Hereditary angioedema diagnosis evaluation score (HADES): A new clinical scoring system for predicting hereditary angioedema with C1 inhibitor deficiency.

• Tác giả: Laura Arias, Laura Brion, Carlos María Galmarini, Andrea Pino, Paula Rozenfeld, Rafael Zamora, Ricardo Zwiener
• Ngôn ngữ: eng
• Thông tin xuất bản: United States : The journal of allergy and clinical immunology. Global , 2025
• Bộ sưu tập: NCBI
• ID: 675700

Tóm tắt

 BACKGROUND: Diagnosis of hereditary angioedema (HAE) poses challenges because of its rarity and its overlapping symptoms with allergic and gastrointestinal conditions, resulting in misdiagnosis. OBJECTIVE: We developed a predictive score using clinical variables for suspected HAE patients with C1 inhibitor deficiency (HAE-C1INH) to increase suspicion of HAE and thus improve diagnosis. METHODS: The HADES (HAE diagnosis evaluation score) study used a nationwide retrospective cohort of individuals with suspected HAE-C1INH in Argentina. A questionnaire was designed to collect relevant clinical information on possible predictors for HAE. Blood samples were analyzed for C1-INH/C1q levels and C1-INH function. A predictive score was developed from the odds ratios derived from multivariate logistic regression analysis. RESULTS: The study included 2423 individuals (1642 suspected index cases and 781 family cases). Only patients with confirmed HAE types I or II (n = 499) were included in the final analysis
  acquired angioedema/ CONCLUSIONS: The predictive HADES offers a simple and efficient method for improving testing for suspicion of HAE by using clinical parameters. Further validation studies are required to confirm its reliability and accuracy.