Encapsulating peritoneal sclerosis in Japan: A prospective, controlled, multicenter study

Background Encapsulating peritoneal sclerosis (EPS) is recognized as a rare but serious complication of peritoneal dialysis (PD). The aim of this study was to determine the incidence, clinical features, and mortality rate of EPS. Methods: The authors requested the registration of all PD patients in facilities across Japan where more than 10 patients were treated with PD in this prospective multicenter study. During the 4-year study, the incidence of EPS was observed in the enrolled patients. Results: A total of 1,958 patients who were treated with PD in 57 facilities were followed up from April 1999 through March 2003. EPS occurred in 48 patients, corresponding to an overall incidence of 2.5%. In 33 of the 48 (68.8%) patients, EPS was found after discontinuation of PD. The incidence (and mortality rate) of EPS was 0%, 0.7% (0%),2.1% (8.3%),5.9% (28.6%),5.8% (61.5%), and 17.2% (100%) in patients who had undergone PD for 3, 5, 8, 10, 15, and more than 15 years, respectively. The recovery ratio with total parenteral nutrition, corticosteroids and surgical treatment were 0%, 38.5%, and 58.3%, respectively. Eighteen patients (37.5%) died, 22 (45.8%) recovered, and the status of the other 8 (16.7%) remained unchanged. Conclusion: The results of this prospective multicenter study showed that the incidence of EPS was 2.5% within a 4-year observation period and that two thirds of the cases were diagnosed after discontinuation of PD. Because of the current progress in diagnostic technology and therapeutic methodology, it appears that PD can be continued successfully with an acceptable, low risk for EPS for at least 8 years, whereas stricter caution is required for patients receiving PD for longer periods.