Among physicians from different medical specialties, disease awareness of hereditary angioedema (HAE) varies, which suggests the importance of educational activities such as academic seminars and medical specialty examinations, according to a new study published in the Journal of Cutaneous Immunology and Allergy.

The investigators of the current study sought to identify issues linked to the diagnostic processes of HAE in Japan and to determine levels of disease awareness among physicians from a variety of specialties.

A noninterventional, cross-sectional study design was used to obtain data via a web-based quantitative survey through a commercially available physician panel. The participants comprised a total of 692 physicians who were recruited through the Nikkei BP Panel. All of the target physicians were members of Nikkei Medical Online, a portal site that is run by Nikkei BP in Tokyo, Japan.


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The current analysis included over 200 physicians from the field of internal medicine, as well as over 100 each from the fields of pediatrics, dermatology, gastroenterological surgery, and emergency medicine.

HAE is a rare disease that is characterized by frequent attacks of unpredictable edema that can occur anywhere in the body. Laryngeal attacks are particularly life-threatening, whereas abdominal attacks are occasionally treated incorrectly with laparotomy. Symptoms suggestive of HAE include subcutaneous edema, submucosal edema, gastrointestinal complaints, convulsions, and laryngeal edema.

Read more about HAE overview

The global prevalence of HAE is estimated to be 1 in 10,000 to 1 in 50,000 individuals. Although it is estimated that approximately 2500 patients with HAE reside in Japan, as of 2018, only about 450 patients with the disease have been reported in that country, thus implying a high number of individuals residing in Japan with undiagnosed HAE.

In Japan, the average time from symptom onset to diagnosis is approximately 16 years, compared with
8 years in the United States and 10 years in the United Kingdom. Awareness of HAE varies among medical specialties, with internal medicine physicians and gastroenterology surgeons appearing to have a lower awareness of the disease than those in other medical specialties. Further, in Japan, regional differences regarding awareness of HAE exist as well.

“Awareness of HAE among physicians can lead not only to early diagnosis but also to the elimination of unnecessary and invasive diagnoses and treatments,” the researchers said. Patients with HAE may remain undiagnosed for years because of a general lack of knowledge about the disease on the part of physicians.

Understanding the importance of complement blood testing for HAE, recognizing life-threatening airway symptoms, and having a basic knowledge of abdominal pain in these patients are all helpful when rendering a diagnosis of the disease.

Reference

Fukunaga A, Kishimoto M, Oh A, et al. Physician awareness and understanding of hereditary angioedema: a web-based study in Japan. J Cut Immunol Allergy. Published online June 29, 2022. doi:10.1002/cia2.12265