This disease has a poor prognosis and high mortality rate, and peritoneal dialysis patients need to pay attention!

Encapsulated peritoneal sclerosis (EPS) is a rare and serious complication that occurs in patients with long-term peritoneal dialysis. Once it occurs, the prognosis is poor and the mortality rate is high. Due to its rarity and the lack of detailed summary and analysis of imaging manifestations, it is easy to be missed or misdiagnosed in clinical practice.

The occurrence of EPS is mainly related to long-term peritoneal dialysis. The toxins in the patient's own metabolism, glucose degradation products, peritoneal dialysis fluid, inflammation and other factors can cause inflammatory reactions in the peritoneum, leading to damage. The decrease in plasminogen causes fibrin accumulation, and the increased production of inflammatory factors and growth factors leads to epithelial-mesenchymal cell differentiation, activation of myofibroblasts, and deposition of extracellular matrix, resulting in simple sclerosis of the peritoneum, which is often manifested as loss of integrity of the peritoneal mesothelium, interstitial fibrosis, vascular lesions and hyperplasia ( 1 blow ). The clinical manifestations are generally not significant.

At this time, if two additional blows are combined on the basis of the above-mentioned chronic peritoneal injury, including sudden termination of peritoneal dialysis, organ transplantation, repeated peritonitis, and the use of profibrotic drugs, further peritoneal fibrosis and adhesion can occur, eventually leading to intestinal encapsulation and the occurrence of EPS [1].

The characteristic manifestations of EPS are recurrent small bowel obstruction and peritoneal sclerosis. The UK EPS clinical practice guidelines propose two main criteria [2]

(1) Symptoms of obstructive intestinal obstruction occur with or without varying degrees of systemic inflammatory response.

(2) Imaging findings include peritoneal thickening, hardening, and calcification, and small intestine aggregation. In addition, recurrent bloody peritoneal effusions may occur, but this is not a characteristic manifestation of EPS.

According to the clinical and pathological characteristics of different stages, EPS can be divided into the following 4 stages:

Currently, there are no specific markers for the diagnosis of EPS. Imaging is one of the important bases for the diagnosis of EPS. Generally, EPS can only be diagnosed through laparotomy or laparoscopy. The characteristic changes of EPS in the peritoneum can be directly observed with the naked eye, but it is not commonly used in clinical practice. CT scans are often used in clinical practice. They can be completed in a short time and comprehensively and intuitively display the relevant changes in the peritoneum, intestinal wall and intestine. Common signs of EPS include: peritoneal thickening, peritoneal enhancement, peritoneal calcification, intestinal wall thickening, intestinal wall calcification, etc., and can show a variety of complications, such as intestinal adhesions, intestinal obstruction, ascites, etc. [3].

The treatment of EPS is relatively complicated, and appropriate treatment must be selected based on the patient's general condition and treatment conditions. Generally, it includes stopping peritoneal dialysis, providing nutritional support, using medication, and performing surgical operations. There are several points to note:

First, once EPS is diagnosed, peritoneal dialysis should be stopped and switched to hemodialysis.

Second, EPS patients are very prone to malnutrition, so nutritional support is very important.

Third, drug treatment mainly includes glucocorticoids and immunosuppressants. Glucocorticoids can inhibit inflammatory response, reduce exudation and cellulose formation.

Fourth, intestinal adhesion lysis can also effectively relieve EPS[4].

The specific treatment process is as follows:

**1. **Closely monitor the patient's vital signs;

**2. **Enteral or total parenteral nutrition support;

**3. **If the symptoms of intestinal obstruction do not improve after 3 to 6 months of total parenteral nutrition support, surgical treatment may be considered;

**4. **The efficacy of intraperitoneal lavage is unclear. It may increase the risk of infection in immunocompromised patients and should be used with caution.

**5. **Try tamoxifen 20-40 mg/d (no randomized controlled trial evidence);

**6. **If the patient has a short survival period (such as combined with malignant tumors, etc.), maintenance peritoneal dialysis can be performed;

**7. **Discontinuation of PD may trigger EPS, and treatment should be considered comprehensively based on age and comorbidities;

**8. **For established sclerosis, hormone therapy is ineffective and should be switched to hemodialysis as soon as possible.

summary

EPS is a potential but rare serious complication in long-term peritoneal dialysis treatment. We must attach great importance to it and ensure early detection, timely diagnosis and timely intervention and treatment.

References:

[1] Chen Yiwen, Yu Yusheng. EPS syndrome[J]. Journal of Nephrology and Dialysis and Kidney Transplantation, 2011, 20(05):481-486.

[2] Garosi G, Mancianti N, Corciulo R, et al. Encapsulating peritoneal sclerosis. J Nephrol. 2013 Nov-Dec;26 Suppl 21:177-87.

[3] Zhang Fan, Guan Jian, Lin Zhi, et al. CT diagnosis and differentiation of EPS after peritoneal dialysis[J]. Radiology Practice, 2018, 33(05):468-472.

[4] Wang Yina, Zhao Huiping. Research progress of EPS[J]. Chinese Journal of Blood Purification, 2017, 16(07): 433-436.