Diagnosis and Management of Hereditary Angioedema in Latin America: International Recommendations and Regional Practice Realities

Background: Current international hereditary angioedema (HAE) guidelines and treat-to-target recommendations consistently emphasize accurate diagnosis, early on-demand treatment of attacks, appropriate short-term prophylaxis for procedures, individualized long-term prophylaxis (LTP), and routine assessment of disease control and quality of life. Published information on how these recommendations are implemented in Latin America remains limited. We therefore aimed to assess the extent to which a Latin American expert panel converges with recommendations across current international HAE guidelines and consensus documents and to identify the principal barriers affecting real-world implementation in the region. Methods: A scientific committee developed and externally validated a 74-item questionnaire (domains summarized in Supplementary Table S1) and conducted a 2-round Delphi process among Latin American HAE experts. Consensus items were rated on a 9-point agreement scale, with consensus defined a priori as at least two-thirds of panelists scoring within a 3-point band in the agreement (7-9) or disagreement (1-3) range. Descriptive items captured expert-reported diagnostic testing, acute and preventive treatment patterns, monitoring practices, and implementation determinants using 5-point Likert scales. Instrument validation (July-November 2024) demonstrated high inter-rater agreement (Kendall W = 0.84; p < 0.001) and excellent internal consistency (Cronbach alpha = 0.92). Results: Thirty experts from 10 countries completed the process (Argentina, Brazil, Chile, Colombia, Ecuador, Mexico, Panama, Peru, Uruguay, Venezuela). Panel consensus broadly converged with recommendations across current international HAE guidelines and consensus documents on core diagnostic work-up (C4 plus C1-INH antigen and function; 84% consensus), selective use of genetics (97% consensus), first-line targeted LTP (pdC1-INH, Lanadelumab, Berotralstat; 94% consensus), retention of on-demand therapy for breakthrough attacks and for patients without access to LTP, routine monitoring, and management in special populations. The principal novel finding was an implementation feasibility gap: frequent or constant interruptions in prophylaxis supply were reported by 19/30 experts (63%), including 13/23 (57%) practicing in settings where public or insurance coverage was reported. Across the panel, cost (19/30; 63%) and medicine availability (8/30; 27%) were the most commonly cited barriers, with additional constraints related to diagnostic testing access and administrative pathways. Conclusions: Latin American expert consensus largely converges with recommendations across current international HAE guidelines and consensus documents on diagnosis, acute management, prophylaxis selection, monitoring, and special-population care. The dominant divergence is not clinical disagreement but implementation feasibility—particularly access to diagnostic testing, financing mechanisms, medicine availability, and continuity of supply. These findings provide region-specific evidence to contextualize international recommendations and support implementation strategies tailored to Latin America.