What Is Protein Losing Enteropathy?

In protein losing enteropathy (Greek enteron = intestine; pathy = disease), proteins are lost through the gut wall into the gut hollow, resulting in low blood proteins and leg swelling. Proteins are lost through the damaged gut wall (see causes bellow) or through intact gut wall due to blocked drainage of proteins by intestinal lymphatic vessels (1).

Symptoms

Symptoms of protein losing enteropathy include:

  • Leg swelling (result of low levels of proteins in the blood)
  • Diarrhea (from the original intestinal disease)
  • Blood in the stool (if ulcers develop during intestinal disease)
  • Weight loss (from reduced absorption of nutrients in damaged small intestine)
  • Various infections (due to lowered immunity)  

Causes

Main causes of protein losing enteropathy:

  • Stomach:
    • Giant hypertrophic gastritis (Ménétrier disease);
  • Small intestine:
    • Crohn’s disease
    • Celiac disease
    • Tropical sprue
    • Connective tissue disorders like SLE
    • Neurofibromatosis
    • Angioedema
    • Henoch-Schönlein purpura
    • Allergic gastroenteritis
    • Eosinophilic gastroenteritis
    • Small intestinal bacterial overgrowth (SIBO)
    • Intestinal parasites
    • Amyloidosis
    • Idiopathic ulcerative jejunoileitis
    • Kaposi sarcoma
    • Carcinoid syndrome
    • Mucosal-based neoplasia
    • Graft versus host disease (in organ transplantation)
  • Colon:
    • Pseudomembranous colitis (Clostridium difficile)
    • Ulcerative colitis
    • Microscopic colitis
    • Infection by Dientamoeba fragilis
  • Systemic diseases:
    • Cutaneous burns
    • Cytomegalovirus (CMV) infection
    • AIDS
    • Protein dyscrasia
  • Increased interstitial pressure or lymphatic obstruction due to:
    • Tuberculosis
    • Sarcoidosis
    • Retroperitoneal fibrosis
    • Lymphoma
    • Intestinal endometriosis
    • Lymphoenteric fistula
    • Whipple disease
    • Heart disease (constrictive pericarditis or congestive heart failure)
    • Intestinal lymphangiectasia

Diagnosis

Protein losing enteropathy may be suspected when low levels of proteins (albumins and immunoglobulins) are found in the blood, and electrolyite imbalance, heart, liver and kidney disease were excluded as a possible cause of leg swelling. Tests that confirm diagnosis may include:

  • Upper endoscopy, capsule endoscopy or colonoscopy to find eventual inflammation in the gut.
  • In the stool sample, protein A1AT (alpha-1-anti-tripsin) may be found.
  • Radionuclide-labeled serum albumin can be injected into a vein, and then, if it leaks through impaired gut wall, measured in the stool.
  • CT and lymphangiography may show obstruction of lymphatic vessels.

Treatment

Treatment includes:

  • Treatment of the underlying disease
  • Replacing of lost proteins by intravenous infusion

References:

  1. Protein losing enteropathy - details  (emedicine.medscape.com)

Article reviewed by Dr. Greg. Last updated on September 19, 2009