Hereditary Angioedema is a dominantly inherited disease. Routine screening of autoantibodies (AAB) is not recommended for individuals with Hereditary Angioedema; however, prevalence of these antibodies in Hereditary Angioedema patients is not well documented. We aim to determine the prevalence of AAB so that individuals at risk of developing autoimmune diseases can be identified. Fifteen patients with Hereditary Angioedema attended at Clementino Fraga Filho University Hospital accepted to participate in this study. Prevalence of AAB was 40%. Our data indicate high prevalence of AAB in patients with Hereditary Angioedema. Large-scale studies should be considered to determine the significance of these AAB in the follow-up care of patients with Hereditary Angioedema.
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http://dx.doi.org/10.1590/s0365-05962012000200030 | DOI Listing |
Ann Allergy Asthma Immunol
January 2025
Takeda Pharmaceuticals USA, Inc., Lexington, MA, USA.
Background: Hereditary angioedema (HAE) is a rare disorder in which unpredictable angioedema attacks significantly impact patient quality of life (QoL). There is limited information regarding patient experiences and perspectives of HAE management within underrepresented racial and ethnic groups.
Objective: To gain insight into the experiences and perspectives of medical care and treatment for HAE among underrepresented racial and ethnic groups in the US.
JAAD Case Rep
February 2025
Department of Dermatology, Henan Provincial People's Hospital, Zhengzhou University People's Hospital, Henan University People's Hospital, Zhengzhou, China.
J Comp Eff Res
January 2025
ICON plc, Insights, Evidence & Value - Health Economics & Epidemiology, Langen, Germany.
To compare the efficacy and safety of lanadelumab versus other approved long-term prophylaxis (LTP) treatments in patients with pediatric hereditary angioedema (HAE) aged <12 years. A systematic literature review was conducted to identify studies of LTP in patients with HAE aged <12 years. Two studies met the inclusion criteria in an indirect treatment comparison of efficacy and safety data in pediatric HAE patients.
View Article and Find Full Text PDFInt Arch Allergy Immunol
January 2025
Introduction: Hereditary angioedema (HAE) is a rare disease caused by dysfunction or lack of the C1 esterase inhibitor (C1-INH) protein. The true prevalence of HAE, and whether this prevalence differs across regions, is uncertain.
Methods: To estimate the prevalence of HAE worldwide, a systematic review and meta-analysis was performed.
JCI Insight
January 2025
Medicine, Washington University School of Medicine, St. Louis, United States of America.
Hereditary angioedema is an autosomal dominant disorder caused by defects in C1-esterase inhibitor (C1-INH), resulting in poorly controlled activation of the kallikrein-kinin system and bradykinin overproduction. C1-INH is a heavily glycosylated protein in the serine protease inhibitor (SERPIN) family, yet the role of these glycosylation sites remains unclear. To elucidate the functional impact of N-glycosylation in the SERPIN domain of C1-INH, we engineered four sets consisting of 26 variants at or near the N-linked sequon (NXS/T).
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