Encapsulating Peritoneal Sclerosis Research Articles

A Rare Case: Tuberculous Peritonitis, Encapsulating Peritoneal Sclerosis, and Incisional Hernia in Continuous Ambulatory Peritoneal Dialysis Patient

BACKGROUND: Peritonitis is the most common infectious complication of peritoneal dialysis (PD) with an estimated ratio of 1:20–30 patients per month. In addition, less than 3% cases are due to Mycobacteria, although not all are caused by Mycobacteria tuberculosis. Therefore, specific examinations are needed for proper diagnosis. Encapsulating peritoneal sclerosis (EPS), another rare complication of PD, accounts for 0.7–13.6 per 1000 patients per year. CASE REPORT: A 37-year-old man undergoing PD, with complaints of intermittent abdominal pain and cloudy fluid, followed by nausea, vomiting, and constipation. Furthermore, visible protrusion was observed on the abdominal wall due to the wound from the Tenckhoff catheter insertion surgery. This is clearly comprehended as the patient sits or stands but disappears on lying down. Along with the condition, continuous ambulatory PD (CAPD) ultrafiltration ability decreases, rough defecation occurs, with a hard sensation on the lower right abdomen. Moreover, the patient had earlier suffered peritonitis for the 3rd time. The results of the dialysate fluid analysis showed a cloudy liquid coloration, as the number of cells 278, polymorphonuclear 87, mononuclear 13, Ziehl–Neelsen +1 and acid-resistant bacteria +3 staining, including GeneXpert MTB/RIF, were positive. Furthermore, abdominal computed tomography (CT) scan revealed a thick peritoneum, partly with calcification, air-filled intestinal, dilated colon with wall thickening. Furthermore, the mesentery lining the liver and intestine were observed to be dense with multiple calcifications to support an EPS. Definitive diagnosis is confirmed by laparotomy and/or laparoscopy, but CT scan provides an alternative. Subsequently, CAPD utilization is discontinued and switched to renal replacement therapy to hemodialysis twice a week due to several complications associated with PD, ranging from recurrent peritonitis, tuberculous peritonitis, EPS, and incisional hernias responsible for an ineffective PD ultrafiltration. CONCLUSION: At present, the combination of clinical symptoms, radiology, and medical pathology remains the key to diagnosing tuberculous peritonitis and EPS. Consequently, prompt and precise analysis determines a good prognosis.

An unusual case of intestinal obstruction due to abdominal cocoon: A case report.

Introduction and importanceAbdominal cocoon (AC) or Encapsulating Peritoneal Sclerosis (EPS) is a rare cause of bowel obstruction and due to non-specific presentation, it can be misdiagnosed and often mistreated.Case presentationWe present the case of 42 years male with a history suggestive of complete small bowel obstruction (SBO) without a history of pulmonary tuberculosis (TB) or peritoneal dialysis. CT imaging as well as the intraoperative finding of a cocoon membrane encasing the small bowel led to the diagnosis of abdominal cocoon.Clinical discussionAbdominal cocoon can be idiopathic or secondary to peritoneal dialysis, tuberculosis, or other rare causes. Patients usually present with features of SBO with varying severity. Diagnosis is aided by imaging investigations mainly CT scan and management is primarily surgical and usually involves adhesiolysis, total removal of the membrane with or without bowel loop resection.ConclusionDiagnosis of abdominal cocoon warrants awareness of the disease and a high index of suspicion of the treating clinician in patients with intestinal obstruction and an abdominal lump without a history of previous abdominal surgery. CT can guide diagnosis and early operative management seems to bear the best outcomes.

Encapsulating peritoneal sclerosis in an AMA-M2 positive patient: A case report

BACKGROUNDEncapsulating peritoneal sclerosis (EPS) is hard to diagnose because of nonspecific symptoms and signs. It is a general consensus that EPS is classified as primary and secondary. There have been several studies discovering some high-risk factors such as liver cirrhosis, of which AMA-M2 is a biomarker, and intra-abdominal surgery such as laparoscopic surgery. Imaging studies help to diagnose EPS and exploratory laparotomy might be an alternative if imaging fails. Nowadays, laparotomy plays a key role in treating EPS, especially when medical treatments do not work and medical therapy fails to ease patients’ symptoms.CASE SUMMARYA 58-year-old man complained of unexplained vomiting and abdominal distension 2 mo after laparoscopic cholecystectomy. Increased alkaline phosphatase and liver enzymes were discovered. An autoimmune liver disease test showed that AMA-M2 was positive. A gastroscopy revealed bile reflux gastritis. A magnetic resonance imaging scan showed a slight dilatation of the intrahepatic bile duct. A colonoscopy showed that there was a mucosal eminence lesion in the sigmoid colon (24 cm away from the anus), with a size of 3 cm × 3 cm and erosive surface. At last, the small intestine and the stomach were found to be encased in a cocoon-like membrane during the surgery. The membrane was dissected and adhesiolysis was done to release the trapped organs. The patient recovered and was discharged 44 d after the operation, and there was no recurrence during a follow-up period of 3 mo.CONCLUSIONAMA-M2 is a marker of primary biliary sclerosis and may help to make a preoperative diagnosis of EPS.

Risk factors and clinical outcomes of encapsulating peritoneal sclerosis: A case-control study from China.

Encapsulating peritoneal sclerosis (EPS) is an uncommon, but serious complication in patients with continuous ambulatory peritoneal dialysis (PD) who have a considerable mortality rate. This study aimed to identify risk factors and outcomes of EPS in Chinese patients on PD. Sixteen patients on PD who met the International Society for Peritoneal Dialysis criteria for diagnosis of EPS in the First Affiliated Hospital of Sun Yat-Sen University from 1997 to 2018 were included. Patients without EPS were matched for age, sex and the duration of PD and selected at a 1:3 ratio for the controls. A case-control study was conducted to analyse the clinical profile and risk factors associated with EPS in patients. The prevalence of EPS in patients on PD in our centre was 0.55%. The percentage of EPS significantly increased with the duration of PD. In univariate regression analysis, a history of peritonitis (odds ratios (OR): 2.83; 95% confidence interval (CI): 0.82-9.68; p = 0.08), peritoneal glucose exposure (OR: 1.12; 95% CI: 1.03-1.22; p < 0.01) and a high peritoneal transport status (OR: 14.70; 95% CI: 1.85-117.02; p < 0.01) were associated with EPS in patients on PD. However in the multivariate model, only a high peritoneal transport status (adjusted odds ratios (aOR): 13.65; 95% CI: 1.69-109.96; p = 0.01) was independently associated with EPS. The rate of EPS significantly increases with the duration of PD. Progressive peritoneal dysfunction, especially a high peritoneal transport status, is associated with a higher risk of EPS in this population.

Peritoneal Dialysis Guidelines 2019 Part 1 (Position paper of the Japanese Society for Dialysis Therapy)

Approximately 10 years have passed since the Peritoneal Dialysis Guidelines were formulated in 2009. Much evidence has been reported during the succeeding years, which were not taken into consideration in the previous guidelines, e.g., the next peritoneal dialysis PD trial of encapsulating peritoneal sclerosis (EPS) in Japan, the significance of angiotensin-converting enzyme inhibitors (ACEIs) and angiotensin receptor blockers (ARBs), the effects of icodextrin solution, new developments in peritoneal pathology, and a new international recommendation on a proposal for exit-site management. It is essential to incorporate these new developments into the new clinical practice guidelines. Meanwhile, the process of creating such guidelines has changed dramatically worldwide and differs from the process of creating what were “clinical practice guides.” For this revision, we not only conducted systematic reviews using global standard methods but also decided to adopt a two-part structure to create a reference tool, which could be used widely by the society’s members attending a variety of patients. Through a working group consensus, it was decided that Part 1 would present conventional descriptions and Part 2 would pose clinical questions (CQs) in a systematic review format. Thus, Part 1 vastly covers PD that would satisfy the requirements of the members of the Japanese Society for Dialysis Therapy (JSDT). This article is the duplicated publication from the Japanese version of the guidelines and has been reproduced with permission from the JSDT.

Encapsulating Peritoneal Sclerosis and Mortality Related to Infection in Patients on Combination Once-Weekly Hemodialysis with Peritoneal Dialysis

Introduction: Previous studies showed that the combination of peritoneal dialysis (PD) and once-weekly hemodialysis is associated with lower all-cause and cardiovascular mortality. This study aimed to compare the incidence of encapsulating peritoneal sclerosis (EPS) and infection-related mortality among those on combination therapy and those on PD alone. Methods: This prospective study on the Japanese Renal Data Registry included patients on PD from 2010 to 2014. Subjects were followed up until the end of 2015. Exposure of interest was combination therapy compared with PD alone. Patients who transitioned to combination therapy were matched with those on PD alone by propensity scores. Outcomes were EPS and infection-related mortality. Data were analyzed using Cox regression models. Results: Among the matched cohort, 608 and 869 patients were on combination therapy and on PD alone, respectively. Dialysate-to-plasma creatinine (D/P Cr) ratio decreased over time among those on combination therapy, while the ratio increased among those on PD alone (p = 0.01 by the mixed-effects model). During a median follow-up of 2.5 years, 33 experienced EPS and 55 died of infection. Combination therapy was associated with lower infection-related mortality (HR [95% CI]: 0.52 [0.28–0.95]) but not with EPS (HR: 1.21 [0.61–2.40]). Lower mortality was not limited to intra-abdominal infection but also observed for pulmonary infection. Sensitivity analyses considering the effects of dialysis facilities yielded similar results. Conclusions: Combination therapy was associated with lower infection-related mortality. It was also associated with a decline in the D/P Cr ratio over time but not with lower incidence of EPS during the short observation period.

A Matrix Metalloproteinase-2-Based Nomogram to Assess the Risk of Encapsulating Peritoneal Sclerosis in Peritoneal Dialysis Patients.

Background Encapsulating peritoneal sclerosis (EPS) is a rare but serious complication of peritoneal dialysis (PD). So far, there is no biomarker-based prediction tool available for EPS. Matrix metalloproteinase-2 (MMP-2) is a protein involved in the breakdown of the extracellular matrix, and the effluent MMP-2 can be a potential biomarker of EPS. This study is aimed at developing a nomogram for EPS based on effluent MMP-2 levels. Patients and Methods. We enrolled 18 EPS patients and 90 gender-matched PD patients without EPS in this cross-sectional case-controlled study. The effluent MMP-2 levels and possible risk factors for EPS were analyzed using multivariable logistic regression, and a nomogram was developed. The nomogram was validated using 200 bootstrap resamples to reduce overfit bias. Results The effluent MMP-2 levels in EPS patients were significantly higher than those in normal PD patients (p < 0.001, Manny-Whitney U test). Effluent MMP-2 levels and PD duration were independently associated with EPS risks (p < 0.001 and p = 0.001) in multivariate logistic regression. A nomogram based on MMP-2 levels and PD duration was proposed. The AUC of MMP-2 was 0.824, and the AUC of the nomogram was 0.907 (p = 0.05). Conclusion A nomogram based on effluent MMP-2 levels and PD duration may predict EPS with high accuracy.

A disposable, ultra-fine endoscope for non-invasive, close examination of the intraluminal surface of the peritoneal dialysis catheter and peritoneal cavity

The ability to visualize intraluminal surface of peritoneal dialysis (PD) catheter and peritoneal cavity could allow elucidation of the cases of outflow problems, and provide information on changes to the peritoneal membrane leading to encapsulating peritoneal sclerosis. A non-invasive examination that allows those monitoring in need is desirable. We have developed a disposable ultra-fine endoscope that can be inserted into the lumen of the existing PD catheter, allowing observation of the luminal side of the catheter and peritoneal cavity from the tip of the PD catheter, with minimum invasion in practice. In a pre-clinical study in pigs and a clinical study in 10 PD patients, the device provided detailed images, enabling safe, easy observation of the intraluminal side of the entire catheter, and of the morphology and status of the peritoneal surface in the abdominal cavity under dwelling PD solution. Since this device can be used repeatedly during PD therapy, clinical application of this device could contribute to improved management of clinical issues in current PD therapy, positioning PD as a safer, more reliable treatment modality for end-stage renal disease.

Abdominal Cocoon or Encapsulating Peritoneal Sclerosis: A Rare Cause of Small Bowel Obstruction.

Encapsulating peritoneal sclerosis (EPS), also known as abdominal cocoon syndrome (AC) or sclerosing encapsulating peritonitis (SEP), is an uncommon condition typically presenting with features of bowel obstruction. We present the case of a 41-year-old male patient who presented to the accident and emergency department with a 7-day history of abdominal pain. Contrast CT of the abdomen and pelvis was ordered and was suggestive of small bowel obstruction involving most of the small bowel with no apparent transition point. Laparotomy showed a tough whitish fibrous membrane encasing the entire length of the small bowel. Advances in CT have made diagnosis possible before a decision on surgical intervention is made.LEARNING POINTSDespite being a rare cause of bowel obstruction, based on the clinical presentation and CT findings, abdominal cocoon syndrome should be included in the differential diagnosis.CT of the abdomen is the investigation of choice for most cases of bowel obstruction and can be very helpful in reaching a diagnosis before operative management is undertaken.Laparotomy is the usual choice for management, but laparoscopy can be considered either to establish the diagnosis or to deal with the abdominal cocoon based on the surgeon’s clinical judgement and experience.

The effect of rituximab on encapsulated peritoneal sclerosis in an experimental rat model

Background/aim Peritoneal sclerosis may be observed in varied manifestations. However, the most serious form is the encapsulated peritoneal sclerosis. We researched the effect of rituximab on peritoneal fibrosis in an experimental rat model.Materials and methodsTwenty-four Wistar Albino rats were divided into 4 equal groups. During weeks 0–3; group I received isotonic saline (IS) solution, group II, group III, and group IV received chlorhexidine gluconate (CG) via intraperitoneal (i.p.) route. In the next 3 weeks nothing adminestred to both group I and group II but IS solution was adminestred to group III via i.p. route and 375 mg/m2/week rituximab was applied intravenously on days 21, 28, and 35 to group IV. Fibrosis, peritoneal thickness, and inflammation were evaluated. Immunohistochemical methods used for the detection of matrix MMP-2, TGF-β1, and VGEF expressions.Results The rituximab (group IV) had significantly lower fibrosis and peritoneal thickness scores than the group II and III (P < 0.001). TGF-β1 and VEGF expressions were significantly lower in the rituximab group than in the group II and III (P < 0.001).Conclusion: We found that rituximab had a significant effect on the peritoneal thickness, total fibrosis, TGF-β1 and VGEF scores which were induced by CG.

Treatments and outcomes of encapsulating peritoneal sclerosis in patients undergoing peritoneal dialysis: 295 cases from a nationwide inpatient database in Japan.

The number of patients undergoing renal replacement therapy is increasing. We evaluated the practice patterns and outcomes of encapsulating peritoneal sclerosis (EPS) in patients undergoing peritoneal dialysis. Using a Japanese national inpatient database, we identified 295 patients with EPS who were hospitalized from July 2010 to March 2017. We categorized them into four groups: those who underwent surgery only (n = 39), those who received corticosteroid treatment only (n = 70), those who underwent both (n = 30), and those who underwent neither (n = 156). We investigated their characteristics, treatments, and outcomes. More than half of patients were males and never-smokers and had a normal body mass index. Patients tended to undergo parenteral nutrition for 2 months. The proportions of emergency admission, intensive care unit (ICU) admission, central venous catheterization, catecholamine use, mechanical ventilation, and continuous hemodiafiltration were significantly different among the four groups (61%, 8.1%, 37.0%, 44.0%, 8.8%, and 5.8%, respectively). The both-treatment group had a significantly longer hospital stay (37.0 vs. 37.5 vs. 72.5 vs. 31.0 days, p < 0.001) and higher costs (US$16,554 vs. US$17,029 vs. US$33,757 vs. US$13,983, p < 0.001) than the other groups. In total, 52 patients (18%) died during hospitalization. There was no significant difference in inhospital complications and death, discharge status, 30-day readmission, or length of ICU stay among the four groups. Our findings provide useful information for clinicians and patients hospitalized for treatment of EPS.

Ten-year survival of patients treated with peritoneal dialysis: A prospective observational cohort study.

Few studies evaluated over 5-year outcomes of dialysis patients. This study examined 10-year all-cause mortality and death-censored technique failure in a cohort of incident peritoneal dialysis (PD) individuals. Five hundred and thirty-three incident PD individuals from 2006 to 2008 were prospectively followed up for more than 10 years until 2018. Clinical characteristics at PD initiation were collected. The primary outcome was all-cause mortality, and the secondary outcome was death-censored technique failure. Cox hazards models were fit using clinical characteristics at PD initiation. The mean age starting PD for these participants was 48 ± 16 years; 130 (24%) patients had diabetic nephropathy. During follow-up, 208 patients died, and 84 patients experienced technique failures. The 1, 3, 5, and 10 years' survival rates for incident PD patients were 93%, 81%, 64%, and 36%, respectively, and the technical survival rates were 98%, 93%, 85%, and 62%, respectively. Mortality risk was much higher after 3 years on PD. The peritonitis rate was 0.19 episodes per patient-year, and 7 (1.3%) patients had encapsulating peritoneal sclerosis (EPS) giving an incidence rate of 3.1 of 1000 patient-years. The main causes of death were cardiovascular events (97 of 208, 47%), and technique failure was mainly due to peritonitis (41 of 84, 49%). Older age, higher Charlson comorbidity index, and lower level of education were strongly associated with mortality, and diabetic nephropathy was an independent risk factor for technique failure. The 10-year's survival and technique survival rates of incident PD patients were 36% and 62%. Long-term PD can be continued successfully with improved outcomes and low risk for EPS.

P1138FLOW CYTOMETRIC ANALYSIS ON LYMPHOCYTE SUBSETS APPEARED IN PERITONEAL DIALYSIS EFFLUENT IN RELATION WITH PERITONEAL FUNCTION

Abstract Background and Aims We previously reported that an increase of lymphocytes in peritoneal dialysis (PD) effluent was correlated with risk of encapsulating peritoneal sclerosis (EPS). In the present study, we analyzed subsets of lymphocytes in PD effluent by flow cytometry and evaluated their changes every six month to elucidate the etiological background of peritoneal dysfunction. Method We enrolled patients who started PD between 2006 and 2017, and of whom the data for PET and flow cytometric analysis was available at least for three consecutive times with an interval of six months. We excluded the patients who experienced PD peritonitis during the observation period. Consequently, the levels and changes of lymphocyte subset, such as CD4+/CD8+ naïve T cell (CCR7+/CD45RA+), CD4+/CD8+ central memory T cell (CCR7+/CD45RA-), CD4+/CD8+ effector memory T cell (CCR7-/CD45RA-), CD4+/CD8+ terminally differentiated effector memory T cell (CCR7-/CD45RA+), D/P creatinine ratio, FSC ratio of mesothelial cells and lymphocytes (a possible indicator for mesothelial cell size) were analysed in 23 patients over one year. Results We evaluated whether the observed variables on the first evaluation (six months after initiation of PD) affected the changes of D/P creatinine and FSC ratio over one year by a simple linear regression analysis. In the examined variables, only a fraction of CD8+ central memory T cell was significantly correlated with the change rate of D/P creatinine ratio (β=1.47, p=0.001, adjusted R2=0.379). We also evaluated whether the change rate of observed variables was correlated with the change rate of D/P creatinine and FSC ratio by a simple linear regression analysis. A fraction of CD8+ naïve T cell or CD8+ central memory cell was negatively correlated with the change rate of D/P creatinine ratio (naïve T cell; β=-0.058, p=0.022, adjusted R2=0.188, central memory T cell; β=-0.096, p=0.046, adjusted R2=0.137). The change rate of CD8+ effector memory T cell was not significantly correlated with the change rate of D/P creatinine ratio (β=0.172, p=0.096, adjusted R2=0.085). However, the change rate of D/P creatinine ratio tends to be higher in accordance with the increased change rate of CD8+ effector memory T cell by One way ANOVA, where the change rate was divided into three groups in descending order (p=0.0796) (Fig.1). Besides, the change rate of CD8+ effector memory T cell tends to be higher in accordance with the increased fraction of CD8+ central memory T cell at the first evaluation by Kruskall-Wallis test, where the change rate was divided into three groups in descending order (p=0.169) (Fig.2). Conclusion A decrease in the fraction of CD8+ naïve or central memory T cell was significantly correlated with the increase of D/P creatinine ratio. An Increase in the fraction of CD8+ effector memory T cell was also possibly correlated with the increase of D/P creatinine ratio, although it was not statistically significant (p=0.096). An initial fraction of CD8+ central memory T cell was significantly correlated with the change rate of D/P creatinine ratio. From these results, central memory T cells and naïve T cells at an initial stage may be transformed into effector memory T cells by repeated exposure to unknown antigens derived from PD solution and these effector memory T cells may damage the peritoneum to increase D/P creatinine ratio. An initial higher fraction of CD8+ central memory T cell suggested an acceleration in the transformation into CD8+ effector memory T cell.

Encapsulating Peritoneal Sclerosis (EPS): Analysis of Current Knowledge in the Literature and Observation of a Suspect Case

Among the medical complications of long-lasting Peritoneal Dialysis (PD) a particular pathology has been observed, the so-called Encapsulating Peritoneal Sclerosis (EPS). The main properties of the pathological process of EPS is represented by proliferative fibrosis and sclerosis of the peritoneum, which lead to the formation of the typical "cocoon" and obstruction. Since glucose, Advanced Glycation End products (AGEs), and Glucose Degradation Products (GDPs), are responsible for peritoneum fibrosis and sclerosis, biocompatible peritoneal dialysis solutions are recommended, with reduced quantities of AGEs and GDPs. Furthermore, careful monitoring of the patients is very important, especially after 5-8 years of PD. We performed an overview of the current literature available and discuss a “suspect” case of a 59-year-old male patient who underwent a single kidney transplant after a period of 8 year of peritoneal dialysis with numerous sub occlusive episodes, some of them required hospitalizations in a period between 2011 and 2019. Patient underwent to several radiologic exams for a suspect of a cocoon syndrome.

Long-term outcomes in patients with encapsulating peritoneal sclerosis managed with nutritional support.

Little is known about long-term survivors with encapsulating peritoneal sclerosis (EPS). Published literature focuses on patients managed surgically. We describe our experience of the long-term outcomes in patients with EPS conservatively managed with nutritional support alone. This is a single-centre retrospective observational study of patients who had survived for ≥5 years since diagnosis. EPS survivors were invited for review of symptoms, nutritional assessment and evaluation of quality of life. Radiological progression was assessed based on serial computed tomography (CT) scores for each patient. A total of 23 patients with a diagnosis of EPS for at least 5 years were identified, with 18 patients alive at the time of the study. Of these 18 patients, 10 patients transferred to haemodialysis (HD) and 8 patients received kidney transplants. Commonest symptoms were nausea (91%) and vomiting (73%). Mean body mass index for patients was within the ideal and healthy range, with only 11% suffering from continued weight loss. In all, 70% EPS survivors on HD received nutritional support compared to 15% of those with transplants; 17% required ongoing parenteral nutrition. Of the 11 patients with serial CT scans at least 4 years apart, 10 had an increase in radiological score for EPS but with no apparent correlation to clinical outcomes. There were no significant differences in the reported quality of life between EPS survivors on HD and those transplanted, with self-rated health status equivalent to that reported for the general end-stage kidney disease (ESKD) population. Long-term survival following EPS managed conservatively with nutritional support is feasible, with the majority no longer requiring nutritional support and having a quality of life similar to other patients with ESKD.

Proposal of peritoneal biopsy procedures for patients undergoing peritoneal dialysis

Prolonged peritoneal dialysis (PD) is responsible for progressive morphological changes such as deterioration of the peritoneal membrane. These changes increase the risk of encapsulating peritoneal sclerosis (EPS). Histological assessments of peritoneal membrane biopsy samples are fundamental for the evaluation of the peritoneal damage caused by PD. For evaluating serial morphological changes induced in the peritoneum by PD, we recommend to perform peritoneal biopsy examinations not only after the cessation of PD but also before performing PD. At the time of PD catheter insertion, the parietal peritoneum (1.5 × 1.5 cm) and rectus abdominal muscle posterior sheath is sampled at a point 3 cm below the PD catheter insertion site. Furthermore, at the time of PD catheter removal, the parietal peritoneum is sampled at a point 3 cm apart from the PD catheter insertion site to avoid artifacts. The peritoneum should be evaluated to detect mesothelial cell denudation, acellular sclerotic changes and thickness of the submesothelial connective tissue, vasculopathy of the post-capillary venules, vascular angiogenesis, and new encapsulating membrane. The method presented herein allows the minimization of surgical invasiveness and artifacts of the specimens and safe performance of peritoneal biopsy examinations. Morphological evaluation of the peritoneum involving an appropriate biopsy strategy, in conjunction with functional markers of deterioration, such as peritoneal permeability or cytokine levels, is a useful approach for examining peritoneal damage and predicting the prognosis of PD patients, especially the onset of EPS.

A Successful Treatment of Encapsulating Peritoneal Sclerosis in an Adolescent Boy on Long-term Peritoneal Dialysis: A Case Report

Encapsulating peritoneal sclerosis (EPS) is a rare life-threatening complication associated with peritoneal dialysis (PD). EPS is characterized by progressive fibrosis and sclerosis of the peritoneum, with the formation of a membrane and tethering of loops of the small intestine resulting in intestinal obstruction. It is very rare in children. We present a case of a 16-year-old adolescent boy who developed EPS seven years after being placed on continuous ambulatory peritoneal dialysis (CAPD) complicated by several episodes of bacterial peritonitis. The diagnosis was based on clinical, radiological, intraoperative and histopathological findings. The patient was successfully treated with surgical enterolysis. During a 7-year follow-up, there have been no further episodes of small bowel obstruction documented. He still continues to be on regular hemodialysis and is awaiting a deceased donor kidney transplant. EPS is a long-term complication of peritoneal dialysis and is typically seen in adults. Rare cases may be seen in the pediatric population and require an appropriate surgical approach that is effective and lifesaving for these patients.

Outcomes of the surgical management of encapsulating peritoneal sclerosis: A case series from a single center in Korea.

BackgroundEncapsulating peritoneal sclerosis (EPS) is a rare but near-fatal complication of peritoneal dialysis (PD). Despite the high mortality rate of EPS, the surgical treatment strategy of severe EPS is yet to be established.MethodsWe retrospectively analyzed outcomes of patients with EPS who underwent enterolysis for intractable EPS at Seoul National University Hospital between 2001 and 2018. EPS was diagnosed based on the clinical symptoms and radiological findings of abdominal computed tomography (CT). CT scans were scored according to an EPS scoring system that assessed peritoneal thickening and calcification as well as bowel thickening, tethering, loculation, and dilatation.ResultsThirteen patients (nine males and four females; age, 48 [29–63] years) underwent enterolysis for severe EPS. PD duration (11 [6–21] years) was not associated with survival. Two patients were newly diagnosed with EPS following kidney transplantation. Five patients died of infectious complications immediately after the surgery. Eight patients survived after the first surgery; however, five of them underwent reoperation but died of persistent infection, fistula formation, or adhesive bowel obstruction. Four young (< 60 years) male patients with relatively low CT scan scores (< 13) survived for > 2 years after the first surgery. Median survival duration from EPS diagnosis was 22 (1.3–184) months and that from the first surgery was 9 (0.3–153) months.ConclusionThe high mortality rate of EPS suggests the importance of appropriate surgical intervention in young symptomatic male EPS patients with relatively low CT scan scores.

Clinical risk factors and outcomes of massive ascites accumulation after discontinuation of peritoneal dialysis

Background Encapsulating peritoneal sclerosis (EPS) is a serious complication of peritoneal dialysis (PD), with high morbidity and mortality that requires an early diagnosis for effective treatment. PD withdrawal and bacterial peritonitis are important triggers for the onset of EPS. However, few studies have focused on cases of PD withdrawal without a clinical diagnosis of peritonitis, cirrhosis, or carcinomatosis. We aimed to compare the clinical characteristics and computed tomography (CT) images of patients with or without ascites in such situations and assess clinical outcomes in terms of mortality. Methods Our retrospective review included 78 patients who withdraw PD between January 2000 and December 2017. Results Ten patients had ascites, and 68 did not have a significant intra-abdominal collection. The ascites group had a significantly longer PD duration (months; 134.41 [range, 35.43–181.80] vs. 32.42 [733–183.47], p < 0.001) and higher peritoneal membrane transport status based on the dialysate-to-plasma ratios of creatinine (0.78 ± 0.08 vs. 0.68 ± 0.11, p = 0.009) and glucose (0.27 ± 0.07 vs. 0.636 ± 0.08, p = 0.001) than the control group. CT parameters, including peritoneal calcification, thickness, bowel tethering, or bowel dilatation, were not all present in each patient with ascites and EPS. During the 12-month study period, the ascites group had a higher risk for developing EPS (70% vs. 0%, p < 0.001) and a higher 12-month all-cause mortality (30% vs. 0%, p = 0.002). Conclusions Ascites accumulation was not rare after PD discontinuation. A longer PD duration and high peritoneal membrane transport status could predict subsequent ascites accumulation. Furthermore, patients with ascites were at a higher risk of EPS.

Inflammatory macrophages switch to CCL17-expressing phenotype and promote peritoneal fibrosis.

Peritoneal fibrosis remains a problem in kidney failure patients treated with peritoneal dialysis. Severe peritoneal fibrosis with encapsulation or encapsulating peritoneal sclerosis is devastating and life-threatening. Although submesothelial fibroblasts as the major precursor of scar-producing myofibroblasts in animal models and M2 macrophage (Mϕ)-derived chemokines in peritoneal effluents of patients before diagnosis of encapsulating peritoneal sclerosis have been identified, attenuation of peritoneal fibrosis is an unmet medical need partly because the mechanism for cross talk between Mϕs and fibroblasts remains unclear. We use a sodium hypochlorite-induced mouse model akin to clinical encapsulated peritoneal sclerosis to study how the peritoneal Mϕs activate fibroblasts and fibrosis. Sodium hypochlorite induces the disappearance of CD11bhigh F4/80high resident Mϕs but accumulation of CD11bint F4/80int inflammatory Mϕs (InfMϕs) through recruiting blood monocytes and activating local cell proliferation. InfMϕs switch to express chemokine (C-C motif) ligand 17 (CCL17), CCL22, and arginase-1 from day 2 after hypochlorite injury. More than 75% of InfMϕs undergo genetic recombination by Csf1r-driven Cre recombinase, providing the possibility to reduce myofibroblasts and fibrosis by diphtheria toxin-induced Mϕ ablation from day 2 after injury. Furthermore, administration of antibody against CCL17 can reduce Mϕs, myofibroblasts, fibrosis, and improve peritoneal function after injury. Mechanistically, CCL17 stimulates migration and collagen production of submesothelial fibroblasts in culture. By breeding mice that are induced to express red fluorescent protein in Mϕs and green fluorescence protein (GFP) in Col1a1-expressing cells, we confirmed that Mϕs do not produce collagen in peritoneum before and after injury. However, small numbers of fibrocytes are found in fibrotic peritoneum of chimeric mice with bone marrow from Col1a1-GFP reporter mice, but they do not contribute to myofibroblasts. These data demonstrate that InfMϕs switch to pro-fibrotic phenotype and activate peritoneal fibroblasts through CCL17 after injury. CCL17 blockade in patients with peritoneal fibrosis may provide a novel therapy. © 2019 Pathological Society of Great Britain and Ireland. Published by John Wiley & Sons, Ltd.