Uncovering the true burden of hereditary angioedema due to C1-inhibitor deficiency: A focus on the Asia-Pacific region

Honda, Daisuke; Li, Philip Hei; Jindal, Ankur Kumar; Katelaris, Constance H.; Zhi, Yu-Xiang; Thong, Bernard Yu-Hor; Longhurst, Hilary J.

doi:10.1016/j.jaci.2023.09.039

Cited by 4 publications

(3 citation statements)

References 33 publications

Supporting

Mentioning

Contrasting

Order By: Relevance

“…Studies have reported broad benefits telemedicine in clinical immunology and allergy clinics since its adoption increased during the COVID-19 pandemic ( 14 , 15 ). Additionally, globally many patients with HAE live in developing nations in the Asia Pacific region, a large proportion of whom reside in rural areas, and up to 99% of affected patients are thought to be undiagnosed in some countries ( 16 , 17 ). The use of digital health technologies coordinated through specialized HAE centers to provide expert advice to rural healthcare providers and directly to patients using smartphones could help address these care gaps as has been described for other diseases ( 18 ).…”

Section: Discussionmentioning

confidence: 99%

Centralized care model for hereditary angioedema overcomes geographical barriers

Holmes,

Srinivasan,

Borle

et al. 2024

Front. Immunol.

View full text Add to dashboard Cite

Hereditary angioedema due to C1 inhibitor deficiency (HAE) is a rare inborn error of immunity that presents with episodic swelling. Management is multifaceted and includes on-demand treatment of swelling episodes, short-term prophylaxis to prevent swelling episodes from procedures, and long-term prophylaxis (LTP) to prevent angioedema on an ongoing basis. All approved on-demand therapies are parenteral, necessitating patient training for home administration, particularly intravenous C1 inhibitor. These complexities can result in care gaps for rural HAE patients. We conducted a cross-sectional study at our Angioedema Center of Reference and Excellence to assess the care provided to urban and rural patients. The proportion of patients receiving LTP, proportion of patients diagnosed as children, and disease control measured using the Angioedema Control Test (AECT) were collected. Logistic and Poisson regression models adjusted for age and sex were used to compare the two groups. The proportion using LTP was similar at 62% and 61% in urban and rural patients, respectively (odds ratio [OR] 1.01 (CI 95% 0.34-2.99)). Among urban patients, 52% were diagnosed as children compared to 60% among rural residents (1.43 (0.37-5.56)). The mean (IQR) AECT score was 14.0 (8.5-15.5) in urban patients and 13.0 (10.0-14.0) in rural patients (Poisson β -0.001 (-0.23-0.23). These data indicate that rural patients received similar high-quality care. We attribute these findings to the centralized care model employed in which HAE patients in the region are seen at a single comprehensive care clinic.

show abstract

Section: Discussionmentioning

confidence: 99%

Centralized care model for hereditary angioedema overcomes geographical barriers

Holmes,

Srinivasan,

Borle

et al. 2024

Front. Immunol.

View full text Add to dashboard Cite

show abstract

“…Furthermore, unpredictability and fear of future attacks lead to severe impairments in health-related quality of life (HRQoL). 2 , 3 As of now, there are no reliable biomarkers for measuring or monitoring disease activity, impact, or control. Patient-reported outcomes remain crucial and are recommended in angioedema guidelines.…”

Section: Introductionmentioning

confidence: 99%

“… 6 , 7 , 8 , 9 , 10 Currently, none of these have been validated for Chinese individuals, and their interrelationship in this population is unknown. 3 To address this issue, we validated the Chinese versions of the AAS, AE-QoL questionnaire, and AECT in a population of Chinese patients with CSU and HAE along with recurrent angioedema.…”

Section: Introductionmentioning

confidence: 99%

Validation and correlations of the Angioedema Activity Score (AAS), Angioedema Quality of Life (AE-QoL) questionnaire, and Angioedema Control Test (AECT) in Chinese patients with angioedema

Mak,

Wong,

et al. 2024

Journal of Allergy and Clinical Immunology: Global

View full text Add to dashboard Cite

Disparities in geography and race in hereditary angioedema: Analysis of 64 trials and 4354 patients

Mak,

So,

Longhurst

et al. 2024

The Journal of Allergy and Clinical Immunology: In Practice

View full text Add to dashboard Cite

Uncovering the true burden of hereditary angioedema due to C1-inhibitor deficiency: A focus on the Asia-Pacific region

Cited by 4 publications

References 33 publications

Centralized care model for hereditary angioedema overcomes geographical barriers

Centralized care model for hereditary angioedema overcomes geographical barriers

Validation and correlations of the Angioedema Activity Score (AAS), Angioedema Quality of Life (AE-QoL) questionnaire, and Angioedema Control Test (AECT) in Chinese patients with angioedema

Disparities in geography and race in hereditary angioedema: Analysis of 64 trials and 4354 patients

Contact Info

Product

Resources

About