Encapsulating peritoneal sclerosis in peritoneal dialysis. a review and European initiative for approaching a serious and rare disease.

Abstract

Encapsulating peritoneal sclerosis (EPS) represents a rare complication in peritoneal dialysis (PD) with high mortality. It is characterised by diffuse peritoneal membrane fibrosis, which develops into encapsulation and manifests as clinical signs and symptoms of intestinal obstruction. Its incidence varies from 0.7%to 3.3%. The most significant risk factor in its development is exposure time to PD solutions, although young age and peritonitis episodes can also contribute. Its aetiopathogeny has not been clearly explained and it is thought that a second hit like peritonitis, hemoperitoneum, surgery, genetic predisposition, etc on an already damaged peritoneal membrane, could also trigger the development of EPS. Some cases appear after transfer to haemodialysis or after transplant. In these cases, the use of calcineurin inhibitors is believed to be related. The presence of clinical symptoms and signs of intestinal obstruction, along with compatible radiological and/or anatomical findings could also confirm the diagnosis. At present there are no clinical or biochemical markers capable of predicting its onset. Therapeutic management comprises the use of immunosuppressors like steroids and tamoxifen, nutritional management and even surgery in advanced cases, all of which provide varying results. This article discusses the diagnosis and treatment of EPS, it encourages the participation in the European Registry and it advocates the need to centralise the management of this medical complication.