Encapsulating peritoneal sclerosis: what have we learned?

Abstract

Encapsulating peritoneal sclerosis (EPS) is a rare complication of peritoneal dialysis (PD), but carries significant morbidity and mortality. We review the clinical features and radiologic and histologic changes found at diagnosis of EPS. Although EPS is strongly associated with the duration of PD, the pathogenesis remains only partly understood. We discuss the mechanisms thought to underlie the abnormally thickened, sclerotic peritoneal membrane seen in long-term PD patients including epithelial to mesenchymal transition and the molecular mediators of fibrosis and angiogenesis. We review how exposure to high-glucose, nonphysiological dialysis fluids, peritonitis, and uremia may be responsible for these changes. Much remains to be learned about optimal management of EPS, both medical and surgical, because the literature lacks controlled studies. Future research challenges include defining the role of surgery, immunosuppression, and antifibrotic agents in the management of EPS. We also need to understand why some patients progress from asymptomatic peritoneal sclerosis to the extreme levels of fibrin deposition and bowel encapsulation seen in EPS. Screening PD patients for potential future EPS remains difficult, and we need strategies for monitoring patients on longer-term PD that enable us to better quantify the risk of EPS for the individual patient.