PhD Scientific Days 2022

Clinical Medicine VII. (Poster discussion will take place in the Aula during the Coffee Break)

Occurrence of Allergic Diseases in Patients With Hereditary Angioedema Due To C1-Inhibitor-Deficiency

Text of the abstract

Introduction: Hereditary angioedema due to C1-inhibitor-deficiency (C1-INH-HAE) is characterised by recurrent, unpredictable submucosal and/or subcutaneous edematous episodes. In C1-INH-HAE bradykinin release in the plasma kinin-kallikrein system (PKKS) leads to angioedema formation. The role of mastocytes in this process has been recently described. Heparin, released from these cells, may activate the PKKS, leading to bradykinin generation. This observation suggests a connection between HAE and allergic diseases.
Aims: To assess the occurrence of allergic diseases in the Hungarian C1-INH-HAE population.
Method: A questionnaire - compiled by us - containing 70 questions about allergic symptoms and diseases was answered by our C1-INH-HAE patients online or on paper.
Results: 107 patients (pts) (65 female, 42 male) responded. Mean age was 46 years (range: 10-90). 51.4% of the pts had or thought to have allergic diseases. Mean duration of allergic diseases was 23.7 years (range: 1.5-60). 16.2% of pts reported drug allergy and 11.4% experienced an HAE attack after taking medicine. Drug allergy was most frequently caused by antibiotics and usually occurred as rash or itching. HAE attacks were mostly abdominal and caused by antibiotics, ACE-inhibitors or oral contraceptives. 25.3% of pts reported food allergy, caused mostly by nuts or dairy products, resulting in bloating, abdominal pain or diarrhoea. 23.8% of pts experienced mostly abdominal HAE attacks after consuming food, specifically onions, nuts or dairy products. 29.3% of pts reported pollen allergy and 89.7% amongst them felt that their allergic symptoms were more frequent during “pollen season”. 12% of this group also had more frequent HAE attacks. After the diagnosis of HAE, one patients’ allergic symptoms disappeared, 5 experienced improvement, 43 remained the same, but none of them experienced worsening. In 6.1% of the allergic pts, an HAE attack worsened the allergic symptoms. In contrast, 17% of the allergic pts experienced HAE attack provoked by contact with the allergen.
Conclusion: In contrast to the 51.4% prevalence of allergy among our patients, the Eurostat’s newest data stated that in Hungary the prevalence of allergic diseases is around 19%. This suggests a connection between the two diseases, but further molecular studies are needed.
Funding: EFOP-3.6.3_VEKOP-16-2017-00009, NKFI 124557