What Is Hereditary Angioedema and Its Causes

What Is Hereditary Angioedema and Its Causes

Hereditary Angioedema (HAE) is a rare disorder that causes the swelling of blood vessels, particularly those in the face, hands, and lower legs. Many causes of this disorder include injury or genetic predisposition to swollen blood vessels. Hereditary Angioedema affects certain blood groups and medications and causes a deficiency in the y C1-esterase inhibitor. Most people with HAE cannot control it and require medical treatment.


The pathophysiology of HAE is the reason for the swelling of blood vessels during this disorder. The body's immune system attacks the walls of blood vessels, causing them to become inflamed. This inflammation causes swelling in the face, hands and legs that often occurs during an attack. The immune system may also attack red blood cells in the blood vessels causing them to become damaged and subsequently die.


Hereditary Angioedema has two forms: type 1 and type 2. In the most severe form, type 1, attacks can occur anytime during a person's life. This disorder is also known as idiopathic Angioedema (IAE). In type 2, a trigger must be present for the attacks to occur. Acquired Angioedema (AAE), also known as type 2 HAE, is triggered by medications, infection, and surgery. Type 1 IAE does not have triggers and is the less severe form of this disorder. In some cases, a person can have symptoms of both types of the disorder.


The symptoms of HAE result from too much fluid in the body. Fluid overload can occur in different tissues throughout the body, including the abdomen, airway, chest, colon and skin. These symptoms include blood and protein in stool, abdominal swelling and itching. The swelling of the blood vessels in the body can cause severe pain and skin blisters, known as hives. In rare cases, people with this disease can die from a ruptured intestine or airway swelling.


HAE often occurs during adolescence and peaks between twenty and thirty. In most cases, physicians can control attacks using steroids, antihistamines and anti-inflammatory drugs. Corticosteroids reduce the swelling and fluid in the body, while antihistamines stop itching.


People with this disorder need to visit a doctor regularly for evaluation. Attacks are unpredictable and can occur at any time. The development of a long-term treatment program is also important. If left untreated, attacks can be life-threatening and may result in damage to major organs. There is no cure for HAE, and medical treatment is the only way to prevent or stop swelling from occurring and attacks from occurring. HAE is a rare disorder, occurring in less than five out of every 1,000 people. This disease affects both men and women equally.

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