Protein-Losing Enteropathy Treatment Approach

Diagnostic Evaluation

The European Society of Cardiology recommends cardiac screening with echocardiogram to assess for cardiac causes such as Fontan circulation or constrictive pericarditis 1
The American Gastroenterological Association suggests endoscopy with biopsies to identify mucosal abnormalities and potential etiologies 2

The European Society for Clinical Nutrition and Metabolism recommends a diet low in long-chain triglycerides (<5% of total energy intake) and enriched with medium-chain triglycerides (>20% of total energy intake) for patients with intestinal lymphangiectasia 3
The American Gastroenterological Association recommends a gluten-free diet with dietitian support for patients with seronegative celiac disease 2
The American Gastroenterological Association suggests follow-up with repeat duodenal biopsies approximately 12 months after starting treatment to assess histologic improvement 2

The American College of Cardiology recommends heart transplantation for severe systemic ventricular dysfunction or protein-losing enteropathy in patients with Fontan circulation 4
The American College of Cardiology suggests creation of an atrial septal fenestration or Fontan conversion for protein-losing enteropathy not amenable to medical therapy 4
The European Society of Cardiology recommends surgical pericardiectomy for definitive treatment of constrictive pericarditis 1

The American Gastroenterological Association recommends regular monitoring of serum albumin levels to evaluate treatment efficacy 2
The American Gastroenterological Association suggests repeat endoscopy with biopsies to assess response to therapy, typically after 12 months of treatment 2

The American Gastroenterological Association recommends discontinuation of the offending agent (such as olmesartan, mycophenolate mofetil, or azathioprine) for patients with medication-induced enteropathy 2

The American Gastroenterological Association recommends laboratory evaluation, including complete blood count and liver function tests, for the diagnosis of PLE 5
The American Heart Association suggests that cardiac screening, such as echocardiogram, is necessary to assess for cardiac causes of PLE 5

The American Gastroenterological Association identifies erosive gastrointestinal disorders, such as inflammatory bowel disease and infections, as primary causes of PLE 6, 7
The American Gastroenterological Association also identifies non-erosive gastrointestinal disorders, such as intestinal lymphangiectasia, eosinophilic gastroenteritis, and celiac disease, as primary causes of PLE 7, 8
The American Heart Association recognizes disorders with increased central venous pressure or lymphatic obstruction, such as congenital heart disease and constrictive pericarditis, as causes of PLE 5

The American Gastroenterological Association recommends targeted treatment based on etiology, including antimicrobial therapy for infectious causes and immunosuppressive therapy for inflammatory conditions 6, 7
The American Heart Association suggests that heart transplantation may be beneficial for severe cardiac causes of PLE 5
The American Gastroenterological Association recommends repeat endoscopy with biopsies to assess response to therapy, typically after 12 months of treatment 9

The American Gastroenterological Association recommends evaluating for celiac disease with HLA testing and considering a trial of gluten-free diet for patients with seronegative enteropathy 8, 10
The American Gastroenterological Association suggests that budesonide may be beneficial for patients with persistent symptoms without identified cause 11
The American Heart Association recommends creation of atrial septal fenestration or Fontan conversion for patients with Fontan circulation and PLE 5