Encapsulating peritoneal sclerosis

Table 1 Classification and etiologies of encapsulating peritoneal sclerosis

Primary (idiopathic)
2:1 (male:female) Most commonly reported in tropical/subtropical regions
Secondary
Medications Practolol[6] Methotrexate[7,8] Antiepileptic drugs[9] Infection Tuberculosis[10] Non-tuberculous mycobacteria[11] Bacterial peritonitis[12] Cytomegalovirus[13] Fungus[14,15] Parasite[16] Cirrhosis[28] Organ transplantation Liver[29] Small intestine[30] Renal[31] Gynecologic neoplasms Luteinized thecoma[33] Luteinizing granulosa cell tumor[34]	Mechanical or chemical irritation Peritoneal dialysis[17] Intraperitoneal chemotherapy[18] Ventriculoperitoneal shunt[20] Peritoneovenous shunt[21] Intraperitoneal iodine[22] Abdominal trauma[23] Intraabdominal surgery[24] Foreign body[25] Talcum powder[19] Asbestos[26] Silica[27] Endometriosis[32] Dermoid cyst rupture[35] Rheumatologic/systemic inflammatory conditions Sarcoidosis[36] Systemic lupus erythematosis[37] Familial Mediterranean fever[38]

Table 2 Stages of peritoneal dialysis-associated encapsulating peritoneal sclerosis with associated clinical, serologic, and radiographic profiles

	Nakamoto 2005		Nakayama 2014
	Terminology	Clinical findings	Terminology	Clinical findings
Stage 1	Pre-EPS stage	Loss of ultrafiltration capacity Development of a high transport Hypoproteinemia Bloody dialysate, ascites Calcifications in the peritoneum	Pre-stage	Abdominal symptoms: Mild Inflammation: Mild Encapsulation: None
Stage 2	Inflammation stage	Increased CRP, leukocytosis Fever, chills, weight loss, anorexia Diarrhea, ascites	Inflammatory	Abdominal symptoms: Nausea, diarrhea Inflammation: Mild to severe Encapsulation: Partial
Stage 3	Encapsulating stage	Decreased clinical signs of systemic inflammation Early signs of ileus (abdominal pain, nausea, vomiting)	Encapsulating	Abdominal symptoms: Periodic ileus Inflammation: Mild Encapsulation: Present
Stage 4	Ileus stage	Anorexia Complete ileus Abdominal mass	Chronic	Abdominal symptoms: Persistent ileus Inflammation: None to mild Encapsulation: Present

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.