The Effect of Systemic Bevacizumab on Epistaxis-Related Outcomes in Hereditary Hemorrhagic Telangiectasia: A Systematic Review and Meta-Analysis.

Background: The evidence-based recommendation for the use of systemic bevacizumab to treat refractory epistaxis in hereditary hemorrhagic telangiectasia (HHT) is based on limited and historical data. An updated synthesis of the available literature is warranted.

Methods: A systematic review and meta-analysis were conducted using PRISMA guidelines to evaluate the use of systemic bevacizumab for HHT-related epistaxis. A search was conducted using three databases up to September 2024 for studies assessing the effect of systemic bevacizumab use on epistaxis outcomes in HHT. Outcome measures included epistaxis severity score (ESS), hemoglobin, red blood cell (RBC), and iron transfusion requirements and adverse effects. Random effects meta-analysis was performed for change in ESS (ΔESS) and change in hemoglobin (ΔHb). Literature quality was assessed using the Joanne Briggs Institute critical appraisal tools.

Results: A total of 10 studies with 225 total patients were included. Systemic bevacizumab was associated with a significant posttreatment reduction in mean ESS of -3.33 (95% CI -3.62 to -3.03) and a significant increase in mean Hb of 2.38 g/dL (95% CI 1.45-3.30) compared to pretreatment. All cohort studies found a significant reduction in RBC and iron transfusions following systemic bevacizumab treatment. The most frequently reported adverse effect of systemic bevacizumab was hypertension.

Conclusion: Systemic bevacizumab is associated with significant improvements in epistaxis severity, hemoglobin, and transfusion requirements in HHT-related epistaxis. Adequately powered studies are required to strengthen this finding. Patient selection criteria, standardized maintenance dosing, and long-term treatment data require further study.