Upper airway edema in 43 patients with hereditary angioedema.
BACKGROUND
Upper airway edema (UAE) occurs infrequently in hereditary angioedema (HAE), but still results in significant morbidity and mortality.


OBJECTIVE
To assess patients with HAE and UAE to determine whether unique features exist that can predict the risk of UAE.


METHODS
Clinical, laboratory, and genetic data were compared between 43 patients with HAE and 743 UAE attacks and those without UAE and normal controls after ethics committee approval.


RESULTS
Most patients had their first episode of UAE in the second (25.6%), third (27.9%), and fourth (23.3%) decades of life, and the mean age at onset was 27.3 years. Evolution of UAE from initial to maximum symptoms was 4.6 hours on average, and most cases (69.8%) progressed within 4 hours. Dyspnea was the most frequent manifestation in per-episode (92.2%) and per-patient (97.7%) analyses. Men developed more asphyxiation attacks (19 vs 2) and underwent more tracheotomies (12 vs 2) than did women. UAE was associated with facial edema in half the studied patients. Patients with a positive family history of UAE had a high risk of UAE attacks.


CONCLUSIONS
Symptoms limited to the upper airway should be taken seriously. Dyspnea may be the only manifestation of UAE. UAE attacks most commonly start spontaneously and usually progress rapidly, as quickly as 30 minutes, from awareness of symptoms to maximum airway involvement. Patients with a positive UAE family history are predisposed to UAE attacks, and men appear to be more apt to develop asphyxiation than women.
Department of Allergy, Peking Union Medical College Hospital, Chinese Academy of Medical Sciences and Peking Union Medical College, Beijing, China.
Ying-Yang
YY
Xu
Department of Allergy, Peking Union Medical College Hospital, Chinese Academy of Medical Sciences and Peking Union Medical College, Beijing, China. Electronic address: yuxiang_zhi@126.com.
Yu-Xiang
YX
Zhi
Department of Allergy, Tianjin Medical University General Hospital, Tianjin, China.
Rui-Ling
RL
Liu
Department of Medicine and Pediatrics, Penn State University, Hershey, Pennsylvania.
Timothy
T
Craig
Department of Allergy, Peking Union Medical College Hospital, Chinese Academy of Medical Sciences and Peking Union Medical College, Beijing, China.
Hong-Yu
HY
Zhang
D000293:Adolescent; D000328:Adult; D017668:Age of Onset; D000402:Airway Obstruction / Q000150:complications; D054179:Angioedemas, Hereditary / Q000209:etiology* / Q000235:genetics / Q000401:mortality; D001237:Asphyxia / Q000150:complications*; D002648:Child; D003174:Complement C1 Inactivator Proteins / Q000235:genetics / Q000378:metabolism; D004417:Dyspnea / Q000150:complications*; D005260:Female; D006801:Humans; D007819:Laryngeal Edema / Q000150:complications*; D008297:Male; D008875:Middle Aged; D011654:Pulmonary Edema / Q000150:complications*; D012189:Retrospective Studies; D055815:Young Adult
D003174:Complement C1 Inactivator Proteins