HADES is a predictive scoring system to enhance the early detection of hereditary angioedema (HAE), a rare condition often misdiagnosed due to overlapping symptoms with other disorders. Using a few clinical variables, the score demonstrated strong performance, with low scores providing high sensitivity and negative predictive value, and high scores offering high specificity and positive predictive value. This simple tool, developed in collaboration with HAE academic and pharma experts, has the potential to improve HAE suspicion.
What’s new: HADES is a predictive scoring system specifically designed for detecting hereditary angioedema (HAE) based on clinical parameters. This tool fills a critical gap by addressing the challenges of misdiagnosis and delayed treatment caused by HAE’s rarity and overlapping symptoms with other conditions. By offering a simple, questionnaire-based approach, HADES introduces a practical and accessible method for improving clinical suspicion of HAE.
Key insights: Detection of HAE often faces significant delays, with an average of 8.5 years reported in Europe and even longer in other regions. HADES leverages a few clinical variables (e.g., age of onset, limb oedema, abdominal pain, absence of urticaria) to predict HAE. The tool achieves high sensitivity and specificity, providing clinicians with a reliable method to identify high-risk patients and prioritize confirmatory testing.
How it works: HADES employs a straightforward, questionnaire-based methodology. A simple survey through an app collects key clinical information. Scores are grouped into different categories that correlate with varying probabilities of HAE.
Why it matters: HAE is a life-threatening condition that often goes unnoticed due to low awareness and overlapping symptoms with other disorders. This leads to significant delays in treatment, impacting patient outcomes and quality of life. HADES provides: i) a practical, scalable solution for clinicians to improve HAE suspicion; ii) a means to increase early detection and improve access to life-saving treatments; iii) a valuable educational tool for increasing awareness of HAE among healthcare providers and patients. HADES represents a transformative step forward in addressing the unmet needs for HAE, offering hope for earlier detection and better management of this rare condition.
Bibliography: https://www.jaci-global.org/article/S2772-8293(25)00015-3/fulltext
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