Encapsulating peritoneal sclerosis in an Italian center: thirty year experience

J Nephrol. 2016 Apr;29(2):259-267. doi: 10.1007/s40620-015-0241-x. Epub 2015 Oct 31.

Abstract

Background: Encapsulating peritoneal sclerosis (EPS) is a rare but life-threatening complication of peritoneal dialysis (PD). Its incidence and prevalence are still not clearly defined. No data exist on the prevalence of EPS in Italy.

Objectives: To evaluate the incidence and prevalence of EPS, and identify potential factors useful for prevention or early diagnosis of EPS.

Methods: A retrospective study in patients starting PD between 1979 and 2013 in one Italian center. Data on demographics, occurrence of EPS, time on PD, peritoneal equilibration test, and therapy for EPS were gathered.

Results: EPS occurred in 26/920 patients with a prevalence of 2.8 % and incidence of 1/105 patient-years. The prevalence increased with the time spent on PD: 0.4 % for PD duration <2 years, 3 % (2-4 years), 4 % (4-6 years), 6 % (6-8 years), 8 % (8-10 years), 18 % (10-12 years), 75 % (12-14 years), 67 % (>14 years). EPS prevalence was not higher in PD patients transplanted: 5/172 (2.9 %); only two of them (1.2 %) were diagnosed while with a functioning graft. In only one patient (0.6 %) was the diagnosis made during hemodialysis; the other 23 were diagnosed while still on PD. Mortality due to EPS was 38.5 %, and was associated with PD duration. Therapy with steroids reduced mortality [hazard ratio 0.047 (95 % CI: 0.008-0.273); p < 0.001].

Conclusions: In our experience the prevalence of EPS is low, but increases progressively with the duration of PD. The transfer to hemodialysis or transplantation does not appear to be a key factor for EPS. Therapy with steroids significantly improves the outcome.

Keywords: Abdominal cocoon; Dialysis; Encapsulating peritoneal sclerosis; Peritoneal dialysis; Peritonitis.

MeSH terms

  • Adolescent
  • Adult
  • Aged
  • Aged, 80 and over
  • Child
  • Early Diagnosis
  • Female
  • Humans
  • Incidence
  • Italy / epidemiology
  • Kaplan-Meier Estimate
  • Male
  • Middle Aged
  • Peritoneal Dialysis / adverse effects*
  • Peritoneal Dialysis / mortality
  • Peritoneal Fibrosis / diagnosis
  • Peritoneal Fibrosis / drug therapy
  • Peritoneal Fibrosis / epidemiology*
  • Peritoneal Fibrosis / mortality
  • Predictive Value of Tests
  • Prevalence
  • Proportional Hazards Models
  • Retrospective Studies
  • Risk Factors
  • Steroids / therapeutic use
  • Time Factors
  • Treatment Outcome
  • Young Adult

Substances

  • Steroids