HEREDITARY ANGIOEDEMA

Course overview

HEREDITARY ANGIOEDEMA (HAE) IS AN AUTOSOMAL DOMINANT DISEASE CAUSED BY EITHER A LACK OF C1-INHIBITOR PROTEIN OR BY DYSFUNCTIONAL C1-INHIBITOR PROTEIN. HAE MANIFESTS WITH SYMPTOMS RELATED TO ANGIOEDEMA OF THE UPPER AIRWAY, SKIN AND/ OR GASTROINTESTINAL TRACT. THE MOST FEARED COMPLICATION IS UPPER AIRWAY SWELLING THAT CAN PROCEED TO ASPHYXIATION. FORTUNATELY, UPPER AIRWAY INVOLVEMENT IS RARE. THIS ACTIVITY REVIEWS THE EVALUATION AND MANAGEMENT OF HAE AND HIGHLIGHTS THE ROLE OF THE INTERPROFESSIONAL TEAM IN THE CARE OF PATIENTS AFFECTED BY THIS CONDITION.