The characteristics of upper airway edema in hereditary and acquired angioedema with C1‐inhibitor deficiency
-
- Zsuzsanna Balla
- Department of Internal Medicine and Haematology Hungarian Angioedema Center of Reference and Excellence Semmelweis University Budapest Hungary
-
- Noémi Andrási
- Department of Internal Medicine and Haematology Hungarian Angioedema Center of Reference and Excellence Semmelweis University Budapest Hungary
-
- Zsófia Pólai
- Department of Internal Medicine and Haematology Hungarian Angioedema Center of Reference and Excellence Semmelweis University Budapest Hungary
-
- Beáta Visy
- Department of Internal Medicine and Haematology Hungarian Angioedema Center of Reference and Excellence Semmelweis University Budapest Hungary
-
- Ibolya Czaller
- Department of Pulmonology Semmelweis University Budapest Hungary
-
- György Temesszentandrási
- Hospital of the Hospitaller Brothers of Saint John of God Budapest Hungary
-
- Dorottya Csuka
- Research Laboratory Department of Internal Medicine and Haematology Semmelweis University Budapest Hungary
-
- Lilian Varga
- Department of Internal Medicine and Haematology Hungarian Angioedema Center of Reference and Excellence Semmelweis University Budapest Hungary
-
- Henriette Farkas
- Department of Internal Medicine and Haematology Hungarian Angioedema Center of Reference and Excellence Semmelweis University Budapest Hungary
Abstract
<jats:title>Abstract</jats:title><jats:sec><jats:title>Background</jats:title><jats:p>Angioedemas localized in the upper airway are potentially life threatening, and without proper treatment, they may lead to death by suffocation. Upper airway edemas (UAE) in bradykinin‐mediated angioedemas can even be the first symptoms of the disease.</jats:p></jats:sec><jats:sec><jats:title>Methods</jats:title><jats:p>Our survey was performed with a retrospective long‐term follow‐up method from the medical history of 197 hereditary (C1‐INH‐HAE) and 20 acquired C1‐inhibitor deficiency (C1‐INH‐AAE), 3 factor XII and 3 plasminogen gene mutation (FXII‐HAE, PLG‐HAE) patients treated at our center between 1990 and 2020. The UAE group included edemas localized to the mesopharynx, hypopharynx, and larynx, as narrowing of these anatomical regions can lead to suffocation.</jats:p></jats:sec><jats:sec><jats:title>Results</jats:title><jats:p>98/197 C1‐INH‐HAE (47 families) and 13/20 C1‐INH‐AAE, 1/3 PLG‐HAE, 1/3 FXII‐HAE patients had experienced UAE at least once according to their medical history. In case of C1‐INH‐HAE patients, in 6/47 families who had undiagnosed ancestors had 13 members who died of suffocation. After the diagnosis, 1‐1 member of two families died of UAE. 44/64 C1‐INH‐HAE patients did not smoke, 20/64 did. The occurrence of UAE was significantly higher in smoker patients. We analyzed 7607 HAE attacks of 56/98 patients. Out of all attacks, the incidence of UAE in the C1‐INH‐HAE group was 4%, and 9.5% in the C1‐INH‐AAE group, respectively.</jats:p></jats:sec><jats:sec><jats:title>Conclusion</jats:title><jats:p>Early diagnosis is key in bradykinin‐mediated angioedemas cases, since the patient must be provided with adequate treatment; and also it is essential to inform patients about the importance of avoiding the trigger factors and the early symptoms of UAE, as these measures could significantly decrease the incidence of lethal UAEs.</jats:p></jats:sec>
Journal
-
- Clinical and Translational Allergy
-
Clinical and Translational Allergy 11 (10), 2021-12
Wiley
- Tweet
Details 詳細情報について
-
- CRID
- 1360861709528878848
-
- ISSN
- 20457022
-
- Data Source
-
- Crossref