What is the management approach for protein losing enteropathy?

Management of Protein-Losing Enteropathy

The management of protein-losing enteropathy requires identifying and treating the underlying cause while implementing dietary modifications—specifically a diet low in long-chain triglycerides (<5% of total energy) and enriched with medium-chain triglycerides (>20% of total energy) for lymphatic causes, combined with high protein intake (≥1.2 g/kg/day) to address hypoalbuminemia. 1

Initial Diagnostic Approach

The first step is determining the etiology through targeted evaluation:

• Cardiac screening with echocardiography is essential to identify cardiac causes such as Fontan circulation or constrictive pericarditis 2
• Endoscopy with biopsies should be performed to identify mucosal abnormalities and specific intestinal pathology 2
• Laboratory evaluation including complete blood count, liver function tests, and serum albumin levels establishes baseline severity 2
• Fecal alpha-1-antitrypsin clearance confirms the diagnosis of protein-losing enteropathy 3, 4

Etiology-Specific Treatment

For Cardiac Causes (Fontan Circulation, Constrictive Pericarditis)

Heart transplantation should be considered for severe systemic ventricular dysfunction or refractory protein-losing enteropathy in Fontan patients. 1, 2

• Creation of atrial septal fenestration or Fontan conversion is recommended for protein-losing enteropathy not responsive to medical therapy 1, 2
• Surgical pericardiectomy is the definitive treatment for constrictive pericarditis 1, 2
• Pulmonary vasoactive medications may improve exercise capacity and help manage symptoms in Fontan patients 1

For Primary Intestinal Disorders

Inflammatory bowel disease requires immunosuppressive therapy targeting the underlying inflammation, with surgical resection (proctocolectomy) reserved for refractory cases 1, 4

• Celiac disease or seronegative enteropathy should be managed with a gluten-free diet with dietitian support, followed by repeat duodenal biopsies at 12 months 2
• Infectious causes require appropriate antimicrobial therapy based on stool cultures and serologic evaluation 1, 2
• Medication-induced enteropathy (olmesartan, mycophenolate mofetil, azathioprine) necessitates immediate discontinuation of the offending agent 1, 2

Dietary Management (Critical for All Patients)

A low long-chain triglyceride diet (<5% of total energy intake) with medium-chain triglyceride enrichment (>20% of total energy intake) is the cornerstone of nutritional management, particularly for intestinal lymphangiectasia. 1, 5

• Energy intake should be at least 30 kcal/kg actual body weight/day to address malnutrition 1
• Protein intake should be at least 1.2 g/kg actual body weight/day due to ongoing protein losses 1
• Medium-chain triglycerides bypass intestinal lymphatics and are absorbed directly into the bloodstream, reducing chyle production and protein loss 5, 3
• Fat-soluble vitamin supplementation is necessary due to fat restriction 3

Pharmacological Management

Budesonide may be beneficial for patients with hypoalbuminemia poorly responsive to other therapies, though it requires close monitoring for hypercortisolism. 1

• Aldosterone antagonists or subcutaneous unfractionated heparin may stabilize the proteoglycan layer of the gut in Fontan-related protein-losing enteropathy 1
• Octreotide (long-acting somatostatin analog) can be considered for refractory cases, particularly those due to AA amyloidosis 6

Monitoring and Follow-Up

Regular monitoring of serum albumin levels is essential to evaluate treatment efficacy and guide therapy adjustments. 1, 2

• Repeat endoscopy with biopsies should be performed approximately 12 months after starting treatment to assess histologic improvement 1, 2
• Cardiac evaluation with echocardiography and electrocardiogram is recommended for ongoing assessment in cardiac-related cases 1
• Micronutrient monitoring is necessary for patients on fat-restricted diets, especially in infants and young children 5

Common Pitfalls to Avoid

• Do not delay cardiac evaluation in patients with unexplained protein-losing enteropathy, as Fontan circulation and constrictive pericarditis are treatable cardiac causes 7
• Do not use standard oral steroids without considering budesonide first, as it has fewer systemic effects 1
• Do not continue offending medications (olmesartan, mycophenolate, azathioprine) once medication-induced enteropathy is suspected 1, 2
• Do not provide long-chain triglycerides in patients with lymphatic causes, as this worsens protein loss 1, 5