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
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 | |||
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. |
- Citation: Danford CJ, Lin SC, Smith MP, Wolf JL. Encapsulating peritoneal sclerosis. World J Gastroenterol 2018; 24(28): 3101-3111
- URL: https://www.wjgnet.com/1007-9327/full/v24/i28/3101.htm
- DOI: https://dx.doi.org/10.3748/wjg.v24.i28.3101