Hereditary Angioedema (HAE) is an inherited disorder, which affects the immune system and can cause episodes of swelling in various parts of the body. It is the result of an abnormal gene in the family, and is inherited in an autosomal dominant manner. It affects both men and women, and tends to occur in a pattern within a family.
HAE is a rare disease, and its exact prevalence is difficult to estimate. It is estimated that 1 in 50,000 people have HAE. The cause of the disorder is unknown, but it is believed to be related to a deficiency in a protein called C1-esterase inhibitor, which normally helps regulate the body’s response to inflammation.
Symptoms of HAE include swelling in different parts of the body such as the hands, feet, arms, face, and throat. Swelling of the airway can be dangerous and may cause difficulty breathing and anaphylactic shock. HAE is also known to cause abdominal pain, nausea, vomiting, and low blood pressure.
The treatment of HAE often includes anti-inflammatory medications, such as nonsteroidal anti-inflammatory drugs or corticosteroids. Some therapies, including C1 esterase inhibitor replacement therapy and antifibrinolytics, have been used to prevent or reduce swelling.
However, the most effective way to manage HAE is to recognize its possible triggers. Common triggers include certain foods, alcohol, stress, and changes in hormone levels associated with the menstrual cycle.
As with any disorder, HAE is best managed with early diagnosis and treatment. If you or a family member think you may have HAE, it is important to contact your physician to discuss diagnosis and treatment options. Early diagnosis and treatment can help to prevent potentially life-threatening episodes of swelling and other complications.