|
||||||||||||||
 |
||||||||||||||
|
|
Hereditary angioedema: A decade of human C1-inhibitor concentrate therapyHave you seen this woman? Many in the allergy community would recognize her as possibly having hereditary angioedema (HAE). Hereditary angioedema is a rare, potentially fatal genetic disorder typified by a deficiency or dysfunction of the plasma protein C1 Inhibitor, and characterized by sudden, recurrent edematous swellings of the subcutaneous or submucosal tissues of the extremities, face, trunk, abdominal viscera, and upper airway. While abdominal attacks can cause severe pain, swelling of the upper airways can be deadly. Mortality from suffocation in this condition due to airway closure has been reported to be as high as 30%. Delays in establishing an accurate diagnosis and introducing appropriate therapy are linked to death. Although urticaria and angioedema are fairly common problems that affect nearly 20% of the population, Hereditary Angioedema (HAE) is an extremely rare disease that accounts for about 2% of clinical angioedema cases. We would usually employ antihistamines, glucocorticoids, and epinephrine to treat angioedema, but these agents are ineffective in angioedema that is caused by C1-INH deficiency. In the October 2007 issue of the Journal of Allergy and Clinical Immunology, Farkas et al summarize more than 10 years of experience reflecting the effectiveness and safety of hC1-INH concentrate, a highly purified protein derived from human plasma that can be successfully used to treat patients who cannot produce their own C1 inhibitor. In this article the experience of Farkas, et al, accumulated over a period of 10 years confirms that hC1-INH concentrate is currently the treatment of choice for controlling severe HAE attacks. In addition, it proves highly effective and safe for short-term prophylaxis, as well as for the treatment of children and pregnant women; hC1-INH concentrate has no contraindications. The trick is knowing when to use it. Although hC1-INH is not yet available in many countries, it is hoped that steps will be taken quickly to provide it for all patients with HAE, to reduce the risks of their disease and improve the quality of their lives. |
||||||||||||
|
© 1996-2008 · All Rights Reserved · American Academy of Allergy Asthma & Immunology |
||||||||||||||
