Abstract
BACKGROUND: Hereditary angioedema due to C1 inhibitor deficiency (HAE-C1INH) is characterized by sudden subcutaneous and/or submucosal angioedema attacks. C1 inhibitor is a serine protease inhibitor that regulates several enzyme cascade systems. The absence of this control raises concerns about the potential development of comorbidities. OBJECTIVE: We aimed to investigate the comorbidities present in the Hungarian HAE-C1INH patient population and to examine the surgical procedures performed on these patients. Additionally, we sought to compare our results to those of the Hungarian general population. METHODS: Demographical, clinical, laboratory, and radiographic data of all 178 adult HAE-C1INH patients followed up at the Hungarian Angioedema Center of Reference and Excellence were used to examine comorbidities and surgical procedures. Information about the general Hungarian population was extracted from national and European statistical databases and individual articles. RESULTS: At least 1 comorbidity was present in 159 of our patients. From the observed 51 diseases, headache (58%), hypercholesterolemia (52%), hypertension (30%), and liver diseases (26%) were the most prevalent. Hypercholesterolemia and depression/anxiety were at least 3 times more common in the HAE-C1INH population as compared to the Hungarian general population. Tonsillectomy was performed 32, appendectomy 11, and inguinal hernioplasty 2 times more often before the diagnosis of HAE-C1INH was established. Every surgical procedure was more prevalent in the HAE-C1INH population. CONCLUSION: Regular, targeted screening is indispensable for the prevention and timely diagnosis of certain diseases found in higher prevalence in the HAE-C1INH population. The early identification and adequate treatment of angioedema attacks help prevent avoidable surgical interventions.