Copyright ©The Author(s) 2018. Published by Baishideng Publishing Group Inc. All rights reserved.
World J Gastroenterol. Jul 28, 2018; 24(28): 3101-3111
Published online Jul 28, 2018. doi: 10.3748/wjg.v24.i28.3101
Encapsulating peritoneal sclerosis
Christopher J Danford, Steven C Lin, Martin P Smith, Jacqueline L Wolf
Christopher J Danford, Steven C Lin, Jacqueline L Wolf, Division of Gastroenterology and Hepatology, Beth Israel Deaconess Medical Center, Harvard Medical School, Boston, MA 02115, United States
Martin P Smith, Department of Radiology, Beth Israel Deaconess Medical Center, Harvard Medical School, Boston, MA 02115, United States
Author contributions: Danford CJ and Lin SC contributed equally in performing the literature search, review, and drafting of the manuscript, and they should be as co-first authors; Danford CJ, Lin SC, Smith MP and Wolf JL participated in the critical review, revision of the manuscript, and approve of the final draft of the manuscript.
Conflict-of-interest statement: There are no financial disclosures or conflicts of interest in the production of this manuscript.
Open-Access: This article is an open-access article which was selected by an in-house editor and fully peer-reviewed by external reviewers. It is distributed in accordance with the Creative Commons Attribution Non Commercial (CC BY-NC 4.0) license, which permits others to distribute, remix, adapt, build upon this work non-commercially, and license their derivative works on different terms, provided the original work is properly cited and the use is non-commercial. See:
Correspondence to: Jacqueline L Wolf, MD, Associate Professor, Division of Gastroenterology and Hepatology, Beth Israel Deaconess Medical Center, 330 Brookline Ave, Rabb 433, Boston, MA 02115, United States.
Telephone: +1-617-6671676 Fax: +1-617-6675826
Received: May 16, 2018
Peer-review started: May 17, 2018
First decision: May 30, 2018
Revised: June 7, 2018
Accepted: June 25, 2018
Article in press: June 25, 2018
Published online: July 28, 2018

Encapsulating peritoneal sclerosis (EPS) is a debilitating condition characterized by a fibrocollagenous membrane encasing the small intestine, resulting in recurrent small bowel obstructions. EPS is most commonly associated with long-term peritoneal dialysis, though medications, peritoneal infection, and systemic inflammatory disorders have been implicated. Many cases remain idiopathic. Diagnosis is often delayed given the rarity of the disorder combined with non-specific symptoms and laboratory findings. Although cross-sectional imaging with computed tomography of the abdomen can be suggestive of the disorder, many patients undergo exploratory laparotomy for diagnosis. Mortality approaches 50% one year after diagnosis. Treatment for EPS involves treating the underlying condition or eliminating possible inciting agents (i.e. peritoneal dialysis, medications, infections) and nutritional support, frequently with total parenteral nutrition. EPS-specific treatment depends on the disease stage. In the inflammatory stage, corticosteroids are the treatment of choice, while in the fibrotic stage, tamoxifen may be beneficial. In practice, distinguishing between stages may be difficult and both may be used. Surgical intervention, consisting of peritonectomy and enterolysis, is time-consuming and high-risk and is reserved for situations in which conservative medical therapy fails in institutions with surgical expertise in this area. Herein we review the available literature of the etiology, pathogenesis, diagnosis, and treatment of this rare, but potentially devastating disease.

Keywords: Abdominal cocoon, Sclerosing encapsulating peritonitis, Peritoneal sclerosis, Peritonectomy, Enterolysis, Peritoneal dialysis, Tamoxifen, Corticosteroids

Core tip: Encapsulating peritoneal sclerosis (EPS) is a rare, but potentially devastating disorder. Most literature is derived from the nephrology literature surrounding peritoneal dialysis, however, the gastroenterologist is likely to encounter EPS from a variety of etiologies. We present a comprehensive review of EPS from all etiologies and a summary of treatments from a gastroenterologist’s perspective including the role of nutrition, surgery, immunosuppression, anti-fibrotic agents, and the novel use of μ-opioid antagonists and guanylate cyclase C agonists in the management of patients with EPS.