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. 2020 Jul 17:13:225-236.
doi: 10.2147/JAA.S245161. eCollection 2020.

Clinical Features and Disease Course of Primary Angioedema Patients in a Tertiary Care Hospital

Amalie Hartvig Pall  1 , Anne Fog Lomholt  1 , Christian von Buchwald  1 , Anette Bygum  2   3   4 , Eva Rye Rasmussen  1   2
Affiliations

Clinical Features and Disease Course of Primary Angioedema Patients in a Tertiary Care Hospital

Amalie Hartvig Pall et al. J Asthma Allergy. .

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 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.

Keywords: angiotensin-converting enzyme inhibitors; bradykinin; hereditary angioedema; primary angioedema; urticaria.

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Conflict of interest statement

A. Pall has received one travel grant from CSL Behring. A. Bygum has been involved in clinical research and educational events involving CSL-Behring, Dyax, BioCryst and Shire/Takeda, reports payment for educational activities from Novartis, and grants and personal fees from CSL Behring and Shire (now part of Takeda), and personal fees from Novartis, outside the submitted work. E. Rasmussen has received research funding, travel grants and speakers fees from Shire, CSL Behring and Viropharma, reports personal fees from Shire-Takeda, outside the submitted work, and served on an advisory board on hereditary angioedema treatment for Shire-Takeda. The authors report no other possible conflicts of interest in this work.

Figures

Figure 1
Figure 1
Patients with primary AE included in study. Seven hundred thirty-four patients referred to a dermatology department. Three hundred fifteen patients included in study.
Figure 2
Figure 2
Underlying factors in 315 primary AE patients.
See this image and copyright information in PMC

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