Leonard Bernstein1. 1. Allergy Section, Division of Immunology, Department of Internal Medicine, University of Cincinnati College of Medicine, Ohio 45267-0563, USA. i.leonard.bernstein@uc.edu
Abstract
OBJECTIVE: To review the evolution of our understanding of hereditary angioedema (HAE) from the first historical reference to the present day. DATA SOURCES: MEDLINE and PubMed were searched using the following keywords: history of HAE, C1 inhibitor, complements system, genetics of HAE, mechanisms of HAE, and treatment of HAE. STUDY SELECTION: Information was selected that outlines the advances made in complementology, the first report of HAE, and subsequent studies that elucidated the underlying mechanisms of this disease, leading to current therapy of this orphan disease. RESULTS: Generational research efforts in HAE have focused on the following: (1) several new clinical presentations, (2) acquired forms of non-histamine-induced angioedema, (3) the genetic basis for the inherited forms, (4) the effects of C1 inhibitor on contact phases of coagulation-fibrinolytic pathways, and (5) various therapies for short- and long-term control of the disease. CONCLUSION: The progress made in understanding the pathogenesis and treatment of HAE is an excellent example of the "bench to the bedside" paradigm involving the collaboration between clinicians and researchers.
OBJECTIVE: To review the evolution of our understanding of hereditary angioedema (HAE) from the first historical reference to the present day. DATA SOURCES: MEDLINE and PubMed were searched using the following keywords: history of HAE, C1 inhibitor, complements system, genetics of HAE, mechanisms of HAE, and treatment of HAE. STUDY SELECTION: Information was selected that outlines the advances made in complementology, the first report of HAE, and subsequent studies that elucidated the underlying mechanisms of this disease, leading to current therapy of this orphan disease. RESULTS: Generational research efforts in HAE have focused on the following: (1) several new clinical presentations, (2) acquired forms of non-histamine-induced angioedema, (3) the genetic basis for the inherited forms, (4) the effects of C1 inhibitor on contact phases of coagulation-fibrinolytic pathways, and (5) various therapies for short- and long-term control of the disease. CONCLUSION: The progress made in understanding the pathogenesis and treatment of HAE is an excellent example of the "bench to the bedside" paradigm involving the collaboration between clinicians and researchers.
Authors: Tom Bowen; Marco Cicardi; Henriette Farkas; Konrad Bork; Hilary J Longhurst; Bruce Zuraw; Emel Aygoeren-Pürsün; Timothy Craig; Karen Binkley; Jacques Hebert; Bruce Ritchie; Laurence Bouillet; Stephen Betschel; Della Cogar; John Dean; Ramachand Devaraj; Azza Hamed; Palinder Kamra; Paul K Keith; Gina Lacuesta; Eric Leith; Harriet Lyons; Sean Mace; Barbara Mako; Doris Neurath; Man-Chiu Poon; Georges-Etienne Rivard; Robert Schellenberg; Dereth Rowan; Anne Rowe; Donald Stark; Smeeksha Sur; Ellie Tsai; Richard Warrington; Susan Waserman; Rohan Ameratunga; Jonathan Bernstein; Janne Björkander; Kristylea Brosz; John Brosz; Anette Bygum; Teresa Caballero; Mike Frank; George Fust; George Harmat; Amin Kanani; Wolfhart Kreuz; Marcel Levi; Henry Li; Inmaculada Martinez-Saguer; Dumitru Moldovan; Istvan Nagy; Erik W Nielsen; Patrik Nordenfelt; Avner Reshef; Eva Rusicke; Sarah Smith-Foltz; Peter Späth; Lilian Varga; Zhi Yu Xiang Journal: Allergy Asthma Clin Immunol Date: 2010-07-28 Impact factor: 3.406