Skip to main content

Abstract

Protein-losing enteropathy (PLE) refers to a pathologic loss of protein from the gastrointestinal tract. PLE may result from a variety of both intra- and extraintestinal diseases. Patients present with symptomatic hypoalbuminemia leading to peripheral edema, pleural and pericardial effusions, and malnutrition sometimes in the context of symptoms of abdominal pain and diarrhea. PLE should be suspected in patients with low serum albumin after ruling out acute illness, dilutional hypoalbuminemia, renal losses, and synthetic dysfunction. Calculation of fecal alpha-1-antitrypsin clearance is the test of choice for diagnosis. While many treatments have been reported, the cornerstone of therapy centers on treating the underlying cause of the PLE.

This is a preview of subscription content, log in via an institution to check access.

Access this chapter

Chapter
USD 29.95
Price excludes VAT (USA)
  • Available as PDF
  • Read on any device
  • Instant download
  • Own it forever
eBook
USD 84.99
Price excludes VAT (USA)
  • Available as EPUB and PDF
  • Read on any device
  • Instant download
  • Own it forever
Softcover Book
USD 109.99
Price excludes VAT (USA)
  • Compact, lightweight edition
  • Dispatched in 3 to 5 business days
  • Free shipping worldwide - see info

Tax calculation will be finalised at checkout

Purchases are for personal use only

Institutional subscriptions

References

  1. Atz AM, Zak V, Mahony L, Uzark K, D’Agincourt N, Goldberg DJ, et al. Longitudinal outcomes of patients with single ventricle after the Fontan procedure. J Am Coll Cardiol. 2017;69(22):2735–44. https://doi.org/10.1016/j.jacc.2017.03.582.

    Article  PubMed  PubMed Central  Google Scholar 

  2. Wilson TG, Shi WY, Iyengar AJ, Winlaw DS, Cordina RL, Wheaton GR, et al. Twenty-five year outcomes of the lateral tunnel Fontan procedure. Semin Thorac Cardiovasc Surg. 2017;29(3):347–53. https://doi.org/10.1053/j.semtcvs.2017.06.002.

    Article  PubMed  Google Scholar 

  3. Perednia DA, Curosh NA. Lupus-associated protein-losing enteropathy. Arch Intern Med. 1990;150(9):1806–10.

    Article  CAS  Google Scholar 

  4. Zheng WJ, Tian XP, Li L, Jing HL, Li F, Zeng XF, et al. Protein-losing enteropathy in systemic lupus erythematosus: analysis of the clinical features of fifteen patients. J Clin Rheumatol. 2007;13(6):313–6. https://doi.org/10.1097/RHU.0b013e31815bf9c6.

    Article  PubMed  Google Scholar 

  5. Rich A, Toro TZ, Tanksley J, Fiske WH, Lind CD, Ayers GD, et al. Distinguishing Menetrier’s disease from its mimics. Gut. 2010;59(12):1617–24. https://doi.org/10.1136/gut.2010.220061.

    Article  PubMed  PubMed Central  Google Scholar 

  6. Schmidt PN, Blirup-Jensen S, Svendsen PJ, Wandall JH. Characterization and quantification of plasma proteins excreted in faeces from healthy humans. Scand J Clin Lab Invest. 1995;55(1):35–45.

    Article  CAS  Google Scholar 

  7. Umar SB, DiBaise JK. Protein-losing enteropathy: case illustrations and clinical review. Am J Gastroenterol. 2010;105(1):43–9; quiz 50. https://doi.org/10.1038/ajg.2009.561.

    Article  PubMed  Google Scholar 

  8. Levitt DG, Levitt MD. Protein losing enteropathy: comprehensive review of the mechanistic association with clinical and subclinical disease states. Clin Exp Gastroenterol. 2017;10:147–68. https://doi.org/10.2147/CEG.S136803.

    Article  PubMed  PubMed Central  Google Scholar 

  9. Sun X, Shen W, Chen X, Wen T, Duan Y, Wang R. Primary intestinal lymphangiectasia: multiple detector computed tomography findings after direct lymphangiography. J Med Imaging Radiat Oncol. 2017;61(5):607–13. https://doi.org/10.1111/1754-9485.12606.

    Article  PubMed  Google Scholar 

  10. Menon S, Chennapragada M, Ugaki S, Sholler GF, Ayer J, Winlaw DS. The lymphatic circulation in adaptations to the Fontan circulation. Pediatr Cardiol. 2017;38(5):886–92. https://doi.org/10.1007/s00246-017-1576-y.

    Article  PubMed  Google Scholar 

  11. Ebert EC, Hagspiel KD. Gastrointestinal and hepatic manifestations of systemic lupus erythematosus. J Clin Gastroenterol. 2011;45(5):436–41. https://doi.org/10.1097/MCG.0b013e31820f81b8.

    Article  CAS  PubMed  Google Scholar 

  12. Ferrante M, Penninckx F, De Hertogh G, Geboes K, D’Hoore A, Noman M, et al. Protein-losing enteropathy in Crohn’s disease. Acta Gastro-Enterol Belg. 2006;69(4):384–9.

    Google Scholar 

  13. Vignes S, Bellanger J. Primary intestinal lymphangiectasia (Waldmann’s disease). Orphanet J Rare Dis. 2008;3:5. https://doi.org/10.1186/1750-1172-3-5.

    Article  PubMed  PubMed Central  Google Scholar 

  14. Desai AP, Guvenc BH, Carachi R. Evidence for medium chain triglycerides in the treatment of primary intestinal lymphangiectasia. Eur J Pediatr Surg. 2009;19(4):241–5. https://doi.org/10.1055/s-0029-1216389.

    Article  CAS  PubMed  Google Scholar 

  15. Kim NR, Lee SK, Suh YL. Primary intestinal lymphangiectasia successfully treated by segmental resections of small bowel. J Pediatr Surg. 2009;44(10):e13–7. https://doi.org/10.1016/j.jpedsurg.2009.06.034.

    Article  PubMed  Google Scholar 

  16. Itkin M, Piccoli DA, Nadolski G, Rychik J, DeWitt A, Pinto E, et al. Protein-losing enteropathy in patients with congenital heart disease. J Am Coll Cardiol. 2017;69(24):2929–37. https://doi.org/10.1016/j.jacc.2017.04.023.

    Article  CAS  PubMed  Google Scholar 

  17. Bayerdorffer E, Ritter MM, Hatz R, Brooks W, Ruckdeschel G, Stolte M. Healing of protein losing hypertrophic gastropathy by eradication of Helicobacter pylori--is Helicobacter pylori a pathogenic factor in Menetrier’s disease? Gut. 1994;35(5):701–4.

    Article  CAS  Google Scholar 

Essential Reading

  1. Levitt DG, Levitt MD. Protein losing enteropathy: comprehensive review of the mechanistic association with clinical and subclinical disease states. Clin Exp Gastroenterol. 2017;10:147–68. https://doi.org/10.2147/CEG.S136803. In-depth review of the pathophysiologic mechanism and diagnosis of PLE.

    Article  PubMed  PubMed Central  Google Scholar 

  2. Umar SB, DiBaise JK. Protein-losing enteropathy: case illustrations and clinical review. Am J Gastroenterol. 2010;105(1):43–9; quiz 50. https://doi.org/10.1038/ajg.2009.561. Additional case illustrations of causes of PLE with accompanying high yield clinical features.

    Article  Google Scholar 

Download references

Author information

Authors and Affiliations

Authors

Corresponding author

Correspondence to Andrew P. Copland .

Editor information

Editors and Affiliations

Self-Test

Self-Test

  • Question 1: PLE is most commonly described in the literature in what patient population?

    1. A.

      Post-Fontan procedure for congenital heart disease

    2. B.

      Inflammatory bowel disease

    3. C.

      Systemic rheumatologic disorders (e.g., lupus)

    4. D.

      Menetrier’s disease

  • Question 2: A patient is referred for evaluation of symptomatic hypoalbuminemia (albumin 1.9 g/dL) and has an exam notable for peripheral edema and small pleural effusions. They have a history of Crohn’s disease and experience ongoing nonbloody diarrhea. You suspect a diagnosis of protein-losing enteropathy. Which of the following would best confirm this diagnosis?

    1. A.

      A spot stool A1AT of 25 mg/dL

    2. B.

      Stool A1AT clearance of 30 mL/24-hr

    3. C.

      Stool A1AT clearance of 80 mL/24-hr

    4. D.

      Echocardiogram to rule out heart failure and urinalysis to rule out proteinuria.

  • Question 3: Which medical therapy would most benefit the patient with Crohn’s disease described in the previous question after confirmation of PLE?

    1. A.

      Oral furosemide daily

    2. B.

      Initiation of a biologic therapy

    3. C.

      Daily intravenous albumin infusion

    4. D.

      Low-protein diet

Rights and permissions

Reprints and permissions

Copyright information

© 2019 Springer Nature Switzerland AG

About this chapter

Check for updates. Verify currency and authenticity via CrossMark

Cite this chapter

Corning, B., Copland, A.P. (2019). Protein-Losing Enteropathy. In: Lacy, B., DiBaise, J., Pimentel, M., Ford, A. (eds) Essential Medical Disorders of the Stomach and Small Intestine. Springer, Cham. https://doi.org/10.1007/978-3-030-01117-8_19

Download citation

  • DOI: https://doi.org/10.1007/978-3-030-01117-8_19

  • Published:

  • Publisher Name: Springer, Cham

  • Print ISBN: 978-3-030-01116-1

  • Online ISBN: 978-3-030-01117-8

  • eBook Packages: MedicineMedicine (R0)

Publish with us

Policies and ethics