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Hereditary angioedema (HAE) is a rare hereditary disease. The disease is characterised by unpredictable and recurring swelling attacks (edema) in the hands, feet, face larynx, genital organs and abdomen. The attacks can be extremely painful. Untreated acute episodes in the larynx can even become potentially life-threatening. In most cases the underlying genetic defect causes a C1 esterase inhibitor (C1-INH) deficiency in the blood plasma of those affected.
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All about HAE
HAE ... stands for hereditary angioedema
HAE affects only 1 in 10,000 to 1 in 50,000 people. However, experts believe that this maybe an underestimate: although HAE is (in principle) easy to diagnose, it is frequently identified very late or not discovered at all. By the time it is diagnosed correctly, the patient has often been through a long lasting ordeal.
An overview of the clinical picture as well as information about diagnosis and current treatment options can be found here.
| Frequently inquired |
Author: Jerini AG
Source: HAE-Network Status: 3/08 |
