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Definition: Encapsulating peritoneal sclerosis (EPS) is a rare but serious condition. It mainly affects people who have been treated with long-term peritoneal dialysis (PD), which is a therapy for patients with end-stage kidney disease. In EPS, the lining around the bowels also known as the peritoneal membrane, becomes thickened and hard. This lining tightly wraps around the intestines like a cocoon, which can lead to significant pain and severe problems with digestion. Common symptoms include abdominal pain, nausea, lack of appetite, weight loss and bowel obstruction. The exact cause of EPS is unknown. It is believed to be due to a “two-hit hypothesis” that involves multiple injuries to the peritoneal membrane over a long period of time. The main risk factor is believed to be a duration of peritoneal dialysis greater than 5 years, but the condition have occurred also in people who never had PD. The primary treatment options for EPS include medication and nutritional support. The two most common medications used are corticosteroids and tamoxifen. Surgery can also be performed, particularly in severe cases by surgeons with high levels of expertise.
See references:
Danford CJ, Lin SC, Smith MP, Wolf JL. Encapsulating peritoneal sclerosis. World J Gastroenterol. Jul 28 2018;24(28):3101-3111. doi:10.3748/wjg.v24.i28.3101
Korte MR, Sampimon DE, Betjes MG, Krediet RT. Encapsulating peritoneal sclerosis: the state of affairs. Nat Rev Nephrol. Aug 2 2011;7(9):528-38. doi:10.1038/nrneph.2011.93
Moinuddin Z, Summers A, Van Dellen D, Augustine T, Herrick SE. Encapsulating peritoneal sclerosis-a rare but devastating peritoneal disease. Front Physiol. 2014;5:470. doi:10.3389/fphys.2014.00470
Jagirdar RM, Bozikas A, Zarogiannis SG, Bartosova M, Schmitt CP, Liakopoulos V. Encapsulating peritoneal sclerosis: pathophysiology and current treatment options. Int J Mol Sci. Nov 16 2019;20(22)doi:10.3390/ijms20225765
Li D, Li Y, Zeng H, Wu Y. Risk factors for encapsulating peritoneal sclerosis in patients undergoing peritoneal dialysis: A meta-analysis. PLoS One. 2022;17(3):e0265584. doi:10.1371/journal.pone.0265584
Brown EA, Bargman J, van Biesen W, et al. Length of time on peritoneal dialysis and encapsulating peritoneal sclerosis - Position Paper for ISPD: 2017 Update. Perit Dial Int. Jul-Aug 2017;37(4):362-374. doi:10.3747/pdi.2017.00018
Machado NO. Sclerosing encapsulating peritonitis: Review. Sultan Qaboos Univ Med J. May 2016;16(2):e142-51. doi:10.18295/squmj.2016.16.02.003
Tseng CC, Chen JB, Wang IK, et al. Incidence and outcomes of encapsulating peritoneal sclerosis (EPS) and factors associated with severe EPS. PLoS One. 2018;13(1):e0190079. doi:10.1371/journal.pone.0190079
Korte MR, Habib SM, Lingsma H, Weimar W, Betjes MG. Posttransplantation encapsulating peritoneal sclerosis contributes significantly to mortality after kidney transplantation. Am J Transplant. Mar 2011;11(3):599-605. doi:10.1111/j.1600-6143.2010.03434.x
Burkart J & Bansal S. Encapsulating peritoneal sclerosis in patients on peritoneal dialysis. Nov 28, 2023. In: UpToDate, Connor RF (Ed), Wolters Kluwer. https://www.uptodate.com/contents/encapsulating-peritoneal-sclerosis-in-patients-on-peritoneal-dialysis#H2208450938. Accessed on January 6, 2025
Norman W R, Ricardo Ramirez S, Seale J R (December 31, 2024) Non-Dialysis-Associated Encapsulating Peritoneal Sclerosis: A Unique Case Presentation and Surgical Intervention. Cureus 16(12): e76701. doi:10.7759/cureus.76701
Label
encapsulating peritoneal sclerosis
Synonyms
abdominal cocoon syndrome, sclerosing encapsulating peritonitis, peritonitis chronica fibrosa incapsulata
Synonym type
exact
Definition
Definition: Encapsulating peritoneal sclerosis (EPS) is a rare but serious condition. It mainly affects people who have been treated with long-term peritoneal dialysis (PD), which is a therapy for patients with end-stage kidney disease. In EPS, the lining around the bowels also known as the peritoneal membrane, becomes thickened and hard. This lining tightly wraps around the intestines like a cocoon, which can lead to significant pain and severe problems with digestion. Common symptoms include abdominal pain, nausea, lack of appetite, weight loss and bowel obstruction. The exact cause of EPS is unknown. It is believed to be due to a “two-hit hypothesis” that involves multiple injuries to the peritoneal membrane over a long period of time. The main risk factor is believed to be a duration of peritoneal dialysis greater than 5 years, but the condition have occurred also in people who never had PD. The primary treatment options for EPS include medication and nutritional support. The two most common medications used are corticosteroids and tamoxifen. Surgery can also be performed, particularly in severe cases by surgeons with high levels of expertise.
See references:
Danford CJ, Lin SC, Smith MP, Wolf JL. Encapsulating peritoneal sclerosis. World J Gastroenterol. Jul 28 2018;24(28):3101-3111. doi:10.3748/wjg.v24.i28.3101
Korte MR, Sampimon DE, Betjes MG, Krediet RT. Encapsulating peritoneal sclerosis: the state of affairs. Nat Rev Nephrol. Aug 2 2011;7(9):528-38. doi:10.1038/nrneph.2011.93
Moinuddin Z, Summers A, Van Dellen D, Augustine T, Herrick SE. Encapsulating peritoneal sclerosis-a rare but devastating peritoneal disease. Front Physiol. 2014;5:470. doi:10.3389/fphys.2014.00470
Jagirdar RM, Bozikas A, Zarogiannis SG, Bartosova M, Schmitt CP, Liakopoulos V. Encapsulating peritoneal sclerosis: pathophysiology and current treatment options. Int J Mol Sci. Nov 16 2019;20(22)doi:10.3390/ijms20225765
Li D, Li Y, Zeng H, Wu Y. Risk factors for encapsulating peritoneal sclerosis in patients undergoing peritoneal dialysis: A meta-analysis. PLoS One. 2022;17(3):e0265584. doi:10.1371/journal.pone.0265584
Brown EA, Bargman J, van Biesen W, et al. Length of time on peritoneal dialysis and encapsulating peritoneal sclerosis - Position Paper for ISPD: 2017 Update. Perit Dial Int. Jul-Aug 2017;37(4):362-374. doi:10.3747/pdi.2017.00018
Machado NO. Sclerosing encapsulating peritonitis: Review. Sultan Qaboos Univ Med J. May 2016;16(2):e142-51. doi:10.18295/squmj.2016.16.02.003
Tseng CC, Chen JB, Wang IK, et al. Incidence and outcomes of encapsulating peritoneal sclerosis (EPS) and factors associated with severe EPS. PLoS One. 2018;13(1):e0190079. doi:10.1371/journal.pone.0190079
Korte MR, Habib SM, Lingsma H, Weimar W, Betjes MG. Posttransplantation encapsulating peritoneal sclerosis contributes significantly to mortality after kidney transplantation. Am J Transplant. Mar 2011;11(3):599-605. doi:10.1111/j.1600-6143.2010.03434.x
Burkart J & Bansal S. Encapsulating peritoneal sclerosis in patients on peritoneal dialysis. Nov 28, 2023. In: UpToDate, Connor RF (Ed), Wolters Kluwer. https://www.uptodate.com/contents/encapsulating-peritoneal-sclerosis-in-patients-on-peritoneal-dialysis#H2208450938. Accessed on January 6, 2025
Norman W R, Ricardo Ramirez S, Seale J R (December 31, 2024) Non-Dialysis-Associated Encapsulating Peritoneal Sclerosis: A Unique Case Presentation and Surgical Intervention. Cureus 16(12): e76701. doi:10.7759/cureus.76701
Parent term
Peritonitis
Children term(s)
No response
ORCID Identifier
No response
Website URL
No response
Additional comments
Note that you have a SNOMED term known as Encapsulating peritoneal sclerosis associated with peritoneal dialysis (disorder) SNOMED:440663004 but the condition can also occur without peritoneal dialysis. https://www.cureus.com/articles/316860-non-dialysis-associated-encapsulating-peritoneal-sclerosis-a-unique-case-presentation-and-surgical-intervention#!/ and also the UpToDate article liste above.
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