Incidence and outcomes of encapsulating peritoneal sclerosis (EPS) and factors associated with severe EPS

January Incidence and outcomes of encapsulating peritoneal sclerosis (EPS) and factors associated with severe EPS Chin-Chung Tseng 1 2 Jin-Bor Chen 0 1 I-Kuan Wang 1 Shang-Chih Liao 0 1 Ben-Chung Cheng 0 1 An-bang Wu 1 2 Yu-Tzu Chang 1 2 Shih-Yuan Hung 1 Chiu-Ching Huang 1 0 Division of Nephrology, Department of Internal Medicine, Kaohsiung Chang Gung Memorial Hospital and College of Medicine, Chang Gung University , Kaohsiung, Taiwan , 4 Kidney Institute and Division of Nephrology, China Medical University Hospital and College of Medicine, China Medical University , Taichung, Taiwan , 5 Division of Nephrology, Department of Internal Medicine, E-DA Hospital, and School of Medicine for International Students, I-Shou University , Kaohsiung , Taiwan 1 Editor: Zhanjun Jia, University of Utah School of Medicine , UNITED STATES 2 Division of Nephrology, Department of Internal Medicine, National Cheng Kung University Hospital , Tainan , Taiwan , 2 Department of Medicine, College of Medicine, National Cheng Kung University , Tainan , Taiwan - OPEN ACCESS Data Availability Statement: All relevant data are within the paper and its Supporting Information files. Funding: This work was supported by Academia Sinica, Taiwan (Grant Number BM104010113), New Century Medical Research Foundation, and Grant NCKUH-10509002 from National Cheng Kung University Hospital, Tainan. The funders had no role in study design, data collection and analysis, decision to publish, or preparation of the manuscript. Background Methods groups. Results Fifty-eight of 3202 patients undergoing PD during the study period had EPS (prevalence 1.8%). The incidence of EPS increased for patients on PD for >6±8 years ( 6 yrs. vs. >6±8 yrs., 0.0% vs. 1.8%, p = 0.001). Relative to those on PD for >6±8 years, the risk of EPS significantly increased with PD duration longer than 10 years (>10±12 years vs. >6±8 years: OR: 5.5, 95% CI: 1.7±17.1, p < 0.01). Twenty-three patients fulfilled the criteria for severe EPS. The overall mortality rate of EPS was 35% (20/58), and was 74% (17/23) in the severe EPS group. The average serum levels of C-reactive protein (CRP) and intact-parathyroid Competing interests: The authors have declared that no competing interests exist. hormone (i-PTH), which were checked every 3~6 months within one year before diagnosis of EPS, were higher in severe EPS group than in mild/moderate group (p = 0.02, p = 0.08, respectively). Multivariate analysis revealed severe EPS was independently associated with bowel tethering (based on CT), presentation with bloody ascites, diagnosis of EPS after withdrawal from PD, and i-PTH 384 pg/mL. Receiver operating characteristic analysis indicated that presentation with 2 or more of the 5 risk factors (EPS diagnosis after PD withdrawal, bloody ascites, bowel tethering, CRP 29 mg/L, and i-PTH 384 pg/mL) had a good accuracy (AUC = 0.80, p = 0.001) for prediction of severe EPS. Conclusions The incidence of EPS increases with PD duration. Severe EPS has high mortality rate and is associated with bowel tethering, presentation of bloody ascites, diagnosis after PD withdrawal, and higher serum levels of i-PTH before EPS diagnosis. Having 2 or more of the 5 risk factors can provide a good accuracy for prediction of severe EPS. Introduction Gandhi and colleagues first reported the presence of encapsulating peritoneal sclerosis (EPS) in 1980 [ 1 ]. Since then, EPS has been recognized as a rare but serious complication of longterm peritoneal dialysis (PD). EPS is characterized by microangiopathy, inflammation, and extreme peritoneal sclerosis, and leads to intestinal encapsulation and obstruction [ 2 ]. Data from EPS registries or study groups indicate that the prevalence of EPS is 0.5±2.5% among PD patients, and that the incidence increases with the duration of PD therapy [3±5]. An Australian registry study reported that the incidence of EPS was 19.4% after 8 years of PD treatment [ 3 ]. Kawanishi et al. performed a prospective study of PD patients in Japan, and reported the incidence of EPS was 0.7% after 5 years, 2.1% after 8 years, 5.9% after 10 years, and 17.2% after 15 years [ 5 ]. The effect of medical therapy for EPS is still unclear, but there are several non-evidencebased medical and surgical treatment options. Although there are currently no specific, evidence-based medical treatment options [ 6 ], the possible treatment regimens include immunosuppression or antifibrotic therapy with steroids, cyclosporine, and tamoxifen. Peritonectomy and enterolysis (PEEL) is the surgery of choice [ 7, 8 ]. Although EPS is the most serious complication of PD, previous studies reported large variations in the mortality rates ranging from 19% to 67% [3±5, 9±14], possibly because they studied patients with different severities. However, the factors associated with severe EPS are not yet well established. In addition, variations in the use of PD, withdrawal rate, and treatment duration among di (...truncated)