Comorbidities in Angioedema Due to C1-Inhibitor Deficiency: An Italian Survey

Abstract

BackgroundHereditary angioedema due to C1-inhibitor deficiency (HAE) is characterized by unpredictable recurrent episodes of swelling affecting the skin and the mucosa tissues, including gastrointestinal tract and/or oro-pharyngeal-laryngeal mucosae. Long-term prophylaxis (LTP) are used to prevent attacks. ObjectiveSince C1-inhibitor (C1-INH) plays a pivotal role in several biological pathways, we investigated the possible association of comorbidities with C1-INH deficiency and the use of LTP with androgens (AA) or tranexamic acid (TXA). MethodsThis retrospective cohort study involved adult patients with HAE referring to Milan and Padua angioedema centers in the period 1979-2021. A qualitative comparison was performed to analyze comorbidities vs. general population. The incidence of comorbidities was evaluated during LTP with AA or TXA vs. patients without LTP. ResultsA total of 446 patients were studied. A greater prevalence among patients was found for: heart diseases (9.6% vs. 4.8%), acute myocardial infarction (5.6% vs. 1.4%), HCV infection (10.5% vs. 2.5%), and appendectomy (15.9% vs. 4.3%). In patients taking AA a greater incidence was found for: hypertension (22.8% vs. 10.8%; OR 2.02), hypercholesterolemia (19.5% vs. 5.3%; OR 3.97), diabetes mellitus (5% vs. 1.4%; OR 3.21), hepatic angioma (4.4% vs. 0.7%; OR 8.35), and focal nodular hyperplasia (2.5% vs. 0.4%; OR 6.9). No association with TXA and comorbidities was found. ConclusionIn this large patient population with a rare disease followed for up to a 43-year period, we found a greater prevalence of comorbidities hitherto unreported in the literature and an association between comorbidities and LTP with AA.