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Copyright ©The Author(s) 2018.
World J Gastroenterol. Jul 28, 2018; 24(28): 3101-3111
Published online Jul 28, 2018. doi: 10.3748/wjg.v24.i28.3101
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
TerminologyClinical findingsTerminologyClinical findings
Stage 1Pre-EPS stageLoss of ultrafiltration capacity Development of a high transport Hypoproteinemia Bloody dialysate, ascites Calcifications in the peritoneumPre-stageAbdominal symptoms: Mild Inflammation: Mild Encapsulation: None
Stage 2Inflammation stageIncreased CRP, leukocytosis Fever, chills, weight loss, anorexia Diarrhea, ascitesInflammatoryAbdominal symptoms: Nausea, diarrhea Inflammation: Mild to severe Encapsulation: Partial
Stage 3Encapsulating stageDecreased clinical signs of systemic inflammation Early signs of ileus (abdominal pain, nausea, vomiting)EncapsulatingAbdominal symptoms: Periodic ileus Inflammation: Mild Encapsulation: Present
Stage 4Ileus stageAnorexia Complete ileus Abdominal massChronicAbdominal symptoms: Persistent ileus Inflammation: None to mild Encapsulation: Present
Table 3 Summary of studies of interventions in encapsulating peritoneal sclerosis
DesignPatient populationTreatmentOutcomeComments
Kuriyama 2001Retrospective case-controln = 11 Japan PD patients Age - 49.1 yr 27% femaleSteroids Prednisolone Dose - 0.5 mg/kg/d Duration - NS n = 5Steroids 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 = 6Control All died of EPS-related complications within 8 mo of diagnosis.
Kawanishi 2004Prospective cohortn = 48 Japan PD patients Age - 54.7 yr 25% femaleSteroids - Prednisolone Dose - 10-40 mg/d Methylprednisolone Dose - 0.5-1.0 g/d Duration – NS n = 39Steroids Recovery - 38.5% Surgery - 15.4% Mortality - 31%Six steroid patients underwent surgery. Surgical treatment consisted of total enterolysis.
Surgery Total enterolysis n = 12Surgery Recovery - 58.3 % Mortality - 33%
Control TPN-alone n = 3Control Recovery - 0% Mortality - 66%
Maruyama 2008Retrospective case seriesn = 79 Japan PD patientsSteroids Prednisolone Dose – 2.6-60 mg/d Duration - 1-36 mo n = 79Steroids Mortality – Stage 2 – 3.6% Stage 3 – 14.3% Stage 4 – 25%Did not compare to a control group.
Balasubramanian 2009Retrospective case seriesn = 111 United Kingdom PD patients Age - 52.0 yr 53% femaleSteroids ± immunosuppression n = 7Steroids ± immunosuppression Median survival 7 moDose 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 = 17Tamoxifen Median survival 15 mo
Tamoxifen + steroids ± immunosuppression n = 8Tamoxifen + 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 2011Retrospective survival analysisn = 63 Netherlands PD patients Age 43.4 yr 50% femaleTamoxifen Dose - Dose 10 mg/d to 20 mg twice daily Duration – at least 4 wk n = 24Tamoxifen 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 =39Control Mortality rate - 74.4%
Kawanishi 2011Retrospective case seriesn = 181 Japan PD patientsSurgery Total enterolysis n = 181Surgery Recurrence – 25.4% Surgical mortality – 7.7% Overall mortality – 35% 0/17 with Noble plication experienced recurrence at 8 moHeterogeneous operation types. Those after April 2007 received Noble plication.
Ulmer 2013Retrospective case seriesn = 26 Netherlands PD patients Age 54 yr 11% femaleSurgery 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.