The following is a summary of “US Hereditary Angioedema Association Scientific Registry: hereditary angioedema demographics, disease severity, and comorbidities,” published in the December 2023  issue of Allergy & Immunology by Christiansen, et al.

Idiopathic nonhistaminergic angioedema (INHA) and hereditary angioedema (HAE) are very uncommon diseases whose natural histories and associated conditions are not fully known.

For a study, researchers sought to create a national register focused on patients to fill in these gaps in their knowledge and make it easier to see how therapy approaches work in the real world. People who were part of the US HAE Association put their information into a public database from 2009 to April 7, 2021. Cohorts were put into groups based on the doctor’s stated diagnosis.

A set of surveys were used to get information from patients. The results of the HAE due to C1 inhibitor deficit (HAE-C1INH) group were compared to those of the HAE with normal C1 inhibitor (HAE-nl-C1INH) plus INHA group in terms of demographics, natural history, and family history. The number of people with multiple conditions in the HAE-C1INH group was compared to the general US community.

The study looked at 485 HAE-C1INH participants, 26 HAE-nl-C1INH participants, and 70 INHA participants. The time it took to diagnose HAE-C1INH was shorter (5 years vs. 11 years), but both delays got shorter over time. Attacks happened in different places and at different times for each group. There were a lot of hospitalizations, emergency room visits, needless stomach surgeries, and intubations, as well as deaths. In fact, 36.9% of HAE-C1INH participants and 15.4% of HAE-nl-C1INH participants said that family members had died from an HAE attack. There were a lot more cases of sadness, sleep problems, kidney disease, anemia, and hepatitis in women who had HAE-C1INH. Heart disease and other cardiovascular problems were much less common in the HAE-C1INH group. The US HAEA Scientific Registry gives them a way to learn more about HAE and INHA.

Source: sciencedirect.com/science/article/pii/S1081120623005896