<p>Clinical Features and Disease Course of Primary Angioedema Patients in a Tertiary Care Hospital</p>

Abstract

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 (monosymptomatic) AE is a manifestation of several different diseases and the diagnosis is not always straightforward. 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.

Clinical profile of idiopathic angioedema based on severity and treatment response is independent of the presence of concomitant wheals

“…In preceding studies, the effect of antihistamines in AE patients was analysed with regard to the current classification of AAE, which defines patients as IH‐AAE or InH‐AAE based on the response to antihistamine therapy 2,3 . The effect of antihistamines on AE was previously studied mainly in AE patients without wheals 10–13 . These studies found similar complete response rates at follow‐up after antihistamine monotherapy (in variable dosage) ranging from 56% to 72% 10,11 and improvement of attack frequency in 86%–91% 12,13 .…”

The Role of Cetirizine in the Changing Landscape of IV Antihistamines: A Narrative Review

“…Histamine-mediated angioedema is caused by the same underlying pathologic mechanism as wheals but occurs deeper in the skin. Angioedema is a common reason for ED visits [ 21 ] and can be life-threatening if the airway is involved [ 22 , 23 ]. H 1 antihistamines, along with corticosteroids, are first-line treatment for histamine-mediated angioedema without anaphylaxis [ 23 ].…”

Angioedema with Three Possible Etiologies

“…It is self-limited, often asymmetrical, and can be life threatening if associated with airway compromise and/or anaphylaxis. While hereditary AE has been examined thoroughly in the literature, [1][2][3] there is much to be discovered of the acquired and idiopathic forms. This case reports a new onset unspecified postoperative angioedema, possible etiologies, and best treatment practices.…”