Encapsulating Peritoneal Sclerosis: Pathophysiology and Current Treatment Options

Abstract

Encapsulating peritoneal sclerosis (EPS) is a life-threatening complication of long-term peritoneal dialysis (PD), which may even occur after patients have switched to hemodialysis (HD) or undergone kidney transplantation. The incidence of EPS varies across the globe and increases with PD vintage. Causative factors are the chronic exposure to bioincompatible PD solutions, which cause long-term modifications of the peritoneum, a high peritoneal transporter status involving high glucose concentrations, peritonitis episodes, and smoldering peritoneal inflammation. Additional potential causes are predisposing genetic factors and some medications. Clinical symptoms comprise signs of intestinal obstruction and a high peritoneal transporter status with incipient ultrafiltration failure. In radiological, macro-, and microscopic studies, a massively fibrotic and calcified peritoneum enclosed the intestine and parietal wall in such cases. Empirical treatments commonly used are corticosteroids and tamoxifen, which has fibrinolytic properties. Immunosuppressants like azathioprine, mycophenolate mofetil, or mTOR inhibitors may also help with reducing inflammation, fibrin deposition, and collagen synthesis and maturation. In animal studies, N-acetylcysteine, colchicine, rosiglitazone, thalidomide, and renin-angiotensin system (RAS) inhibitors yielded promising results. Surgical treatment has mainly been performed in severe cases of intestinal obstruction, with varying results. Mortality rates are still 25–55% in adults and about 14% in children. To reduce the incidence of EPS and improve the outcome of this devastating complication of chronic PD, vigorous consideration of the risk factors, early diagnosis, and timely discontinuation of PD and therapeutic interventions are mandatory, even though these are merely based on empirical evidence.

Encapsulating Peritoneal Sclerosis: A Case Report and Literature Review

“…Our patient was on PD for more than 5 years, which likely resulted in long-term changes to his peritoneal membrane (i.e., first hit). Moreover, he was exposed to multiple potential inflammatory stimuli, which included renal transplant, removal of the PD catheter, and immunosuppressive medications, all of which may have potentially served as the second hit, leading to the development of EPS [ 7 ].…”

Parietal fibrinous peritonitis in cattle: A literature review

“…PFP has some similarities with sclerosing encapsulating peritonitis (SEP), a complication of peritoneal dialysis and abdominal surgery described in humans, 21-23 dogs, 24 and cats 25 . The main risk factors for SEP are repeated laparoscopic interventions, unphysiological composition of dialysis fluids and disinfectants, perturbing the normal peritoneal histology, and resorption capacities 21,23,26 . This finally leads to the accumulation of a modified transudate containing neutrophils, lymphocytes, and monocytes in a thickened parietal peritoneum 24,27 ; the process can be contaminated.…”

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

“…It is very uncommon in pediatric population and there are only very few reported and well documented cases of EPS in children (Hoshii et al, 2000;Ekim et al, 2005;Tan et al, 2005). However, the reported outcome of pediatric EPS cases is significantly better with a lower mortality and morbidity compared with adult patients (Shroff et al, 2013;Jagirdar et al, 2019).…”

“…EPS has also been reported after organ transplantation suggesting that transplantation may be an additional trigger for its onset (Fieren et al, 2007). Etiological factors that cause EPS in cases of long-term PD are insufficiently clear but there is evidence that high glucose concentration and acidic pH in dialysis fluid, as well as the heat sterilization of PD fluids, cause peritoneal damage and formation of deposits comprising glucose degradation products (GDP) and advanced glycation end products (AGEs) ( Jorres et al, 1992;Fieren et al, 2007;Schmitt et al, 2007;Jagirdar et al, 2019). This may be aggravated by recurrent attacks of peritonitis.…”