title: Phenotypes of encapsulating peritoneal sclerosis--macroscopic appearance, histologic findings, and outcome.
abstract: BACKGROUND
  Encapsulating peritoneal sclerosis (EPS) is a rare but devastating complication of peritoneal dialysis (PD), with clinical signs of abdominal pain, bowel obstruction, and weight loss in late stages.
  
  
  METHODS
  We retrospectively analyzed all patients who were diagnosed with EPS between March 1998 and October 2011 in our department of nephrology. We focused on the 24 EPS patients who underwent surgery because of symptomatic late-stage EPS. We identified 3 different macroscopic phenotypes of EPS that we categorized as types I - III. We correlated histologic findings with those macroscopic phenotypes of EPS. The postoperative and long-term outcomes were evaluated by macroscopic phenotype.
  
  
  RESULTS
  Duration of PD was longer in type III than in types I and II EPS (p = 0.05). We observed no other statistically significant differences between the groups in baseline characteristics, except for operation time, which was longer in the type I than in the type III group (p = 0.02). Furthermore, we observed no statistically significant difference between the groups with respect to the onset of complaints before surgery (7.8 ± 5.9 months vs 7.0 ± 7.0 months vs 6.5 ± 5.3 months). Concerning patient outcomes, there was no evidence that any of the macroscopic EPS types was associated with more major or minor complications after surgery. For all study patients, follow-up was at least 3 years, with 19 patients still being alive, and 16 having no or very mild complaints. The typical histologic findings of EPS were present in all macroscopic types; only fibrin deposits were more prominent in type II than in type III.
  
  
  CONCLUSIONS
  We describe 3 subtypes of EPS based on macroscopic findings. Postoperative treatment should probably not be influenced by the macroscopic EPS phenotype. Whether the different phenotypes represent different pathophysiologic processes remains unclear and has to be further evaluated.
authors: Department of Internal Medicine,1 Division of Nephrology, Robert-Bosch-Hospital, and Department of General, Visceral and Trauma Surgery,2 Robert-Bosch-Hospital, Stuttgart, Germany;
authors: Joerg
authors: J
authors: Latus
authors: Christoph
authors: C
authors: Ulmer
authors: Peter
authors: P
authors: Fritz
authors: Bianka
authors: B
authors: Rettenmaier
authors: Dagmar
authors: D
authors: Biegger
authors: Thomas
authors: T
authors: Lang
authors: German
authors: G
authors: Ott
authors: Martin
authors: M
authors: Kimmel
authors: Wolfgang
authors: W
authors: Steurer
authors: M Dominik
authors: MD
authors: Alscher
authors: Stephan
authors: S
authors: Segerer
authors: Niko
authors: N
authors: Braun
mesh_terms: D005260:Female; D005347:Fibroblasts / Q000473:pathology; D005500:Follow-Up Studies; D006801:Humans; D007676:Kidney Failure, Chronic / Q000628:therapy*; D008297:Male; D008875:Middle Aged; D010530:Peritoneal Dialysis / Q000009:adverse effects*; D056627:Peritoneal Fibrosis / Q000209:etiology / Q000473:pathology*; D010537:Peritoneum / Q000473:pathology*; D010641:Phenotype; D011379:Prognosis; D012189:Retrospective Studies; D012720:Severity of Illness Index; D013997:Time Factors
keywords: Encapsulating peritoneal sclerosis; macroscopic phenotype
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