ACAAI Survey Reveals Challenges in Diagnosing, Treating Hereditary Angioedema

Among ACAAI members surveyed about HAE, patients’ difficulties in accessing specialist care, especially in rural areas, and the cost of treatment were significant concerns.

Misdiagnosis of hereditary angioedema (HAE) is the top diagnostic challenge in managing patients with the disorder for allergy and immunology health professionals, according to survey findings reported in the Annals of Allergy, Asthma, & Immunology.

The findings are based on a survey of members of the American College of Allergy, Asthma, and Immunology (ACAAI) that sought to determine their experiences in treating patients with HAE.

The online survey was conducted for ACAAI by the Harris Poll, which surveyed 2996 US ACAAI members. All respondents included the final analysis were currently practicing allergy or immunology and seeing or treating at least 1 patient with HAE each year. Data were collected from April 13 to May 3, 2022.

A total of 138 respondents (mean age, 48 years; 54% male) completed the survey and were included, of whom 80 were physician members, 48 were physician fellows, and 10 were allied health professionals (nurse practitioners or physician assistants).

The respondents, who saw a mean number of 9 patients with HAE annually, reported that 66% of their patients with HAE had type I, 15% had type II, and 19% had HAE with normal C1-esterase inhibitor (C1nl-INH; formerly called type III HAE). An average of 12% of respondents said their patients lived in a rural area.

Misdiagnosis of symptoms, access to a specialist, and affording medications remain high-priority concerns among those surveyed, and these obstacles are heightened for patients living in rural areas.

About 55% of respondents diagnosed more than half of their patients with HAE. Top concerns of respondents when selecting a treatment were the frequency of HAE attacks (90%) and severity of attacks (88%).

The top diagnostic challenge reported by respondents was misattributing HAE symptoms to other conditions (82%). Another challenge commonly reported (by 66% of respondents) was the absence of common indicators of HAE in every patient.

Patients’ inability to afford treatment, including insurance coverage and out-of-pocket costs, was also reported by approximately 76% of respondents as a challenge of providing HAE treatment.

Survey findings indicated that rural areas have significant barriers to care; 59% of respondents agreed with the statement, “Some of my HAE patients have to travel a great distance to see me/their health care provider.” Also, 23% of respondents said their patients needed to travel an hour or more to get to the practitioner’s office. When clinicians were asked how they could better support the treatment of patients in rural settings, and the most frequently selected option (72%) was to increase the availability of treatments that could be administered at home.

About 93% of the respondents provide telehealth services to patients within the same state where their practice is located, and 82% do not provide telehealth services to any patients outside the state in which their practice is located. Since the COVID-19 pandemic began, 86% of respondents have been using telehealth for appointments at least occasionally, and 17% have been doing so “most” or “all” of the time.

Among several limitations, the sample size of respondents who completed the survey was less than 100 for some analyses, and only 12% of the respondents’ patients lived in rural areas. Other limitations include the likelihood of respondents who estimated their answers to some questions.

“Our survey results highlight the real and ongoing challenges with diagnosing and treating patients with HAE,” stated the investigators. “Misdiagnosis of symptoms, access to a specialist, and affording medications remain high-priority concerns among those surveyed, and these obstacles are heightened for patients living in rural areas. HAE is rare, but collectively, rare diseases affect many patients.”

Disclosure: Takeda Pharmaceuticals USA, Inc provided support in writing and editing of this manuscript through an independent medical education grant. Some of the study authors declared affiliations with biotech, pharmaceutical, and/or device companies. Please see the original reference for a full list of authors’ disclosures.


Meadows JA, Anderson J, Gower RG. Challenges in the management of hereditary angioedema in urban and rural settings: results of a United States survey. Ann Allergy Asthma Immunol. Published online March 12, 2023. doi:10.1016/j.anai.2023.03.005