New facts about encapsulating peritoneal sclerosis as a sequel of long-term peritoneal dialysis - what can we do?

Abstract

A long lasting peritoneal dialysis (PD) leads to a special disease, so-called encapsulating peritoneal sclerosis (EPS). The hallmarks of the latter stages of the disease are intestinal obstructions and, as a consequence, malnourishment. For the precise diagnosis radiology and pathology are essential. (Triad ''typical clinical picture- typical radiology- typical pathology''.) In the middle of the pathological process of EPS is proliferative fibrosis and sclerosis of the peritoneum that subsequently leads to the assembly of the typical ''cocoon'' and obstruction. In EPS we found in the peritoneum increased amounts of vascular endothelial growth factor (VEGF) fitting the hallmark of increased neoangiogenesis and blood exudates with fibrinous matrix on the peritoneum as a feeding ground for proliferation of fibroblasts. Additionally, the number of mast cells in EPS is decreased and therefore the chymase and other fibrinolytic enzymes. The ''plasma-leak'' hypothesis focuses on fibrin and our findings help to explain most of the pathophysiology. Since the mortality of EPS is still high, emphasis should be laid on preventive treatment. Since glucose and advanced glycation endproducts (AGEs), including glucose degradation products (GDPs), are responsible for fibrosis and sclerosis of the peritoneum, biocompatible peritoneal dialysis solutions with reduced amounts of AGEs and GDPs are recommended. Additionally, a careful monitoring of patients, especially after 5-8 years of PD is very important. In case of the first signs of EPS, cessation of the modality is necessary. Thanks to this approach, most end-stage EPS pictures can be avoided.