Peritoneal sclerosis

Encapsulating peritoneal sclerosis is a rare but devastating complication of long-term peritoneal dialysis with a high mortality rate. The incidence is between 0.5 and 2.5%, decreasing with time. PSE is defined as a clinical syndrome with signs of gastrointestinal obstruction, inflammation parameters, radiological and macroscopic changes. The duration of treatment and the cessation of peritoneal dialysis are the main risks. About 75% occured in patients on hemodialysis or after kidney transplantation. Morphological alterations are disappearance of mesothelial layer, submesothelial fibrosis, interstitial sclerosis and vasculopathy...

BACKGROUND: Peritoneal injury is an important cause of technical failure of long-term peritoneal dialysis (PD). Encapsulating peritoneal sclerosis (EPS) is a severe complication of long-term PD with potentially life threatening consequences. CD147 is a glycoprotein with diverse functions including modulation of extracellular matrix via induction of matrix metalloproteinases, cell adhesion, and regulation of immune reactions. We hypothesized that CD 147 plays a role in the peritoneal cavity...

OBJECTIVE: CXCL6, a chemokine with proangiogenic property, is reported to be involved in vasculopathy associated with systemic sclerosis (SSc). We investigated the contribution of CXCL6 to SSc development by focusing on the association of friend leukemia virus integration 1 (Fli1) deficiency, a potential predisposing factor of SSc, with CXCL6 expression and clinical correlation of serum CXCL6 levels. METHODS: mRNA levels of target genes and the binding of Fli1 to the CXCL6 promoter were evaluated by quantitative reverse transcription-PCR and chromatin immunoprecipitation, respectively...

Peritoneal membrane failure (PMF) and, ultimately, encapsulating peritoneal sclerosis (EPS) are the most serious peritoneal dialysis (PD) complications. Combining clinical and peritoneal transport data with the measurement of molecular biomarkers, such as the chemokine CCL18, would improve the complex diagnosis and management of PMF. We measured CCL18 levels in 43 patients' effluent and serum at baseline and after 1, 2, and 3 years of PD treatment by retrospective longitudinal study, and evaluated their association with PMF/EPS development and peritoneal risk factors...

BACKGROUND: Encapsulating peritoneal sclerosis (EPS) is a complication of peritoneal dialysis (PD) with a low prevalence but high mortality. It is characterized by peritoneal inflammation and fibrosis with subsequent development of intestinal encapsulation. It is associated with a long lapse on PD, frequent episodes of peritonitis, high glucose solution use, and high peritoneal transport status. AIM: To report the clinical features of patients on PD, who developed EPS...

The Dutch Encapsulating Peritoneal Sclerosis (EPS) Registry was started in 2009. Cases were identified by contacting all Dutch nephrologists twice yearly. The predefined criteria for EPS allowed for inclusion of patients with diagnosed and suspected EPS. Cases registered between January 2009 and January 2015 were analyzed with follow-up until September 2015. Fifty-three EPS cases were identified, of which 28.3% were post-transplantation EPS cases. Fourteen patients were initially categorized as suspected EPS, of whom 13 developed EPS...

Encapsulating peritoneal sclerosis (EPS) is a rare cause of intestinal obstruction by a thick fibrous membrane wrapping around the small intestine. It is a possible complication after liver transplantation (LT) that can be fatal. This report describes 2 cases of EPS after LT that were successfully treated with surgery, corticosteroids, tamoxifen, and mammalian target of rapamycin inhibitor. After treatment in both cases, the patients were able to start oral feeding and have been symptom free for more than 1 year...

Encapsulating peritoneal sclerosis (EPS) is a rare but serious complication of peritoneal dialysis. In this review, we describe the clinical picture and histologic changes to the peritoneal membrane that are associated with EPS and provide an update on current diagnosis and management. We also discuss the recent studies that have suggested that the use of more biocompatible solutions containing lower concentrations of glucose degradation product that often are pH neutral in combination with a change in clinical practice (reducing glucose exposure and monitoring peritoneal membrane function) might ameliorate peritoneal degeneration, reduce the incidence of EPS, and minimize the severity of the disease...

Autoimmunity and inflammation are controlled in part by regulatory B (Breg) cells, including the recently identified IL-10-competent B10 cell subset that represents 1%-3% of mouse spleen B cells. In this study, the influence of irradiation on Breg/B10 cell generation and IL-10 production mediated by TLR9 signaling pathways was investigated. Spleen and peritoneal cavity Breg/B10 cell frequencies were significantly expanded three weeks after sub-total body irradiation (sub-TBI, 5Gy or 10Gy) in adult male wild type (WT) C57BL/6(B6) mice but not in TLR9(-/-) mice...

BACKGROUND AND AIMS: Encapsulating peritoneal sclerosis (EPS) is an uncommon but severe complication of peritoneal dialysis (PD). A reliable screening tool to identify patients at risk of developing or not EPS is currently not available. We aimed to evaluate whether the reduction in dialysate sodium concentration (sodium sieving) at 60 min (ΔDNa60), during a peritoneal equilibration test with 3.86% glucose concentration (3.86%-PET) was able to early rule out patients who will not develop EPS...

Multiple sclerosis is a chronic inflammatory and autoimmune disease of the central nervous system that affects more than 2.5 million people worldwide. Experimental autoimmune encephalomyelitis is a murine autoimmune disease used to study multiple sclerosis. Parthenolide, a natural sesquiterpene lactone found in Tanacetum parthenium L., is known for its strong anti-inflammatory activity. Herein, we have investigated the in vitro immunomodulatory effects of parthenolide on cytokine production and nitric oxide in cultured cells from myelin oligodendrocyte glycoprotein 35-55 amino acid peptide mice...

BACKGROUND: Encapsulating Peritoneal Sclerosis (EPS) describes a variety of diseases that are frequently confused with different names and different etiopathogeneses. The aim of this article is to report personal experience of focusing on correct classification and the status of current diagnosis and treatment. METHODS: A retrospective analysis was performed. Age, sex, ethnic origin, past medical history, symptoms and their duration, radiological tools and signs, laboratory tests, preoperative diagnosis, surgical approach, intraoperative findings, pathological findings, hospital stay, morbidity and mortality were studied...

Encapsulating peritoneal sclerosis (EPS) is an infrequent but serious complication of long-term peritoneal dialysis with 50% mortality and lack consensus on treatment. The pathogenesis of EPS is ill understood, and diagnosis lacks specificity and relies on clinical, radiographic and microscopic evaluation. Since there is no general agreement on managing EPS, we report a case treated successfully with surgery followed by immunosuppressive therapy with tamoxifen and steroids. He later successfully underwent deceased donor renal transplantation...

INTRODUCTION: Encapsulating peritoneal sclerosis (EPS) is a rare but devastating complication of peritoneal dialysis (PD). Tamoxifen has been generally well-tolerated, even without randomized controlled trials. CASE PRESENTATION: Herein, we report a case of a patient undergoing 12 years of PD who developed EPS and calciphylaxis simultaneously. We also provide a comprehensive discussion about the association between EPS and calciphylaxis. Moreover, although tamoxifen is used in EPS due to its inhibition of fibroblast-transforming growth factor beta (TGFβ) production, it may worsen the calciphylaxis due to a hypercoagulable state...

Peritoneal dialysis (PD) offers the healthiest way for starting renal replacement therapy (RRT) in End Stage Renal Disease patients, however exposes long-term PD patients to a dangerous complication named encapsulating peritoneal sclerosis (EPS). In this study, we searched for possible risk factors of EPS. Data were collected from two PD centers covering period 1995-2012 and comprised 464 patients. Control group defined as PD patients stayed on PD >42 month (n = 122), and case group was 12 confirmed EPS patients...

OBJECTIVE: Association between cancer and systemic sclerosis (SSc) has been described. However, paraneoplastic SSc is not well known. The aim of this article is to describe cases of paraneoplastic systemic sclerosis and to compare them to other cases of the literature, to find characteristics that can make suspect a paraneoplastic mechanism when SSc is diagnosed. METHODS: We retrospectively analyzed patients, in our department who, over the last 15 years, presented with Raynaud's phenomenon with a diagnosis of SSc (including cancer during the period of SSc)...

Encapsulating peritoneal sclerosis (EPS) is a rare complication of peritoneal dialysis that carries a high morbidity and mortality. Although its pathogenesis is still not clear, the "two hit theory" suggests that long term deterioration of the peritoneum combined with intraperitoneal inflammation is needed in the pathogenesis of EPS. To date, there is no proven effective therapy with an absence of randomized controlled trials. Individual case reports and small case series have reported on the use of tamoxifen and corticosteroids for medical management of EPS...

After 20 years of peritoneal dialysis in Iran, we have encountered with several cases of encapsulating peritoneal sclerosis (EPS) in past few years. Many of these cases remained undiagnosed until advanced stages due to lack of suspicion. In centers with more experience about EPS, mortality has decreased by early diagnostic interventions. Peritoneal dialysis nurses may not be aware of EPS and radiologists are usually not familiar with EPS, either. To increase knowledge about this condition, we decided to present this review article with the case study of one of the 1st patients with EPS at our center...

Surgical enterolysis is the final option for patients with encapsulating peritoneal sclerosis (EPS). However, EPS is sometimes refractory to surgical enterolysis. This is the first report of successful use of percutaneous endoscopic gastrostomy with jejunal extension (PEG-J) in a patient with EPS that was refractory to surgical enterolysis. We propose that sustained drainage of digestive juices by PEG-J, along with central venous nourishment at home, can be a treatment option for patients with EPS that cannot be treated with radical measures...

Pneumatosis cystoides intestinalis (PCI) is a rare gastrointestinal complication of systemic sclerosis (SSc) characterized by intramural accumulation of gas within thin-walled cysts. We report the case of an 82-year-old female patient with pneumoperitoneum due to PCI associated with SSc and review the features of the 39 Japanese cases. The median patient age was 57 years (range 24-83 years) and the male/female ratio was 1 : 12. In the recent decade, 14 out of 15 cases (93.3%) evaluated with CT scans were diagnosed with PCI...