What is the treatment for hyperchylomicronemia?

Treatment for Hyperchylomicronemia

The treatment for hyperchylomicronemia requires aggressive dietary fat restriction, with total fat limited to 10-15% of total calories for triglycerides ≥1,000 mg/dL and 20-25% for triglycerides 500-999 mg/dL, combined with pharmacotherapy such as fibrates and management of secondary causes. 1, 2

Dietary Modifications

• For patients with triglycerides ≥1,000 mg/dL, restrict total fat to <20-40g per day or 10-15% of total calories 1
• For patients with triglycerides 500-999 mg/dL, limit fat to 20-25% of total calories 1
• Eliminate added sugars completely for severe hypertriglyceridemia 2, 3
• Restrict or completely eliminate alcohol consumption 1, 3
• Increase soluble fiber intake to >10g/day 1, 2
• Avoid simple and refined carbohydrates 1, 3
• Implement weight loss strategies if overweight/obese, targeting 5-10% weight loss 2, 4

Pharmacologic Therapy

• Fibrates are indicated as adjunctive therapy for severe hypertriglyceridemia 5, 2
  • Initial dose of fenofibrate is 54-160 mg per day, individualized according to patient response 5
  • Fibrates can reduce triglycerides by 46-54% in patients with severe hypertriglyceridemia 5
• Prescription omega-3 fatty acids are recommended as adjunctive therapy 1, 3
• For patients with diabetes, improving glycemic control is essential as it can significantly reduce triglyceride levels 1, 3
• In acute severe cases with risk of pancreatitis, insulin therapy may be considered to rapidly lower triglyceride levels 3, 6

Management of Secondary Causes

• Evaluate and treat uncontrolled diabetes mellitus 2, 3
• Assess for hypothyroidism, which can contribute to elevated triglycerides 2, 7
• Review medications that may raise triglycerides (e.g., estrogens, corticosteroids, beta-blockers) 1, 7
• Address chronic kidney disease and liver disease if present 2, 7
• Eliminate alcohol consumption completely 1, 3

Special Considerations

• For triglycerides ≥1,000 mg/dL, the primary goal is to reduce levels below 500 mg/dL to prevent acute pancreatitis 3, 7
• Patients with familial chylomicronemia syndrome (FCS) may have limited response to traditional therapies due to genetic defects in lipoprotein lipase or its regulators 8, 9
• In cases of acute pancreatitis due to severe hypertriglyceridemia, plasmapheresis or LDL-apheresis may be considered 10, 6
• Newer therapies targeting apolipoprotein C-III (volanesorsen, olezarsen) show promise for FCS but are still investigational 8, 9

Clinical Pearls

• Medical nutrition therapy plays a pivotal role in reducing triglyceride levels, with similar effectiveness regardless of medication use 1
• Physical activity (150 minutes/week moderate or 75 minutes/week vigorous) should be recommended alongside dietary modifications 2, 4
• Low-carbohydrate diets may be more effective than low-fat diets for triglyceride reduction in some patients 2, 4
• The combination of dietary intervention and regular physical activity can result in 20-50% reductions in triglyceride levels 2, 3
• Registered dietitian nutritionists are essential for providing individualized medical nutrition therapy for optimal lipid lowering 1