HAE Attack Presentation

Swelling occurs within the skin and/or mucous membranes and can be extremely painful, disfiguring, and/or disabling. Depending on the area of the body affected, patients may not feel they can leave the house to go to work or school, which can have a significant impact on their lives.

Hereditary angioedema (HAE) symptoms most often affect 3 areas of the body—skin, gastrointestinal (GI) tract, and upper airway.12

Skin
Skin swelling can cause pain, dysfunction, and disfigurement. Common affected areas on the face include the lips and eyes.

  • Face
  • Hands
  • Arms
  • Legs
  • Genitals
  • Buttocks
GI Tract
Swelling along the GI tract, which starts at the esophagus and ends at the intestines, can cause symptoms such as nausea, vomiting, diarrhea, and abdominal pain.

  • Stomach
  • Intestines
  • Bladder
  • Urethra
Upper Airway
Swelling in the upper airway, which includes the tongue and throat, can affect the ability to breathe and become life-threatening. However, most attacks resolve before the airway closes completely, but the feeling can cause anxiety. Approximately 50% of people with HAE will experience at least one airway attack.13

  • Throat
  • Tongue

 

Less common symptoms include neurological, pulmonary, renal, urinary, and musculoskeletal manifestations. Severe headache accompanied by other neurological symptoms, such as vision disturbances, impaired balance, and disorientation, have been reported.2

Symptoms typically worsen and reach their peak within the first 24 hours before slowly resolving within 2-5 days. Most patients experience an attack at a single site; however, some can have simultaneous sites or a cutaneous episode closely followed by an abdominal one (and vice versa).2

Prodromal Symptoms Prior to an Attack

  • Nonerythematous rash
  • Tingling sensation
  • Anxiety
  • Mood changes
  • Exhaustion

Prodromes can occur hours to days before an attack; however, there is significant variability in the expression, manifestation, prevalence, timing, and predictive reliability of prodromes.29

Understanding and recognizing the early signs of an attack may help patients treat symptoms quickly.


References

[2] Lumry WR. Overview of epidemiology, pathophysiology, and disease progression in hereditary angioedema. Am J Manag Care. 2013;19(7 Suppl):s103-110.
[12] Genetic and Rare Diseases Information Center. Hereditary angioedema. National Institutes of Health. https://rarediseases.info.nih.gov/diseases/5979/hereditary-angioedema.
[13] US Hereditary Angioedema Association. Patient Guide: Understanding HAE! https://www.haea.org/assets/img/PatientGuide061819.pdf.
[29] Kemp, John G.; Craig, Timothy J. Allergy and Asthma Proceedings, Volume 30, Number 5, September/October 2009, pp. 493-499(7)