From the Guidelines
The management of hereditary hemorrhagic telangiectasia (HHT) should prioritize a stepwise approach, starting with moisturizing topical therapies, followed by oral tranexamic acid, and then considering systemic antiangiogenic agents like bevacizumab for epistaxis that has failed to respond to initial treatments, as recommended by the Second International HHT Guidelines 1.
Key Recommendations
- For epistaxis, consider the use of oral tranexamic acid, starting at 500 mg twice daily, gradually increasing up to 1000 mg 4 times daily or 1500 mg 3 times daily, with contraindications including recent thrombosis and relative contraindications including atrial fibrillation or known thrombophilia 1.
- Systemic antiangiogenic agents, such as bevacizumab, can be considered for epistaxis that has failed to respond to moisturizing topical therapies, ablative therapies, and/or tranexamic acid, with a recommended induction dose of 5 mg/kg every 2 weeks for 4-6 doses, followed by maintenance dosing variable, but potentially 5 mg/kg every 1-3 months 1.
- For GI bleeding, grade the severity of HHT-related GI bleeding and consider treatment with oral antifibrinolytics for mild cases, and intravenous bevacizumab or other systemic antiangiogenic therapy for moderate to severe cases 1.
Rationale
The Second International HHT Guidelines provide a framework for managing HHT, emphasizing a stepwise approach to treatment, with a focus on reducing bleeding complications and improving quality of life 1. The guidelines recommend considering systemic antiangiogenic agents, such as bevacizumab, for epistaxis that has failed to respond to initial treatments, based on evidence from studies demonstrating substantial improvements in epistaxis severity and quality of life 1.
Important Considerations
- The use of systemic antiangiogenic agents, such as bevacizumab, requires careful consideration of the risks and benefits, including the potential for hypertension, proteinuria, infection, delayed wound healing, and venous thromboembolism (VTE) 1.
- Shared decision-making with patients is crucial, taking into account individual preferences, values, and comorbidities 1.
- The guidelines also emphasize the importance of anemia screening and management, as anemia is a common complication of HHT, resulting from chronic bleeding and iron deficiency 1.
From the Research
Baveno VII Summary
- The Baveno VII conference redefined screening and prophylaxis in patients with cirrhosis 2.
- The Baveno criteria may not be appropriate to exclude high-risk esophageal varices (EV) in HCC patients, and endoscopy should be performed except in HCC patients with a liver stiffness measurement (LSM) ≥25 kPa, who should benefit from nonselective beta-blockers (NSSBs) without performing endoscopy 2.
- Non-selective beta blockers are very useful drugs in preventing first variceal bleeding and re-bleeding in patients with cirrhosis 3.
- The use of non-selective beta blockers in portal hypertension has been studied extensively, with satisfactory reduction in portal pressure possible in a third to half of patients with propranolol and nadolol 4.
- A tailored approach to the use of nonselective beta-blockers in patients with portal hypertension is proposed, taking into account safety concerns in advanced disease, such as in patients with refractory ascites 5.
Key Points
- Non-selective beta blockers reduce the bleeding risk from 30 to 15% in primary prophylaxis, and from 60 to 42% in secondary prophylaxis in the first year 3.
- The reduction in hepatic venous pressure gradient (HVPG) is a key factor in reducing the risk of bleeding, with a reduction of > 20% from basal values associated with a low bleeding risk 3.
- Combining non-selective beta blockers with nitrates or endoscopic band ligation may be more effective in reducing portal pressure and preventing variceal bleeding 4, 6.
- Early covered-TIPS within 72 h should be considered in patients at high risk of treatment failure despite using combination therapy with NSBB + EBL 6.