Clinical Features and Disease Course of Primary Angioedema Patients in a Tertiary Care Hospital
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Clinical Features and Disease Course of Primary Angioedema Patients in a Tertiary Care Hospital. / Pall, Amalie Hartvig; Lomholt, Anne Fog; Buchwald, Christian von; Bygum, Anette; Rasmussen, Eva Rye.
In: Journal of Asthma and Allergy, Vol. 13, 07.2020, p. 225-236.Research output: Contribution to journal › Journal article › Research › peer-review
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TY - JOUR
T1 - Clinical Features and Disease Course of Primary Angioedema Patients in a Tertiary Care Hospital
AU - Pall, Amalie Hartvig
AU - Lomholt, Anne Fog
AU - Buchwald, Christian von
AU - Bygum, Anette
AU - Rasmussen, Eva Rye
PY - 2020/7
Y1 - 2020/7
N2 - Purpose: To give a better understanding of primary AE, the clinical characteristics and the possible therapeutic approaches. Background: Angioedema (AE) is a non-pitting, non-itching swelling of skin or mucosa. The symptom can become life-threatening if located in the airways. Primary (monosympto-matic) AE is a manifestation of several different diseases and the diagnosis is not always straight-forward. The aetiological and pathophysiological factors of primary AE are not completely clarified. There is a need for further investigation. Patients and Methods: This was a retrospective cohort study of patients referred to an outpatient dermatology clinic in a tertiary care hospital for clinical assessment due to primary AE in the period from 1996 to 2014. Results: A total of 315 patients were identified with primary AE. The most frequent subtype was idiopathic AE (42.5%) and the second most common was angiotensin-converting enzymeinhibitor (ACEi)-induced AE (31.1%). Three patients were diagnosed with hereditary AE and one patient was diagnosed with acquired C1-inhibitor deficiency. At least 107 (34.0%) patients had established histaminergic AE. More than 1/3 of the patients were treated in an emergency room or hospitalized due to AE. A 98.1% of patients had experienced AE in the head and neck area. Seven patients were in the need of acute airway intervention. Six of these had ACEi-induced AE. Female sex and smoking were found to be risk factors for developing AE. Conclusion: The most frequent diagnoses were histaminergic-, non-histaminergic idiopathic AE and ACEi-induced AE, whereas complement C1-inhibitor deficiency was rare. Histaminergic AE made up a substantial group of patients with primary AE. Even though there are different pathophysiological causes of AE, many cases have overlapping clinical manifestations, which make diagnosis and treatment difficult.
AB - Purpose: To give a better understanding of primary AE, the clinical characteristics and the possible therapeutic approaches. Background: Angioedema (AE) is a non-pitting, non-itching swelling of skin or mucosa. The symptom can become life-threatening if located in the airways. Primary (monosympto-matic) AE is a manifestation of several different diseases and the diagnosis is not always straight-forward. The aetiological and pathophysiological factors of primary AE are not completely clarified. There is a need for further investigation. Patients and Methods: This was a retrospective cohort study of patients referred to an outpatient dermatology clinic in a tertiary care hospital for clinical assessment due to primary AE in the period from 1996 to 2014. Results: A total of 315 patients were identified with primary AE. The most frequent subtype was idiopathic AE (42.5%) and the second most common was angiotensin-converting enzymeinhibitor (ACEi)-induced AE (31.1%). Three patients were diagnosed with hereditary AE and one patient was diagnosed with acquired C1-inhibitor deficiency. At least 107 (34.0%) patients had established histaminergic AE. More than 1/3 of the patients were treated in an emergency room or hospitalized due to AE. A 98.1% of patients had experienced AE in the head and neck area. Seven patients were in the need of acute airway intervention. Six of these had ACEi-induced AE. Female sex and smoking were found to be risk factors for developing AE. Conclusion: The most frequent diagnoses were histaminergic-, non-histaminergic idiopathic AE and ACEi-induced AE, whereas complement C1-inhibitor deficiency was rare. Histaminergic AE made up a substantial group of patients with primary AE. Even though there are different pathophysiological causes of AE, many cases have overlapping clinical manifestations, which make diagnosis and treatment difficult.
KW - Angiotensin-converting enzyme inhibitors
KW - Bradykinin
KW - Hereditary angioedema
KW - Primary angioedema
KW - Urticaria
UR - http://www.scopus.com/inward/record.url?scp=85088485270&partnerID=8YFLogxK
U2 - 10.2147/JAA.S245161
DO - 10.2147/JAA.S245161
M3 - Journal article
C2 - 32764994
AN - SCOPUS:85088485270
VL - 13
SP - 225
EP - 236
JO - Journal of Asthma and Allergy
JF - Journal of Asthma and Allergy
SN - 1178-6965
ER -
ID: 249528033