Clinical Characteristics and Management of Angioedema Attacks in Polish Adult Patients with Hereditary Angioedema Due to C1-Inhibitor Deficiency.

Katarzyna Piotrowicz-Wójcik,Krzysztof Kuziemski,Jacek Gocki,Robert Pawłowicz,Grzegorz Porebski,Aldona Juchacz,Joanna Jamróz-Brzeska,Małgorzata Bulanda

doi:10.3390/jcm10235609

Abstract

Hereditary angioedema (HAE) due to C1-inhibitor (C1-INH) deficiency is a rare disease characterized by recurrent swellings. This study aims to determine (i) the clinical characteristics of the HAE patient population from Poland, and (ii) real-life patients’ treatment practices. A cross-sectional study involved 138 adult HAE patients (88 females, 50 males) treated in six regional HAE centers in Poland. Consecutive patients during routine follow-up visits underwent a structured medical interview on the clinical characteristics of the course and treatment of HAE attacks within the last six months. A total of 118 of 138 patients was symptomatic. They reported in total 2835 HAE attacks predominantly peripheral and abdominal, treated with plasma-derived C1-INH (61.4%), icatibant (36.7%) and recombinant C1-INH (1.9%). An amount of 116 patients carried the rescue medication with them while traveling, and 74 patients self-administrated on demand treatment. There were twice as many symptomatic women (n = 78) as there were men (n = 40). Women treated their HAE attacks significantly more often than men. Older patients (≥65 years) reported a longer delay in diagnosis, and practiced the self-administration of rescue medication less frequently in comparison to other patients. Clinical features of the surveyed population are similar to other European, but not Asian, HAE patient groups. Self-administration still remains an unmet medical need. Some distinct HAE patients may require special attention due to the severe course of the disease (females) or a delay in diagnosis (the elderly).

Highlights

This article is an open access articleHereditary angioedema due to C1-inhibitor deficiency (HAE) is a rare, autosomal dominant disease
Taking into account the available data [28], our study group involved about half of the Polish HAE patient population
Our data showed a higher prevalence of HAE type 1 (90% of the patients) over HAE type 2 (10%), confirming the findings from several other countries such as Switzerland (98.1%) [4], the UK (85%) [5], Italy (87%) [6], Greece (80.5%) [7], Sweden (93.2%) [8], Serbia (89%) [9], Romania (91.7%) [11], Belarus (84.4%) [12], Austria (80.2%) [13], Korea (90.8%) [16] and the USA (78.4%) [19]

Summary

Introduction

This article is an open access article. Hereditary angioedema due to C1-inhibitor deficiency (HAE) is a rare, autosomal dominant disease. It is characterized by recurrent, unpredictable episodes of angioedema [1]. Swelling episodes affect the skin, mucous membranes, e.g., the mouth, gastrointestinal tract, or larynx [2]. Increased vascular permeability leads to excessive fluid displacement and severe angioedema attacks that could be spontaneous or triggered by some factors distributed under the terms and conditions of the Creative Commons.

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