The Pan-Thames EPS study: treatment and outcomes of encapsulating peritoneal sclerosis.
BACKGROUND
Encapsulating peritoneal sclerosis (EPS) is a disease process that can occur as a complication of peritoneal dialysis (PD). The aim of this study was to make a general assessment of the clinical features, diagnosis, management and outcome of PD-related EPS cases from London and South-East England.


METHODS
Questionnaires were sent to 11 PD units in March 2007; cases were identified retrospectively. Outcome data on surviving patients were collected in March 2008.


RESULTS
A total of 111 patients were identified; the mean time on PD was 82 months (range 8-247). Mortality increased with length of time on PD, being 42% at <3 years (n = 12), 32% at 3-4 years (n = 19), 61% at 5-6 years (n = 31), 54% at 7-8 years (n = 24), 75% at 9-10 years (n = 8) and 59% at >10 years (n = 17). Twelve patients had no previous peritonitis episodes, 28 had one previous episode, 30 had two previous episodes and 33 had three or more previous episodes. Of the patients with PD details available, 41/63 were high (>0.81) transporters and 44/71 had ultrafiltration <1 l/24 h, but 7/63 were low average transporters (0.5-<0.65) and 27/71 had ultrafiltration >1 l/24 h and a few had significant residual renal function. Sixty-five (59%) patients had their PD discontinued prior to diagnosis (51 HD; 14 transplanted). CT scans were performed on 91 patients and laparotomy on 47 patients. Drug treatment consisted of tamoxifen, immunosuppression or both. The median survival was 15 months in patients treated with tamoxifen (n = 17), 12 months in patients treated with immunosuppression (n = 24) and 21 months in patients who received both (n = 13), against 13 months (n = 46) in patients who received no specific treatment. Adhesionolysis was performed in 5 patients, and 39 patients were given parenteral nutrition. The overall mortality was 53% with a median survival of 14 months and a median time to death of 7 months. Conclusion. This is one of the largest cohorts of patients with EPS in the literature. Long-term survival occurred in over 50%, regardless of the various treatments strategies undertaken by the centres.
Balasubramaniam
Gowrie
G
West London Renal and Transplant Centre, London, UK. gowrie@doctors.org.uk
Brown
Edwina A
EA
Davenport
Andrew
A
Cairns
Hugh
H
Cooper
Barbara
B
Fan
Stanley L S
SL
Farrington
Ken
K
Gallagher
Hugh
H
Harnett
Patrick
P
Krausze
Sally
S
Steddon
Simon
S
D000328:Adult; D000368:Aged; D004739:England; D005260:Female; D006801:Humans; D008131:London; D008297:Male; D008875:Middle Aged; D056627:Peritoneal Fibrosis / Q000175:diagnosis* / Q000401:mortality / Q000628:therapy*; D012189:Retrospective Studies; D015996:Survival Rate; D016896:Treatment Outcome; D055815:Young Adult