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The history of hereditary angioedema starting with Irenäus Quincke
The clinical picture of hereditary angioedema (HAE) was first described in 1882 by Heinrich Irenäus Quincke, a physician from Kiel, and at that time it was called Quincke’s edema. The older literature also refers to the disease as hereditary angioneurotic edema (HANE). Today it is known as “hereditary angioedema” (HAE).
HAE is caused by an autosomal dominantly inherited change in a gene on chromosome 11, which is responsible for production of the inhibitor of an enzyme, C1 esterase inhibitor. As a result, only a reduced or partially inactive form of this protein is produced leading to an increased level of bradykinin.
Time table
HAE is caused by an autosomal dominantly inherited change in a gene on chromosome 11, which is responsible for production of the inhibitor of an enzyme, C1 esterase inhibitor. As a result, only a reduced or partially inactive form of this protein is produced leading to an increased level of bradykinin.
Time table
- 1882 - Heinrich Irenäus Quincke initially describes 'Quincke’s edema'.
- 1888 - HAE first described by Osler in a 24-year old woman with episodic attacks, from five successive generations with attacks. HAE was then described as angio-neurotic edema
- 1917 - Crowder and Crowder reported that the condition is inherited as an autosomal dominant trait
- 1963 - Donaldson and Evans showed that HAE is linked to a C1INH deficiency
- 1986 - C1INH gene discovered on chromosome 11
- 1986 - First case of acquired angioedema (AAE) caused by anti-C1INH antibodies
- 1998 - Bradykinin appears to be main mediator of angioedema
