Epidemiology and Management of Acquired Angioedema Due to C1 Inhibitor Deficiency
Mohammed Olaythah Alraddadi
Consultant dermatologist, University of Tabuk, Saudi Arabia.
Yousef Hussain J. Alharthi
University of Tabuk, Saudi Arabia.
Rayyan Fahad H. Altemani
University of Tabuk, Saudi Arabia.
Wejdan Mohammed S. Alshehri
University of Tabuk, Saudi Arabia.
Amal Nafea J. Alharbi
University of Tabuk, Saudi Arabia.
Raghad Saud A. Albalawi
University of Tabuk, Saudi Arabia.
Khawla Abdulrahman S. Alzahrani
University of Tabuk, Saudi Arabia.
Shuruq Mousa D. Albalawi
University of Tabuk, Saudi Arabia.
Sultan Suliman Q. Al-Ruwaili
University of Tabuk, Saudi Arabia.
Sultan Faraj Alruwaili
AL-qurayyat Gerneral Hospital, Aljouf Region, Saudi Arabia.
Mousa Amer M. Alshehri
Aseer General Hospital-Abha-Aseer Region, Saudi Arabia.
Asma Saleh S. Alruwaili
North Medical Tower Hospital-Northern Border-arar-Saudi Arabia.
Sarah Mohammad M. Ali
Alrayan College, Saudi Arabia.
Ahmad Arafat A. Abualaz
University of Jordan, Amman, Jordan.
Marah Saud Alenezi
Alfaisal University, Saudi Arabia.
*Author to whom correspondence should be addressed.
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.
Keywords: Angioedema, acquired angioedema due to c1 inhibitor deficiency, non - pitting edema, edema