Abstract
AAE-C1-INH (acquired angioedema owing to C1-inhibitor (C1-INH) deficiency) is a dangerous illness that can lead to asphyxiation due to laryngeal edoema. Only around 1% to 2% of angioedema cases are classified as HAE or AAE, with HAE being 10 times more prevalent than AAE. The sole clinical distinction between HAE and AAE is the age at which symptoms appea, AAE-C1-INH is usually diagnosed after 40 years of age. There is no licensed therapy for AAE-C1-INH at this time. AAE-C1-INH attacks are treated with HAE-C1-INH medicines such plasma-derived C1-INH concentrate (pdC1-INH) and the bradykinin B2 receptor antagonist, icatibant. These on-demand medications are thought to be most helpful when provided early in the attack. However, there is a scarcity of published data on the efficacy and safety of AAE-C1-INH therapies.
Highlights
Angioedema is characterized by one or more patches of well-demarcated, non-pitting edoema of deep subcutaneous tissues
AAE-C1 inhibitor (C1-INH) (acquired angioedema owing to C1inhibitor (C1-INH) deficiency) is a dangerous illness that can lead to asphyxiation due to laryngeal edoema
Angioedema is characterised by disfiguring, non-pitting, non-pruritic edoema of the skin, severe abdominal pain due to edoema of the gastrointestinal mucosa leading to temporary bowel occlusion, life-threatening edoema of the upper respiratory tract, and edoema of the oral mucosa and tongue [6]
Summary
Angioedema is characterized by one or more patches of well-demarcated, non-pitting edoema of deep subcutaneous tissues. Due to edoema of the gastrointestinal mucosa, patients have recurring swellings or attacks of the skin (facial, limbs, and genitals) as well as severe stomach episodes, often with diarrhoea and vomiting. They may have edoema of the upper respiratory tract, oral mucosa, and tongue, which can be life-threatening. Angioedema is characterised by disfiguring, non-pitting, non-pruritic edoema of the skin (face, limbs, genitals), severe abdominal pain due to edoema of the gastrointestinal mucosa leading to temporary bowel occlusion, life-threatening edoema of the upper respiratory tract, and edoema of the oral mucosa and tongue [6]. Treatment of C1 inhibitordependent angioedema relies on restoring regulation of BK formation by inhibiting CP proteases or inhibiting BK-mediated effects at the BKR2 on endothelial cells by restoring the balance between CP inhibitors and BK breakdown [7]
Sign-in/Register to view highlights & summary Sign-in/Register to access full text options 
Talk to us
Join us for a 30 min session where you can share your feedback and ask us any queries you have
Schedule a call
