Signs & Symptoms of Hereditary AngioedemaNext Topic
Attacks of HAE occur at variable times over a patient’s lifetime. Attacks usually begin in early childhood, may worsen
at puberty, and occur episodically and with varying frequency and severity throughout the patient’s life.
HAE symptoms include recurring attacks of edema (swelling) in various parts of the body, including the upper airways
(throat or larynx), hands, feet, face, and abdomen (stomach). HAE attacks affecting the hands, face, and feet can be severely
disfiguring, whereas abdominal attacks, caused by swelling of the intestinal wall, are marked by severe abdominal pain.
Attacks that affect the airway/throat can be life threatening, as swelling can constrict the larynx or enlarge the tongue
which can quickly lead to suffocation. See the table below for a checklist of symptoms.
| Symptom checklist for HAE attacks |
| Tightness or tingling of skin prior to onset of attack |
Hoarseness or change in sound of voice |
| Swelling of face |
Difficulty swallowing |
| Eyelid swelling |
Swelling of pharynx |
| Swelling of extremities |
Swelling of larynx |
| Genital swelling |
Acute abdominal pain |
| Lip swelling |
Constipation or diarrhea |
| Tongue swelling |
Nausea and/or vomiting |
Some triggers of HAE attacks have been identified. These include the following:
- Trauma, even if minor
- Stress
- Dental work
- Invasive medical procedures
- Vigorous exercise
- Alcohol consumption
- Estrogen
- Oral contraceptives
In most cases, the edema develops gradually over a period of 12 to 36 hours and then goes away on its own within 2 to 5
days. It has been reported that the time from the onset of symptoms to a full-blown attack ranges from 20 minutes to as long
as 14 hours and that patients have a median of 12 attacks per year.
The development of edema does not affect one site consistently, which makes it impossible for patients to predict which
site will be affected by the next attack. The frequency, duration, and severity of the edema may also vary from one attack
to the next. Most patients have an average of one episode of swelling per month. However, some patients may experience weekly
attacks while others have only one or two attacks per year.
HAE places extraordinary strain on patients and family members. Depending on the severity of attacks, just one episode per
month may prevent the patient from having a normal social and professional life for several days at a time. There is also
constant worry about a life-threatening episode that involves the airway or larynx. As HAE is a rare disease, many healthcare professionals are unfamiliar with the condition. Correct diagnosis and subsequent referral to a physician familiar with treating and managing HAE often takes several years.
Next Topic