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Encapsulating peritoneal sclerosis-a rare but devastating peritoneal disease.

Zia Moinuddin1, Angela Summers2, David Van Dellen2

  • 1Department of Transplantation, Manchester Royal Infirmary Manchester, UK ; Faculty of Medical and Human Sciences, Institute of Inflammation and Repair, University of Manchester, Manchester Academic Health Science Centre Manchester, UK.

Frontiers in Physiology
|January 21, 2015
PubMed
Summary
This summary is machine-generated.

Encapsulating peritoneal sclerosis (EPS) is a rare complication of long-term peritoneal dialysis, causing bowel obstruction. While its exact cause is uncertain, the "two-hit theory" suggests chronic injury plus a second insult like peritonitis may trigger EPS.

Keywords:
encapsulating peritoneal sclerosisepithelial–mesenchymal transitionfibrosismesotheliumperitoneal dialysis

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Area of Science:

  • Nephrology
  • Gastroenterology
  • Pathology

Background:

  • Encapsulating peritoneal sclerosis (EPS) is a rare but severe complication of long-term peritoneal dialysis (PD).
  • It involves peritoneal thickening and fibrosis, forming a cocoon that obstructs the bowel.
  • The incidence of EPS increases with PD duration.

Purpose of the Study:

  • To summarize the current understanding of Encapsulating peritoneal sclerosis (EPS) pathogenesis, clinical presentation, and management.
  • To highlight the role of peritoneal dialysis in EPS development.
  • To discuss potential therapeutic strategies and the "two-hit theory".

Main Methods:

  • Review of existing literature on Encapsulating peritoneal sclerosis.
  • Analysis of the proposed pathogenesis, including the "two-hit theory".
  • Discussion of clinical manifestations, diagnostic challenges, and treatment options.

Main Results:

  • Peritoneal dialysis fluid causes chronic peritoneal injury, inflammation, and fibrosis, contributing to EPS.
  • The "two-hit theory" posits that chronic PD injury followed by a second insult (e.g., peritonitis, transplantation) triggers EPS.
  • Epithelial-mesenchymal transition is implicated, but its role in EPS requires further clarification.

Conclusions:

  • EPS is a serious complication of PD, necessitating further research into its pathogenesis.
  • While corticosteroids and tamoxifen show promise, established treatments are lacking.
  • Nutritional support and surgical intervention are crucial for managing advanced EPS and intestinal obstruction.