OBJECTIVE: To review the evolution of our understanding of hereditary angioedema (HAE) from the first historical reference to the present day.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. [References: 65]. ”
Jan;100(1 Suppl 2):S2-6
Available online at: http://www.annallergy.org/article/S1081-1206%2810%2960579-0/fulltext (small fee)