Encapsulating peritoneal sclerosis

Table 3 Summary of studies of interventions in encapsulating peritoneal sclerosis

	Design	Patient population	Treatment	Outcome	Comments
Kuriyama 2001	Retrospective case-control	n = 11 Japan PD patients Age - 49.1 yr 27% female	Steroids Prednisolone Dose - 0.5 mg/kg/d Duration - NS n = 5	Steroids All remained alive at 1-3 yr after diagnosis.	All control patients were diagnosed prior to 1997 and all who received steroids were diagnosed after 1997.
			Control TPN-alone n = 6	Control All died of EPS-related complications within 8 mo of diagnosis.
Kawanishi 2004	Prospective cohort	n = 48 Japan PD patients Age - 54.7 yr 25% female	Steroids - Prednisolone Dose - 10-40 mg/d Methylprednisolone Dose - 0.5-1.0 g/d Duration – NS n = 39	Steroids Recovery - 38.5% Surgery - 15.4% Mortality - 31%	Six steroid patients underwent surgery. Surgical treatment consisted of total enterolysis.
			Surgery Total enterolysis n = 12	Surgery Recovery - 58.3 % Mortality - 33%
			Control TPN-alone n = 3	Control Recovery - 0% Mortality - 66%
Maruyama 2008	Retrospective case series	n = 79 Japan PD patients	Steroids Prednisolone Dose – 2.6-60 mg/d Duration - 1-36 mo n = 79	Steroids Mortality – Stage 2 – 3.6% Stage 3 – 14.3% Stage 4 – 25%	Did not compare to a control group.
Balasubramanian 2009	Retrospective case series	n = 111 United Kingdom PD patients Age - 52.0 yr 53% female	Steroids ± immunosuppression n = 7	Steroids ± immunosuppression Median survival 7 mo	Dose and duration of medications not specified. Numerous patients received combinations of therapies. Immunosuppression consisted of azathioprine, cyclosporin, tacrolimus, mycophenolate mofetil, or sirolimus. Unable to analyze statistically due to heterogeneity in groups.
			Tamoxifen n = 17	Tamoxifen Median survival 15 mo
			Tamoxifen + steroids ± immunosuppression n = 8	Tamoxifen + steroids ± immunosuppression Median survival 14 mo
			Surgery Adhesiolysis (n = 5) jejunostomy (n = 1) Small bowel resection (n = 1) Ileal-transverse colon bypass (n = 1)	Surgery Median survival 17 mo
			Control No specific drug therapy (n = 46)	Control Median survival 13 mo
Korte 2011	Retrospective survival analysis	n = 63 Netherlands PD patients Age 43.4 yr 50% female	Tamoxifen Dose - Dose 10 mg/d to 20 mg twice daily Duration – at least 4 wk n = 24	Tamoxifen Mortality rate - 45.8%	None underwent surgery in either group. Patients in both groups received steroids, which was not analyzed separately other than noting a trend towards improved mortality in the tamoxifen group.
			Control No tamoxifen n =39	Control Mortality rate - 74.4%
Kawanishi 2011	Retrospective case series	n = 181 Japan PD patients	Surgery Total enterolysis n = 181	Surgery Recurrence – 25.4% Surgical mortality – 7.7% Overall mortality – 35% 0/17 with Noble plication experienced recurrence at 8 mo	Heterogeneous operation types. Those after April 2007 received Noble plication.
Ulmer 2013	Retrospective case series	n = 26 Netherlands PD patients Age 54 yr 11% female	Surgery Peritonectomy and enterolysis (PEEL)	Surgery Major morbidity - 31% Reoperation – 17% Recurrence – 10% Surgical mortality – 10%	8 patients received steroids, 1 tamoxifen, and 1 tacrolimus pre-operatively.

PD: Peritoneal dialysis; EPS: Encapsulating peritoneal sclerosis.