Encapsulating Peritoneal Sclerosis: Case report and Current Status

Encapsulating peritoneal sclerosis (EPS), is a rare but devastating complication of long-term peritoneal dialysis (PD) with a high mortality rate. The incidence is between 0.5 and 3.3%, decreasing with time. EPS is defi ned as a clinical syndrome with major signs of gastrointestinal obstruction, infl ammatory parameters, radiological and macroscopic changes. Duration of treatment and cessation of PD are the main risk for development of EPS: about 75% of EPS occurred in patients transferred on hemodialysis or in the two years after kidney transplantation. Morphological alterations are disappearance of mesothelial layer, sub-mesothelial fi brosis, interstitial sclerosis and vasculopathy, ultrafi ltration failure, fast transport status of the peritoneal membrane, and loss of sodium sieving are the most predicting functional abnormalities. Some biomarkers could be found in the peritoneal effl uent. The pathophysiology is probably a consequence of a multiple-hit process in which expression of growth factors and cytokines play a role. Medical strategies (corticosteroids, immunosuppressive drugs, tamoxifen) in association with parenteral nutrition and/or surgery (enterolysis) are discussed. Prevention is the use of physiological peritoneal solutions, icodextrine instead of high glucose concentration solution, and peritoneal lavage fter peritoneal dialysis cessation for any reason.