Last updated:
1/7/2025
Years published: 2025
NORD gratefully acknowledges Lawrence Slapcoff, MD and Sharon J. Nessim, MD, MSc, Division of Nephrology, Jewish General Hospital, McGill University, Montreal, QC, Canada, for the preparation of this report.
Encapsulating peritoneal sclerosis (EPS) is a rare but serious condition. It mainly affects people who have been treated with long-term peritoneal dialysis (PD), which is a therapy for patients with end-stage kidney disease.1 In EPS, the lining around the bowels also known as the peritoneal membrane, becomes thickened and hard. This lining tightly wraps around the intestines like a cocoon, which can lead to significant pain and severe problems with digestion. Common symptoms include abdominal pain, nausea, lack of appetite, weight loss and bowel obstruction.2 The exact cause of EPS is unknown. It is believed to be due to a “two-hit hypothesis” that involves multiple injuries to the peritoneal membrane over a long period of time.3,4 The main risk factor is thought to be a duration of peritoneal dialysis greater than 5 years.5,6 The primary treatment options for EPS include medication and nutritional support. The two most common medications used are corticosteroids and tamoxifen.4,7 Surgery can also be performed, particularly in severe cases by surgeons with high levels of expertise.8-10
The presentation of EPS can vary between individuals. The most common reported signs and symptoms include:1,2,11
Encapsulating peritoneal sclerosis is usually seen in people who have been on long-term peritoneal dialysis therapy. It occurs when the peritoneal membrane surrounding the bowels becomes thickened and hard. This can create an “abdominal cocoon” around the bowels which causes abdominal pain and makes it difficult to digest food and pass bowel movements. This unfortunately can lead to profound malnutrition and recurrent infections.
Peritoneal dialysis is a treatment for kidney failure, which happens when the kidneys can’t clean (filter) the blood properly. In this process, a special cleaning fluid is put into the belly (abdomen) through a soft tube. The lining inside the abdomen, called the peritoneum, works like a natural filter to remove waste from the blood. After some time, the fluid filled with waste flows out from the abdomen through the tube and is safely disposed of.
The main risk factors for encapsulating peritoneal sclerosis are thought to be:5,12-14
Organ transplantation is often listed as a cause of EPS, although it is not clear if it is the transplantation process or the medications provided to prevent transplant rejection that are driving the development of the disease.15,16 Transplantation should not be avoided due to risk of EPS.
Encapsulating peritoneal sclerosis (EPS) can sometimes develop in people who are not undergoing peritoneal dialysis. It may be linked to other health conditions, including autoimmune diseases, sarcoidosis, cancers in the abdomen or peritoneum, long-term fluid buildup in the abdomen (ascites), chemotherapy delivered directly into the abdomen, exposure to certain particles or disinfectants in the abdomen, abdominal surgery, endometriosis, infections in the peritoneum (like tuberculosis) and even the use of some medications, such as beta-blockers.3
The International Society for Peritoneal Dialysis (ISPD) recognizes EPS as a rare complication of PD. While the duration of PD is a significant risk factor, the ISPD does not recommend routinely discontinuing PD prematurely. Instead, they advise evaluating each person individually, considering the following factors:
This approach ensures that decisions regarding the duration of PD therapy are tailored to each patient’s unique circumstances, balancing the potential risks of EPS against the benefits of continued PD treatment. 6
EPS occurs most often in people who have been on peritoneal dialysis for more than 5 years, and the risk increases the longer someone remains on PD. The prevalence is not precisely defined, but current research suggests it ranges from 0.4% to 8.9%, which means out of every 1,000 people on peritoneal dialysis, approximately 4 to 89 people might develop this condition.6
Encouragingly, several countries have reported a decrease in the occurrence of EPS.18,19
The diagnosis of EPS is often delayed due to its rarity and non-specific symptoms. It is confirmed by the clinical presentation described above, with specific radiological findings (CT scan showing peritoneal thickening, calcification, bowel tethering and/or encapsulation).17 Although not required for diagnosis, the disease can be confirmed by direct visualization of the intestines during surgery showing encasing of the bowels by thick peritoneum, also known as the “abdominal cocoon.” 6,18
Treatment for EPS is not yet standardized and can be divided into three main categories: medication, nutritional support and surgery.4
Medication
The primary medications used in the treatment of EPS include:
Other drugs such as mycophenolate, azathioprine, cyclosporine and rapamycin have shown positive results in a limited number of patients, though they have not been well studied in larger populations. Ongoing research is investigating new therapeutic options.4,20,21
Nutritional Support
Nutritional support is critical for patients with EPS. Some individuals may require total parenteral nutrition (TPN), where nutrients are delivered intravenously via a central line, which is a large catheter that can often be used at home.
Surgery
In severe cases, surgery may be necessary. Enterolysis, a procedure to release adhesions (scar tissue), is occasionally performed. Due to the complexity of EPS, surgery should only be carried out by surgeons with specific expertise in this field.10
Currently, there are no reliable ways to fully prevent EPS. For people who are on peritoneal dialysis (PD), some strategies may help reduce risks:22
The main risk factor for EPS is the duration of PD, especially after five years. While some suggest setting a time limit for PD, this is not standard practice. Most people on PD do not develop EPS, and stopping PD early might lead to complications with transitioning to hemodialysis (HD).22
However, in cases where the peritoneum shows early signs of strain, such as repeated infections or reduced fluid removal, resting the peritoneum by temporarily stopping PD and switching to HD can help relieve symptoms. During this time, the peritoneum may be flushed twice a week to prevent adhesion. The rest period usually lasts 4–12 weeks. As commented, this approach is controversial, because symptoms of EPS might worsen after stopping PD. 22
Information on current clinical trials is posted on the Internet at www.clinicaltrials.gov. All studies receiving U.S. government funding, and some supported by private industry, are posted on this government web site.
For information about clinical trials being conducted at the NIH Clinical Center in Bethesda, MD, contact the NIH Patient Recruitment Office:
Tollfree: (800) 411-1222
TTY: (866) 411-1010
Email: [email protected]
Some current clinical trials also are posted on the following page on the NORD website:
https://rarediseases.org/living-with-a-rare-disease/find-clinical-trials/
For information about clinical trials sponsored by private sources, contact: www.centerwatch.com
For information about clinical trials conducted in Europe, contact: https://www.clinicaltrialsregister.eu/
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