Encapsulating peritoneal sclerosis (EPS) is the most severe complication of peritoneal dialysis (PD) and is associated with a high morbidity and mortality. The diagnosis needs to be considered in patients with a history of PD who are currently having or have experienced symptoms of recurrent partial or complete intestinal obstruction. Most cases have been described in PD patients with a long PD duration. In addition, EPS has also been documented as a complication in former PD patients relatively shortly after kidney transplantation. This has been coined post-transplantation EPS.
Early presenting symptoms of EPS are anorexia, nausea, vomiting, abdominal pain, and weight loss. In advanced stages, bowel obstruction occurs leading to malnutrition and further weight loss. To confirm the diagnosis, abdominal imaging (preferably CT scanning) and/or macroscopical inspection is necessary (figure 1). It should be realized that EPS may evolve in an insidious manner with slow progression of abdominal symptoms and radiological abnormalities of the peritoneum to a variable degree.
Figure 1. Characteristic CT findings in an EPS patient: dilated bowel loops that are drawn into the center of the abdominal cavity indicating adhesions, and an enhanced, thickened peritoneum with calcifications (1A). At laparotomy, there is a clear fibrotic and thickened peritoneal membrane covering the bowel with extensive adhesions (1B).
The EPS-Registry is an international collaboration of renal centers in Europe aiming at collaborating on a global basis. The main aim of the registry is to improve the knowledge on EPS by gaining insight into its epidemiology, pathophysiology, early diagnosis, and optimal treatment. With this knowledge, uniform diagnostic criteria and management strategies can be developed. Furthermore, the registry is of significant importance for research projects focusing on EPS.
The EPS-Registry is designed to register and follow patients with a possible and definite diagnosis of EPS. Treating physicians are encouraged to report a patient even if there is only a slight suspicion of EPS. It is also possible to ask advice regarding diagnosis and treatment by the Q&A section. It is intended also to store frozen samples of serum, plasma, blood (DNA) and dialysate of the patients being included in the registry. The information gathered by the registry is reviewed by the steering committee of the registry on a regular basis. Furthermore, on the website information on EPS can be shared and a list of publications on EPS is made available.