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The 2025 WAO Guidelines for the classification, diagnosis, and treatment of hereditary angioedema, with consideration of worldwide disparities - PubMed

3 hours ago
  • #medical guidelines
  • #rare disease management
  • #hereditary angioedema
  • The 2025 WAO Guidelines provide a comprehensive framework for hereditary angioedema (HAE) management, emphasizing global applicability.
  • HAE classification is updated: HAE-C1-INH unifies types 1 and 2; HAE-nC1-INH includes forms linked to various genetic mutations and unknown causes.
  • Diagnosis focuses on early clinical recognition using characteristic features and a simplified algorithm with C1 inhibitor functional assay as the initial test.
  • Management includes on-demand therapy, short-term prophylaxis, and individualized long-term prophylaxis within a treat-to-target framework for disease control.
  • Guideline development involved 40 experts from 22 countries, using systematic literature review, GRADE framework adapted for rare diseases, and Delphi consensus.
  • Global disparities are considered, with alternative diagnostic pathways for resource-limited settings and emphasis on patient empowerment and structured education.
  • The guidelines aim for equitable, high-quality care across healthcare systems, reducing avoidable morbidity and mortality from HAE.