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Epidemiology and Outcomes
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Encapsulating Peritoneal Sclerosis in the New Millennium: A National Cohort Study

Michaela C. Brown, Keith Simpson, Jan J. Kerssens, Robert A. Mactier and ; on behalf of the Scottish Renal Registry
CJASN July 2009, 4 (7) 1222-1229; DOI: https://doi.org/10.2215/CJN.01260209
Michaela C. Brown
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Keith Simpson
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Jan J. Kerssens
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Robert A. Mactier
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    Figure 1.

    Cumulative risk function of developing EPS according to days since first PD exposure between January 1, 2000, and December 31, 2007.

  • Figure 2.
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    Figure 2.

    (A) PD Duration: Non-EPS cohort first exposed to PD after January 1, 2000 (n = 1219). (B) PD Duration: EPS cases first exposed to PD after January 1, 2000 (n = 19).

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    Figure 3.

    Kaplan-Meier survival plots of patients from start of PD for EPS cases (n = 19) and unaffected cohort (n = 1219).

Tables

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    Table 1.

    Incidence rates of EPS related to total duration of PD exposure (not necessarily continuous)

    PD Exposure (years)PD Patients at Risk (n)EPS Cases (n)Incidence (%)95% Confidence Intervals (%)
    <148000–
    1–232620.60.2–2.1
    >2–320242.00.8–5.0
    >3–411443.51.4–8.7
    >4–56258.13.6–17.6
    >5–63438.83.2–23.1
    >62015.01.2–23.8
    Total123819
    • EPS, encapsulating peritoneal sclerosis; PD, peritoneal dialysis.

    • View popup
    Table 2.

    Comparison of previous and current epidemiological studies of EPS

    Study
    Nomoto et al. Japan 1996Rigby et al. Australia 1998Lee et al. Korea 2003Kawanishi et al. Japan 2001Kawanishi et al. Japan 2004Summers et al. UK 2005Brown et al. UK (current study)
    Number of EPS Casesa6254 (46)31174827 (23)46
    Dates of Study1980–19941980–19941981–20021999–20011999–20031998–20032000–2007
    Study DesignRetrospective Multi-centerRetrospective Multi-centerRetrospective Multi-centerProspective Multi-centerProspective Multi-centerRetrospective Single-centerRetrospective Multi-center
    Denominator Populationb692373743888221619588101638
    Overall Rate0.9%0.7%0.8%0.8%2.5%3.3%2.8%
    Mean PD Exposure (yrs)5.14.35.8104.36.15.4
    Mortality (over study period)43.5%56%25.8%35%37.5%29.6%56.5%
    • EPS, encapsulating peritoneal sclerosis; PD, peritoneal dialysis.

    • ↵a Those meeting ISPD 2000 criteria in brackets.

    • ↵b Prevalent + incident PD patients.

    • View popup
    Table 3.

    Underlying cause of renal failure of the EPS cases and incident PD population 2000-2007 (GN = glomerulonephritis)

    Cause of Renal FailurePD Cohort (N)PD Cohort (%)EPS Group A (N)EPS Group A (%)EPS Group B (N)EPS Group B (%)
    CRF, uncertain etiology21918.0817.4421.1
    Diabetes Type I (insulin dependent)18615.312.215.3
    Autosomal Dominant Polycystic Kidney Disease13110.7510.9315.8
    Pyelonephritis with Urinary Tract Disease12810.5715.215.3
    Renovascular Disease12610.312.2
    Chronic GN (unspecified)675.548.7210.5
    Diabetes Type II (non insulin dependent)665.412.215.3
    IgA Nephropathy635.236.5210.5
    Other564.648.715.3
    Multisystem Disorders524.312.2
    Membranous Nephropathy242.024.3
    Myeloma/Light Chain Deposition Disease221.8
    Focal Segment Glomerulosclerosis151.2510.9315.8
    Drug-induced Nephropathy171.4
    Amyloidosis151.2
    Herediatry Nephropahy (inc. Alport's)121.024.315.3
    Rapidly Progressive/Crescentic GN110.912.2
    Membranoproliferative GN90.712.2
    Total12191004610019100
    • EPS, encapsulating peritoneal sclerosis; PD, peritoneal dialysis.

    • View popup
    Table 4.

    Main clinical features and radiological abnormalities found in the 46 cases of EPS diagnosed in Scotland 2000-2007

    Clinical Features (Most cases had >1)NImaging Findings (US or CT scan) (some cases had >1)N
    Abdominal Pain30Ascites33
    Vomiting28Septate/loculated ascites22
    Weight Loss24Peritoneal thickening14
    Ascites15Peritoneal calcification11
    Elevated Inflammatory Markers14Bowel obstruction3
    Bowel Obstruction11Matted/tethered bowel10
    Hypoalbuminaemia10Dilated Small Bowel4
    Unexplained Anaemia10
    Bloody Ascites/Dialysate4
    Abdominal Mass4
    Diarrhoea4
    • EPS, encapsulating peritoneal sclerosis.

    • View popup
    Table 5.

    Total creatinine clearance (renal and PD clearance combined) for EPS cases within 6 mo before stopping PD compared to Scottish PD patients’ achieved clearance from Scottish Renal Registry data 2000-2007

    Total Clearance (L/wk)EPS Cases (N)EPS Cases (%)PD Population 2000–2007 (%)
    <50510.95.7
    >50-601226.111.0
    >60-701430.416.0
    >701430.467.0
    Unknown12.20.0
    Total46100.0100.0
    • EPS, encapsulating peritoneal sclerosis; PD, peritoneal dialysis.

    • View popup
    Table 6.

    Reasons for stopping PD in the EPS cases and the Scottish PD population from Scottish Renal Registry data 2000-2007

    Reason for Stopping PDEPS Cases (N)EPS Cases (%)PD Population 2000-2007 (%)P value
    Peritonitis1532.624.30.2
    Ultrafiltration Failure613.02.3<0.001
    Inadequate Dialysis919.611.70.03
    Failed Access——5.3—
    High Intra—abdominal Pressure——4.5—
    Transplant715.220.10.4
    Patient Choice12.27.50.2
    Death——24.3<0.001
    EPS817.4——
    Total46.0100.0100.0
    • EPS, encapsulating peritoneal sclerosis; PD, peritoneal dialysis.

    • View popup
    Table 7.

    Immunosuppressive drug therapy prescribed to EPS cases

    Treatment/Transplant StatusNo. of Cases
    Tamoxifen only6
    Sirolimus3a
    Prednisolone + Tamoxifen4
    Tamoxifen + Azathioprine1
    Prednisolone + Azathioprine1
    Functioning transplant (Tamoxifen added)6
    Transplanted <4 months post-diagnosis4
    Total treated with Tamoxifen13
    Total Treated with immunosuppression24
    • EPS, encapsulating peritoneal sclerosis.

    • ↵a For one of the three treated with Sirolimus it was commenced as part of posttransplant immunosuppressive therapy.

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Clinical Journal of the American Society of Nephrology
Vol. 4, Issue 7
July 2009
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Encapsulating Peritoneal Sclerosis in the New Millennium: A National Cohort Study
Michaela C. Brown, Keith Simpson, Jan J. Kerssens, Robert A. Mactier
CJASN Jul 2009, 4 (7) 1222-1229; DOI: 10.2215/CJN.01260209

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Encapsulating Peritoneal Sclerosis in the New Millennium: A National Cohort Study
Michaela C. Brown, Keith Simpson, Jan J. Kerssens, Robert A. Mactier
CJASN Jul 2009, 4 (7) 1222-1229; DOI: 10.2215/CJN.01260209
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  • Association between Monocyte Count and Risk of Incident CKD and Progression to ESRD
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  • Length of Time on Peritoneal Dialysis and Encapsulating Peritoneal Sclerosis -- Position Paper for ISPD: 2017 Update
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  • The Current State of Peritoneal Dialysis
  • Higher Dialysate Matrix Metalloproteinase-2 Levels Are Associated with Peritoneal Membrane Dysfunction
  • Interstitial Fibrosis Restricts Osmotic Water Transport in Encapsulating Peritoneal Sclerosis
  • Genetic Polymorphisms and Peritoneal Membrane Function
  • Encapsulating Peritoneal Sclerosis in the Era of a Multi-Disciplinary Approach Based on Biocompatible Solutions: the NEXT-PD Study
  • Living with Encapsulating Peritoneal Sclerosis (EPS): The Patient's Perspective
  • Is There an End in Sight for Encapsulating Peritoneal Sclerosis?
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  • Can EPS Development Be Avoided with Early Interventions? The Potential Role of Tamoxifen--A Single-Center Study
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  • Canadian Society of Nephrology Guidelines/Recommendations on Peritoneal Dialysis Adequacy 2011--Response to Comments by Liakopoulos et al.
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  • DIFFERENCE IN THE EXPRESSION OF HORMONE RECEPTORS AND FIBROTIC MARKERS IN THE HUMAN PERITONEUM--IMPLICATIONS FOR THERAPEUTIC TARGETS TO PREVENT ENCAPSULATING PERITONEAL SCLEROSIS
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