Encapsulating peritoneal sclerosis

The purpose of this best practice is to briefly define what has now been accepted regarding encapsulating peritoneal sclerosis (EPS), highlighting the latest developments and outlining future lines of research. The medical therapy that can be proposed (to be discussed individually, verifying the individual features of the patient) appears to include steroids, tamoxifen, and sirolimus or everolimus, with blood levels maintained at reference values for post-transplantation therapy. In view of the high incidence of relapse also in responders, it appears appropriate to continue therapy for prolonged periods, at least for 6 months. Moreover, a surgical assessment is indicated, especially for patients with intestinal symptoms including subocclusion status. To date the prevention of EPS is an unresolved issue. The recommended measures include the accurate prevention and best treatment of acute peritonitis, the use of biocompatible dialysis fluids (there is no consensus on their exact definition) and the monitoring of ultrafiltration characteristics and peritoneal membrane transport. Other recommended measures are the extensive use of renin-angiotensin-aldosterone axis inhibitors for the treatment of arterial hypertension in PD and the exclusion of beta-blockers. Other suggested strategies are tamoxifen prophylaxis in cases at risk and to adopt personalized immunosuppressive protocols for patients with PD who undergo renal transplantation.