Protein-Losing Enteropathy Clinical Presentation

  • Author: Naeem Aslam, MD; Chief Editor: Julian Katz, MD   more...
 
Updated: Nov 28, 2011
 

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.
  • 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.[4]
  • Patients with primary gastrointestinal disease present with diarrhea with or without bleeding, abdominal pain, and/or weight loss.
  • Along with a loss of proteins, a significant loss of immunoglobulins and lymphocytes can also occur. This may lead to the development of an immunological deficiency, predisposing to infections.[2]
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Physical

  • Physical examination reveals peripheral edema and, in rare cases, anasarca.
  • Evidence of the underlying medical problem (eg, cardiac disease, amyloidosis) may exist.[5, 6]
  • 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:
    • Erosions or ulcerations of the esophagus, stomach, or duodenum
    • Regional enteritis
    • Graft versus host disease
    • Pseudomembranous colitis (Clostridium difficile)[7]
    • Mucosal-based neoplasia
    • Carcinoid syndrome
    • Idiopathic ulcerative jejunoileitis
    • Amyloidosis[5]
    • Kaposi sarcoma
    • Protein dyscrasia
    • Ulcerative colitis
    • Neurofibromatosis
    • Cytomegalovirus infection[8]
  • Increased interstitial pressure or lymphatic obstruction leading to protein loss can be caused by the following:
    • Tuberculosis
    • Sarcoidosis
    • Retroperitoneal fibrosis
    • Lymphoma
    • Intestinal endometriosis
    • Lymphoenteric fistula
    • Whipple disease
    • Cardiac disease (constrictive pericarditis or congestive heart failure)
    • Intestinal lymphangiectasia[9]
  • Nonerosive upper gastrointestinal diseases include the following:
    • Cutaneous burns[10]
    • Whipple disease
    • Connective tissue disorders
    • Acquired immunodeficiency syndrome (AIDS)[2]
    • Enteropathy, such as angioedema (idiopathic or hereditary) and Henoch-Schönlein purpura
    • Celiac sprue
    • Tropical sprue
    • Allergic gastroenteritis
    • Eosinophilic gastroenteritis
    • Giant hypertrophic gastritis (Ménétrier disease)[11, 12, 13]
    • Bacterial overgrowth
    • Intestinal parasites
    • Microscopic colitis
    • Dientamoeba fragilis[14]
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Contributor Information and Disclosures
Author

Naeem Aslam, MD  Fellow, Department of Medicine, Division of Gastroenterology/Hepatology, University of Louisville School of Medicine

Naeem Aslam, MD is a member of the following medical societies: American Society for Gastrointestinal Endoscopy

Disclosure: Nothing to disclose.

Coauthor(s)

Richard Wright, MD  Professor and Chief, Department of Medicine, Division of Gastroenterology/Hepatology, University of Louisville School of Medicine

Richard Wright, MD 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

Disclosure: Nothing to disclose.

Specialty Editor Board

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

Terence David Lewis, MBBS, FRACP, FRCPC, FACP is a member of the following medical societies: American College of Gastroenterology, American College of Physicians, American Gastroenterological Association, American Medical Association, California Medical Association, Royal College of Physicians and Surgeons of Canada, and Sigma Xi

Disclosure: Nothing to disclose.

Francisco Talavera, PharmD, PhD  Adjunct Assistant Professor, University of Nebraska Medical Center College of Pharmacy; Editor-in-Chief, Medscape Drug Reference

Disclosure: Medscape Salary Employment

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

Noel Williams, MD is a member of the following medical societies: Royal College of Physicians and Surgeons of Canada

Disclosure: Nothing to disclose.

Alex J Mechaber, MD, FACP  Senior Associate Dean for Undergraduate Medical Education, Associate Professor of Medicine, University of Miami Miller School of Medicine

Alex J Mechaber, MD, FACP is a member of the following medical societies: Alpha Omega Alpha, American College of Physicians-American Society of Internal Medicine, and Society of General Internal Medicine

Disclosure: Nothing to disclose.

Chief Editor

Julian Katz, MD  Clinical Professor of Medicine, Drexel University College of Medicine

Julian Katz, MD is a member of the following medical societies: American College of Gastroenterology, American College of Physicians, American Gastroenterological Association, American Geriatrics Society, American Medical Association, American Society for Gastrointestinal Endoscopy, American Society of Law, Medicine & Ethics, American Trauma Society, Association of American Medical Colleges, and Physicians for Social Responsibility

Disclosure: Nothing to disclose.

References

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  2. Laine L, Garcia F, McGilligan K, et al. Protein-losing enteropathy and hypoalbuminemia in AIDS. AIDS. Jun 1993;7(6):837-40. [Medline].

  3. Waldmann TA, Wochner RD, Strober W. The role of the gastrointestinal tract in plasma protein metabolism. Studies with 51Cr-albumin. Am J Med. Feb 1969;46(2):275-85. [Medline].

  4. Waldmann TA. Protein-losing gastroenteropathy. Gastroenterology. 1966;50:422.

  5. Akgün A, Tani Acar E, Taner MS, et al. Scintigraphic diagnosis of protein-losing enteropathy secondary to amyloidosis. Turk J Gastroenterol. Mar 2005;16(1):41-3. [Medline].

  6. Chau TN, Mok MY, Chan EY, Luk WH, Lai KB, Li FT, et al. Evaluation of performance of measurement of faecal a(1)-antitrypsin clearance and technetium-99m human serum albumin scintigraphy in protein-losing enteropathy. Digestion. 2011;84(3):199-206. [Medline].

  7. Rybolt AH, Bennett RG, Laughon BE, et al. Protein-losing enteropathy associated with Clostridium difficile infection. Lancet. Jun 17 1989;1(8651):1353-5. [Medline].

  8. Cheong JL, Cowan FM, Modi N. Gastrointestinal manifestations of postnatal cytomegalovirus infection in infants admitted to a neonatal intensive care unit over a five year period. Arch Dis Child Fetal Neonatal Ed. Jul 2004;89(4):F367-9. [Medline].

  9. Pratz KW, Dingli D, Smyrk TC, et al. Intestinal lymphangiectasia with protein-losing enteropathy in Waldenstrom macroglobulinemia. Medicine (Baltimore). Jul 2007;86(4):210-4. [Medline].

  10. Venkatesh B, Gough J, Ralston DR, et al. Protein losing enteropathy in critically ill adult patients with burns: a preliminary report. Intensive Care Med. Jan 2004;30(1):162-6. [Medline].

  11. Citrin Y, Sterling K, Halsted JA. The mechanism of hypoproteinemia associated with giant hypertrophy of the gastric mucosa. N Engl J Med. Nov 7 1957;257(19):906-12. [Medline].

  12. Overholt BF, Jeffries GH. Hypertrophic, hypersecretory protein-losing gastropathy. Gastroenterology. Jan 1970;58(1):80-7. [Medline].

  13. Fretzayas A, Moustaki M, Alexopoulou E, Nicolaidou P. Menetrier's disease associated with Helicobacter pylori: three cases with sonographic findings and a literature review. Ann Trop Paediatr. 2011;31(2):141-7. [Medline].

  14. Ito R, Sakagami J, Kataoka K, et al. Chronic diarrhea and protein-losing gastroenteropathy caused by Dientamoeba fragilis. J Gastroenterol. Nov 2004;39(11):1117-9. [Medline].

  15. Bindl L, Buderus S, Bindl C, Lentze MJ. Protein-losing enteropathy: report of four cases and review of etiology, diagnostic work-up and treatment. Klin Padiatr. 2005;217:201-210.

  16. Florent C, L'Hirondel C, Desmazures C, et al. Intestinal clearance of alpha 1-antitrypsin. A sensitive method for the detection of protein-losing enteropathy. Gastroenterology. Oct 1981;81(4):777-80. [Medline].

  17. Albright F, Bartter FC, Forbes AP. The fate of human serum albumin administered intravenously to a patient with idiopathic hypoalbuminemia and hypoproteinemia. Trans Assoc Am Physicians. 1949;204:62.

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  19. Stark ME, Batts KP, Alexander GL. Protein-losing enteropathy with collagenous colitis. Am J Gastroenterol. Jun 1992;87(6):780-3. [Medline].

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  21. Yeaton P, Frierson HF Jr. Octreotide reduces enteral protein losses in Ménétrier's disease. Am J Gastroenterol. Jan 1993;88(1):95-8. [Medline].

  22. Gordon RS Jr. Exudative enteropathy: abnormal permeability of the gastrointestinal tract demonstrable with labelled polyvinylpyrrolidone. Lancet. Feb 14 1959;1(7068):325-6. [Medline].

  23. Samarkos M, Vaiopoulos G, Andreopoulos A, et al. Association of protein-losing enteropathy and cryoglobulinaemia. Scand J Gastroenterol. Mar 2003;38(3):334-6. [Medline].

  24. Vyas H, Driscoll DJ, Cetta F, et al. Gastrointestinal bleeding and protein-losing enteropathy after the fontan operation. Am J Cardiol. Sep 1 2006;98(5):666-7. [Medline].

 
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