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Background: Hereditary angioedema (HAE) is a rare, potentially life-threatening condition. In recent years, the emergence of novel therapies has likely contributed to substantial growth in HAE research; however, a comprehensive review of the HAE research landscape remains lacking. Methods: We conducted a systematic literature search on the Web of Science Core Collection and extracted metadata for all HAE records from 1972 to 2023. Bibliometric analysis was performed to outline trends in research productivity, participation, collaboration, focus, and global patterns in HAE research. Results: We analysed a total of 2443 publications. Research output increased over time (annual growth rate = 6.34%) and was correlated to countries' Human Development Index and Gross National Income per capita (Spearman ρ > 0.40, p 85% of HAE studies were led by researchers from Europe and America. However, contributions from the Western Pacific rose significantly (1972-2010: 4.6% vs 2011-2023: 8.6%, p < 0.001). International collaboration rose significantly over time, increasing from 10.9% (1972-2010) to 27.7% (2011-2023) (p < 0.001). Keyword and trend analyses indicated that research focus shifted from pathophysiology and diagnosis to patient outcomes, treatment and prophylaxis. Specifically, long-term prophylaxis emerged as a key area in recent research. Conclusions: The volume of HAE-related publications is rapidly increasing, reflecting a transition from basic science to more clinically oriented research, particularly focusing on long-term prophylaxis. While global research efforts are becoming increasingly collaborative and diverse, participation from Africa, the Eastern Mediterranean, and South-East Asia remains limited. Greater support for these regions is essential to ensure equitable progress in HAE research and care.
Mak et al. (Sun,) studied this question.