Encapsulating peritoneal sclerosis

Abstract

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 (. 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.

Proteomic Research in Peritoneal Dialysis

“…Encapsulating peritoneal sclerosis (EPS) is a rare but potentially devastating disorder of PD. It is characterized by an acquired inflammatory fibrocollagenous membrane encasing the small intestine, which results in symptoms of bowel obstruction [89]. The prevalence of EPS in PD is between 0.4% and 8.9%, and the most significant risk factor for development of it is the duration of PD treatment [90].…”

“…The "first" hit or predisposing condition is non-inflammatory peritoneal sclerosis resulting from repeated exposure to PD solution. A pro-inflammatory "second hit" precipitates a cascade of proinflammatory and proangiogenic cytokines [89]. Genetic variation in the receptor for AGEs might predispose to peritoneal deterioration [92].…”

Case report of a rare cause of intestinal obstruction: Abdominal cocoon syndrome

“…ACS is a rare peritoneal disease with unclear pathogenesis. AKS can be divided into two, as idiopathic and secondary [7]. It has been reported that the idiopathic form may be associated with congenital dysplasia [8].…”

Man with abdominal pain

“…Fatal outcome occurs in more than 50% of the cases because of malnutrition or sepsis. 3 The incidence ranges from 0.7% to 2.5% and the occurrence increases with time.…”