HAE International Community

History of Hereditary AngioEdema

The history of hereditary angioedema starting with Irenäus Quincke.

The clinical picture was first described in 1482 by Heinrich Irenäus Quincke, a physician from Kiel, Germany, and was at that time called Quincke’s edema. The older literature also refers to the disease as hereditary angioneurotic edema (HANE). However, as the origin (pathogenesis) of hereditary angioedema is not influenced by neurological factors, it has gradually become known as “Hereditary AngioEdema” (HAE).

The majority of HAE cases are caused by a defect in the gene that controls a blood protein called C1-esterase inhibitor (C1-INH).

Time table

1882 - Heinrich Irenäus Quincke initially describes 'Quincke’s edema'.

1888 - HAE first described by William Osler in a 18-year old woman with episodic attacks, from five successive generations with attacks. HAE was then described as angioneurotic edema

1913 - Crowder and Crowder reported that the condition is inherited as an autosomal dominant trait (it’s located on one of the 16 pairs of chromosomes called autosomes and is inherited independent of the sex)

1963 - Donaldson and Evans showed that HAE is linked to a C1-INH deficiency

1986 - C1-INH gene localized on chromosome 11

1986 - First case of acquired angioedema (AAE) caused by anti-C1-INH antibodies described

1998 - Bradykinin appears to be main mediator of angioedema(3)