Treatment for Anasarca Related to Protein-Losing Enteropathy
The treatment for anasarca related to protein-losing enteropathy should focus primarily on the underlying cause, with dietary modifications being the cornerstone of therapy, specifically a diet low in long-chain triglycerides (<5% of total energy intake) and enriched with medium-chain triglycerides (>20% of total energy intake). 1
Dietary Management
- A diet low in long-chain triglycerides (<5% of total energy intake) and enriched with medium-chain triglycerides (>20% of total energy intake) is recommended for protein-losing enteropathy, particularly for intestinal lymphangiectasia 1
- Energy and protein intakes should be at least 30 kcal/kg actual body weight/day and 1.2 g/kg actual body weight/day to address malnutrition 1
- A protein-energy enriched diet should be provided due to frequent malnutrition in these patients 1
Cardiac-Related Protein-Losing Enteropathy
- Heart transplantation may be beneficial for severe systemic ventricular dysfunction or protein-losing enteropathy in patients with Fontan circulation 1
- Creation of an atrial septal fenestration or Fontan conversion should be considered for protein-losing enteropathy not amenable to medical therapy in Fontan patients 1
- Pulmonary vasoactive medications can improve exercise capacity in adults with Fontan repair and may help manage symptoms of protein-losing enteropathy 1
Pharmacological Management
- Corticosteroids, specifically budesonide, may be helpful for patients with hypoalbuminemia in the setting of protein-losing enteropathy poorly responsive to other therapies 1
- Budesonide has fewer systemic effects than other oral steroids, but close monitoring for signs of hypercortisolism remains necessary 1
- Octreotide (a somatostatin analog) may be considered as a treatment option with favorable but limited anecdotal experience 1, 2
- Aldosterone antagonists or subcutaneous unfractionated heparin may stabilize the proteoglycan layer of the gut in Fontan-related protein-losing enteropathy 1
Management Based on Specific Etiologies
- For inflammatory bowel disease-related protein-losing enteropathy, immunosuppressive therapy targeting the underlying inflammation is indicated 3, 4
- For infectious causes, appropriate antimicrobial therapy should be initiated 3, 4, 5
- For medication-induced enteropathy, discontinuation of the offending agent (such as olmesartan, mycophenolate mofetil, or azathioprine) is recommended 3, 4
- For constrictive pericarditis, surgical pericardiectomy is the definitive treatment 3, 4
Monitoring and Follow-up
- Regular monitoring of serum albumin levels is essential to evaluate treatment efficacy 3, 6
- Repeat endoscopy with biopsies may be necessary to assess response to therapy, typically after 12 months of treatment 3
- For cardiac causes, regular cardiac evaluation with echocardiography, electrocardiogram, and other cardiac testing is recommended 1
Combination Therapy Approach
- A combination of dietary modifications, targeted therapy for the underlying cause, and supportive care often produces improved outcomes compared to historic controls 1
- For severe cases with anasarca, additional supportive measures including diuretics, albumin infusions, and nutritional support may be necessary 7, 8
- In cases refractory to conventional treatment, consideration of novel therapies or surgical interventions based on the underlying etiology is warranted 2, 9