Acute attack of hereditary angioedema

What is Acute Attack of Hereditary Angioedema?

Acute attack of hereditary angioedema (HAE) is a rare, potentially life-threatening disorder that is caused by a genetic defect. It is characterized by recurrent episodes of swelling of the skin, mucous membranes, and internal organs, including the larynx and gastrointestinal tract.

The most common symptoms of an acute attack of HAE are swelling of the face, eyelids, lips, tongue, hands, feet, and genitalia. Swelling of the throat, abdominal pain, nausea, vomiting, and diarrhea may also occur. The episodes usually last for a few days, although this can vary from person to person.

The exact cause of an acute attack of HAE is unknown, but it is thought to be related to a deficiency or abnormality of a specific protein called C1-esterase inhibitor (C1-INH). This protein helps regulate the activity of certain substances in the body which are involved in inflammatory and immune responses.

Risk Factors for Acute Attack of Hereditary Angioedema

Common triggers for an acute attack of hereditary angioedema can include emotional stress, extreme temperatures, infections, trauma, and certain medications. Some people may experience an attack without a known trigger.

In addition to the risk factors listed above, HAE patients are also at risk for certain types of cancer, autoimmune disorders, and heart disease.

Diagnosing Acute Attack of Hereditary Angioedema

An acute attack of hereditary angioedema can usually be diagnosed based on the symptoms and the medical history of the patient. A physical exam may reveal swelling or edema, and laboratory tests can be used to measure C1-INH levels. Genetic testing can confirm the diagnosis.

Treatment of Acute Attack of Hereditary Angioedema

Acute attacks of HAE can usually be treated with medications that suppress inflammation. These drugs include antihistamines, corticosteroids, and immune-modulating agents. Other medications may be used to treat the acute symptoms such as itching, pain, and swelling.

In severe cases of an acute attack, intravenous drugs such as ecallantide and icatibant can be used. These drugs are designed to decrease the release of inflammatory chemicals from cells in the body.

In some cases, surgery may be necessary to remove excess fluid from the affected area or to repair damaged tissue. In more severe cases, a medical team may provide intensive care in the hospital to ensure the patient’s condition does not worsen.

Prevention of Acute Attack of Hereditary Angioedema

The best way to prevent an acute attack of HAE is to avoid known triggers and be aware of any other potential risk factors. It’s important for HAE patients to get regular checkups so the doctor can monitor any changes in their condition.

HAE patients should also be educated about the signs and symptoms of an acute attack, so they can seek medical help as soon as possible. A prompt response is critical in order to reduce the severity and duration of the swelling.

Conclusion

Acute attack of hereditary angioedema is a rare, potentially life-threatening disorder caused by a genetic defect. Its symptoms include swelling of the face, eyelids, lips, tongue, hands, feet, and genitalia. Common triggers include emotional stress, extreme temperatures, infections, trauma, and certain medications. Treatment consists of medications and, in some cases, surgery.