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Gastroenterology > Intestine
Protein-Losing Enteropathy
Article Last Updated: Sep 28, 2006
AUTHOR AND EDITOR INFORMATION
Section 1 of 9  
Author: Richard Wright, MD, Professor and Chief, Department of Medicine, Division of Gastroenterology/Hepatology, University of Louisville School of Medicine
Richard Wright is a member of the following medical societies: American College of Physician Executives, American College of Physicians, American Gastroenterological Association, American Medical Association, and American Society for Gastrointestinal Endoscopy
Editors: Terence David Lewis, MBBS, FRACP, FRCPC, FACP, Program Director, Internal Medicine Residency, & Assistant Chairman, Associate Professor, Department of Internal Medicine, Division of Gastroenterology, Loma Linda University Medical Center; Francisco Talavera, PharmD, PhD, Senior Pharmacy Editor, eMedicine; Noel Williams, MD, Professor Emeritus, Department of Medicine, Dalhousie University, Halifax, Nova Scotia, Canada; Professor, Department of Internal Medicine, Division of Gastroenterology, University of Alberta, Edmonton, Alberta, Canada; Alex J Mechaber, MD, FACP, Assistant Dean for Medical Curriculum, Associate Professor of Medicine, Division of General Internal Medicine, University of Miami Miller School of Medicine; Julian Katz, MD, Clinical Professor of Medicine, Drexel University College of Medicine; Consulting Staff, Department of Medicine, Section of Gastroenterology and Hepatology, Hospital of the Medical College of Pennsylvania
Author and Editor Disclosure
Synonyms and related keywords:
protein losing enteropathy, protein loss, protein deficiency, GI protein loss, gastrointestinal protein loss, protein-losing gastroenteropathy, protein losing gastroenteropathy, gastroenteropathy, gastric protein loss, Helicobacter pylori, H pylori, giant hypertrophic gastropathy, Menetrier disease, Ménétrier, disease, loss of plasma proteins from the gastrointestinal tract, excessive leakage of plasma proteins into the lumen of the gastrointestinal tract, lymphatic obstruction, mucosal disease with erosions, ulcerations, swelling of the legs, peripheral edema, decreased plasma oncotic pressure
Background
Protein-losing enteropathy represents a multiplicity of abnormalities that result in the loss of plasma proteins from the gastrointestinal tract.
Pathophysiology
The pathophysiology of this disorder is directly related to the excessive leakage of plasma proteins into the lumen of the gastrointestinal tract. The liver and reticuloendothelial system are unable to compensate for the loss. Mechanisms for gastrointestinal protein loss include lymphatic obstruction, mucosal disease with erosions, or ulcerations. Mucosal disease without erosions or ulcerations may also be associated with protein loss.
Frequency
United States
The prevalence rate is not known.
International
The prevalence rate is not known.
Mortality/Morbidity
Morbidity and mortality of this condition directly relate to its cause, either primary gastrointestinal disease or a multisystem disorder.
Race
No race predilection exists.
Sex
No sex predilection exists.
Age
No age predilection exists.
History
- The most common presenting symptom is swelling of the legs or other areas due to peripheral edema secondary to decreased plasma oncotic pressure, with subsequent transudation of fluid from the capillary bed to the subcutaneous tissue.
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- If the protein-losing gastroenteropathy is related to other systemic diseases (eg, congestive heart failure, constrictive pericarditis, connective-tissue disease, amyloidosis, protein dyscrasias), the clinical presentation may be that of the primary disease process.
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- Patients with primary gastrointestinal disease present with diarrhea with or without bleeding, abdominal pain, and/or weight loss.
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Physical
- Physical examination reveals peripheral edema and, in rare cases, anasarca.
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- Evidence of the underlying medical problem (eg, cardiac disease, amyloidosis) may exist.
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- If a primary gastrointestinal etiology exists, the abdominal examination findings may be unremarkable. Hepatosplenomegaly may be present, depending on the underlying process.
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Causes
- Primary gastrointestinal mucosal diseases (typically ulcerative/erosive) include the following:
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- Erosions or ulcerations of the esophagus, stomach, or duodenum
- Regional enteritis
- Graft versus host disease
- Pseudomembranous colitis (Clostridium difficile)
- Mucosal-based neoplasia
- Carcinoid syndrome
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- Idiopathic ulcerative jejunoileitis
- Amyloidosis
- Kaposi sarcoma
- Protein dyscrasia
- Ulcerative colitis
- Neurofibromatosis
- Cytomegalovirus infection
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- Increased interstitial pressure or lymphatic obstruction leading to protein loss can be caused by the following:
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- Tuberculosis
- Sarcoidosis
- Retroperitoneal fibrosis
- Lymphoma
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- Intestinal endometriosis
- Lymphoenteric fistula
- Whipple disease
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- Cardiac disease (constrictive pericarditis or congestive heart failure)
- Intestinal lymphangiectasia
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- Nonerosive upper gastrointestinal diseases include the following:
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- Cutaneous burns
- Whipple disease
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- Connective-tissue disorders
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- Acquired immunodeficiency syndrome (AIDS)
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- Enteropathy, such as angioedema (idiopathic or hereditary) and Henoch-Schönlein purpura
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- Celiac sprue
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- Tropical sprue
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- Allergic gastroenteritis
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- Eosinophilic gastroenteritis
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- Giant hypertrophic gastritis (Ménétrier disease)
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- Bacterial overgrowth
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- Intestinal parasites
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- Microscopic colitis
- Dientamoeba fragilis
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Cardiomyopathy, Restrictive
Collagenous and Lymphocytic Colitis
Hypoalbuminemia
Hypogammaglobulinemia
Inflammatory Bowel Disease
Malabsorption
Mycoplasma Infections
Pericarditis, Acute
Pericarditis, Constrictive
Pericarditis, Constrictive-Effusive
Salmonellosis
Yersinia Enterocolitica
Other Problems to be Considered
Cutaneous burns
Parasitic infections
Viral enteritis
Lab Studies
- The most prominent laboratory abnormality is a decrease in serum albumin and globulin.
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- The presence of alpha1-antitrypsin in the stool is an important diagnostic clue because it is not normally absorbed or secreted into the bowel.
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- In patients with hyperacidity syndromes, this study is not accurate because of the degradation of alpha1-antitrypsin in an environment where the pH is less than 3.
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- Measuring stool volume and stool alpha1-antitrypsin concentration shows plasma clearance of alpha1-antitrypsin.
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- Viral serologies may be helpful.
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Imaging Studies
- Radionuclide-labeled serum albumin can be administered intravenously, and stool can be collected as a measure of protein exudation into the gastrointestinal tract.
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- Computed tomography scanning may suggest lymphatic obstruction. This needs to be confirmed with lymphangiography.
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- Chest radiography, echocardiography, and radionuclide scanning of the heart detect cardiac disease.
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- Erosive or ulcerative conditions are diagnosed using radiographic contrast studies or, when possible, endoscopy and mucosal biopsies.
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Other Tests
- Primary gastrointestinal tract disease can be detected by endoscopy and biopsy, barium radiography, stools for ova and parasites, and culture. Primary gastrointestinal tract disease can be suggested by fecal occult blood.
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- Perform a hydrogen breath test for bacterial overgrowth in the small intestine.
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Procedures
- Findings on endoscopic studies are usually normal unless primary gastrointestinal disease (eg, ulcerative colitis, Crohn disease, Ménétrier disease, viral mucosal ulcerations) is present.
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Histologic Findings
Mucosal abnormalities can be observed with inflammatory (colitis) and infectious (viral tuberculosis) causes and in lymphatic obstruction (lymphangiectasia).
Medical Care
Focus treatment on correcting the underlying process causing the protein-losing gastroenteropathy. For example, the patient with congestive heart failure may respond to digitalis and diuretics. The patient with intestinal parasites should be treated with the appropriate medication for the infestation.
Surgical Care
- Surgery for giant hypertrophic gastropathy (Ménétrier disease) and localized lymphatic obstruction has been suggested. Surgical lymphovenous anastomosis may also be of benefit in these patients.
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- Eradicating Helicobacter pylori has also been shown to decrease gastric protein loss in some patients with giant hypertrophic gastropathy.
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Diet
- A low-fat diet with supplementation with medium-chain triglycerides is theoretically of benefit in patients with lymphangiectasias. However, in practice, ingesting a diet containing medium-chain triglycerides results in increased blood flow with no reduction in fecal protein loss.
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- Patients with celiac sprue typically respond to a gluten-free diet. A minority requires corticosteroids.
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Octreotide has limited benefit in treating patients with Ménétrier disease, but a therapeutic trial may be worthwhile. A monoclonal antibody against the epidermal growth factor receptor has been shown to be effective in treating Ménétrier disease. External elastic support is helpful in reducing peripheral edema.
Prednisone may be used in patients with total villous atrophy that is unresponsive to gluten restriction. Prednisone rapidly reverses the symptoms and signs of eosinophilic gastroenteritis and returns the serum albumin to the reference range. Medium-chain triglycerides are not helpful in hereditary intestinal lymphangiectasia. Specific treatment of infectious enteritides is indicated when present.
Drug Category: Corticosteroids
These agents have anti-inflammatory properties and cause profound and varied metabolic effects. Corticosteroids modify the body's immune response to diverse stimuli.
| Drug Name | Prednisone (Orasone, Sterapred) |
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| Description | Immunosuppressant for treatment of autoimmune disorders; may decrease inflammation by reversing increased capillary permeability and suppressing PMN activity. Stabilizes lysosomal membranes and also suppresses lymphocyte and antibody production. |
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| Adult Dose | 5-60 mg/d PO qd or divided bid/qid; taper over 2 wk, as symptoms resolve, to 5-10 mg PO qd |
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| Pediatric Dose | Not established |
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| Contraindications | Documented hypersensitivity; viral infection, peptic ulcer disease, hepatic dysfunction, connective tissue infections, and fungal or tubercular skin infections; GI bleeding or ulceration |
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| Interactions | Coadministration with estrogens may decrease clearance; concurrent use with digoxin may cause digitalis toxicity secondary to hypokalemia; phenobarbital, phenytoin, and rifampin may increase metabolism of glucocorticoids (consider increasing maintenance dose); monitor for hypokalemia with coadministration of diuretics |
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| Pregnancy | B - Usually safe but benefits must outweigh the risks.
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| Precautions | Abrupt discontinuation of glucocorticoids may cause adrenal crisis; hyperglycemia, edema, osteonecrosis, myopathy, peptic ulcer disease, hypokalemia, osteoporosis, euphoria, psychosis, myasthenia gravis, growth suppression, and infections may occur with glucocorticoid use |
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Medical/Legal Pitfalls
- Failure to diagnose and treat
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Protein-Losing Enteropathy excerpt Article Last Updated: Sep 28, 2006
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