What is Encapsulating Peritoneal Sclerosis?
Encapsulating peritoneal sclerosis (EPS) is a rarely occurring condition where a thick, fibrous tissue membrane can wrap around parts of the small intestine. This can cause repeated blockages in the small intestine and lead to poor nutrition. The condition is most commonly associated with long-term peritoneal dialysis. Peritoneal dialysis is a treatment for kidney failure that involves circulating a clean solution through the stomach area or “peritoneum” to help remove waste products from the blood; over time, this can lead to chronic inflammation and hardening of the area.
Frequent severe infections in the peritoneum area or systemic inflammatory disorders (conditions that cause inflammation throughout the body) can increase a person’s likelihood of developing EPS. EPS can also occur as a potential late-appearing complication after a kidney transplant, even if the person does not have a systemic inflammation. However, in many cases, the cause of EPS is unknown or “idiopathic.” Because this condition is so rare and can severely affect health and well-being, diagnosis is often delayed.
What Causes Encapsulating Peritoneal Sclerosis?
Encapsulating peritoneal sclerosis (EPS) is a condition that’s not completely understood just yet. However, what we do know is that it’s often seen in patients undergoing long-term peritoneal dialysis—a treatment for advanced kidney disease. In peritoneal dialysis, a fluid containing substances like glucose is used, which can sometimes cause inflammation in the peritoneum (a thin tissue lining the inside of your abdomen). This inflammation can then lead to a higher chance of damage and buildup of a protein called fibrin.
Several fibrin buildups can create a thick capsule around parts of the small intestine, which can cause blockages and poor nutrition. There are various factors that can increase the risk of getting EPS, especially for those on peritoneal dialysis. These include how long you’ve been on peritoneal dialysis, getting a kidney transplant, suffering from peritonitis (an inflammation of the peritoneum), being young, having a low pH level, high glucose in the dialysis fluid, unsuccessful fluid removal during dialysis, and being exposed to a disinfectant known as chlorhexidine.
There are other factors not directly linked to peritoneal dialysis that might lead to EPS. These include autoimmune diseases, sarcoidosis (a disease that causes inflammation in different parts of your body), gut and abdominal cancers, chronic build-up of fluid in the abdomen, chemotherapy in the abdomen, specific dialysis fluids such as acetate, having had an abdominal surgery, endometriosis (a condition where tissue similar to the lining of the uterus grows outside of it), intra-abdominal infections, particularly those caused by Staphylococcus aureus, Pseudomonas, and Mycobacterium tuberculosis and fungal species, and taking certain medications including calcineurin inhibitors (a type of immunosuppressant) and beta-blockers.
EPS risk is also increased after receiving a kidney transplant, usually shortly afterwards. This may be because of the increase in scarring tissue due to medications that suppress the immune system like calcineurin inhibitors. It could also be because of sudden stoppage of peritoneal dialysis.
Risk Factors and Frequency for Encapsulating Peritoneal Sclerosis
Encapsulating peritoneal sclerosis (EPS) is a condition that has been researched in several countries like Scotland, Australia, New Zealand, and Japan, but not much is known about its prevalence in the United States. In New Haven, Connecticut, a study of patients on peritoneal dialysis for five years or more found that 18.4% developed EPS. On the other hand, another study indicated that the EPS rate was 1.2% among 676 patients, but it increased to 15% after six years and 38% after nine years of peritoneal dialysis.
This increase in EPS cases among patients on peritoneal dialysis for five years or more mirrors findings in other countries. In a Scottish study involving 1238 adult patients on peritoneal dialysis between 2000 and 2007, the incidence of EPS started at 1.1% in the first year and steadily grew to 22.2% by the seventh year.
In Australia and New Zealand, a study of 7618 patients over 13 years showed that the EPS rate was 1.8% per 1000 patients, increasing over time with dialysis – 0.3% at three years, 0.8% at five years, and 3.9% at eight years. A Japanese study showed an even more dramatic increase: 0% at three years, 0.7% at five years, 2.1% at eight years, 5.9% at ten years, 5.8% at fifteen years, and finally, a whopping 17.2% for those who had received peritoneal dialysis for 15 years or more.
Overall, studies from different countries show that the prevalence of EPS can range from 0.4% to 8.9%, with some cases as high as 22.2%, especially after seven years or more of peritoneal dialysis.
Signs and Symptoms of Encapsulating Peritoneal Sclerosis
Encapsulating peritoneal sclerosis (EPS) is a condition that can be diagnosed based on a collection of symptoms and the timeline of symptom appearance. It generally evolves in stages, including an inflammatory stage, an encapsulating stage, and an ileus stage.
At the outset, during the inflammatory stage, the patient may experience symptoms such as fever, anemia, low albumin levels in blood, nausea, diarrhea, and an increase in C-reactive protein, a marker for inflammation in the body. These symptoms are connected with the start of bowel encapsulation. At this time, a physical check-up might not reveal anything unusual as the fibrotic capsule, which encloses the bowel, might still be too immature to stop intestinal movement.
As the condition progresses, the fibrotic capsule hardens and may cause symptoms resembling a bowel blockage, marking the ileus stage. Initial symptoms in this stage include nausea, vomiting, diarrhea, intermittent abdominal pain, anorexia, and loss of appetite.
While a physical examination might again show nothing unusual, some patients who are on peritoneal dialysis may observe their ascitic fluid or dialysis fluid becoming tinged with blood, especially after periods when the peritoneal dialysis cavity has been dry. As the ileus stage progresses, symptoms can further evolve into severe abdominal pain, continuous vomiting, malnutrition, constipation, and weight loss. By this point in the disease progression, a physical examination could show the presence of an abdominal mass, tenderness when the abdomen is pressed lightly or firmly, reduced bowel sounds, and possibly a rigid abdomen.
The timing of symptom onset also plays a crucial role in the diagnosis. It’s noteworthy that most patients (70 to 90%) are diagnosed with EPS after they’ve stopped peritoneal dialysis, and this could even happen as late as five years after they’ve stopped the treatment.
Testing for Encapsulating Peritoneal Sclerosis
If your doctor suspects that you may have a condition called encapsulating peritoneal sclerosis (EPS), they will likely recommend imaging tests for a definitive diagnosis, as there are no specific laboratory tests currently available for this condition. One frequently used imaging technique is a computed tomography scan, or CT scan. This method was shown in a study to correctly identify EPS with 100% sensitivity (ability to correctly identify the disease when it is indeed present) and 94% specificity (accuracy in ruling out the disease when it is not present). Important findings in a CT scan that can indicate EPS include changes in the peritoneum, a thin membrane inside the abdomen, such as unusual enhancement, thickening, and calcifications. Additionally, abnormalities of the bowel, such as thickening, widening, or changes in the distribution of digestive fluids, may be seen.
EPS can typically be diagnosed when there are clinical signs of an obstructed intestine, along with imaging evidence of the intestine being encapsulated. While other imaging methods, like abdominal X-rays, may also be used, they may not be as reliable as a CT scan. This is because X-rays may not show the condition as accurately (lower sensitivity) or rule it out when it’s not present (lower specificity). Even though X-rays might not show the condition, signs like abnormal air-fluid distributions and unusual width of the bowel or calcification in the peritoneum can raise suspicion of EPS in the presence of intestinal blockage.
The most accurate way to confirm EPS is through surgical procedures like laparoscopy or laparotomy, which allow the doctor to directly observe the thickening of the peritoneum around the bowel. However, these methods are invasive and carry higher risks, so they are rarely used solely for the purpose of diagnosing EPS.
Treatment Options for Encapsulating Peritoneal Sclerosis
The first step in treating Encapsulating Peritoneal Sclerosis (EPS) is to think about stopping a treatment called peritoneal dialysis to prevent additional damage to a thin tissue called the peritoneal membrane that lines the abdomen. However, this won’t be beneficial for everyone, so doctors need to weigh up the pros and cons because most patients will have to switch to another treatment called hemodialysis. Hemodialysis, which cleans your blood outside of your body, comes with its own set of potential risks and lifestyle changes compared to peritoneal dialysis, which takes place inside your body. Even more challenging, in many instances, symptoms of EPS can worsen or may only be diagnosed years after peritoneal dialysis has stopped.
If the decision to stop peritoneal dialysis is made, the standard care includes moving from peritoneal dialysis to hemodialysis and taking out the peritoneal dialysis catheter, a small tube put into your abdomen for the dialysis treatment. To reduce the risk of abdominal adhesions, which are bands of tissue that can cause your abdominal tissues and organs to stick together, the peritoneum should be flushed with a specific fluid twice a week. Additionally, bowel rest should be given for four to twelve weeks, depending on the severity of symptoms.
Medication for EPS may involve the use of corticosteroids, which are drugs that reduce inflammation, and/or an estrogen-blocking drug called tamoxifen. Corticosteroids function by decreasing inflammation and the development of a protein called fibrin, which can cause tissue to thicken and scar. They have been shown to be effective, particularly in the early stages of EPS. However, corticosteroids become less effective in later stages, when significant scar tissue formation and bowel obstruction can occur. Tamoxifen can be an alternative treatment and may sometimes be preferred, considering the potential side effects of steroids. Often, though, tamoxifen is given along with steroids.
Surgery becomes an option when there’s a risk of obstruction due to fibrotic adhesions, bands of fibrous tissue that can cause organs to stick together. This may involve a procedure known as a peritonectomy, which removes the adhesions and sclerotic tissue (hardened tissue) in the abdomen. It’s considered when patients have severe chronic symptoms that haven’t responded to other treatments or when there’s an immediate risk of bowel obstruction. However, it does carry risks like perforation of the intestines, infection, bleeding, formation of abnormal connections between organs known as fistulas, and even death. In spite of these risks, some techniques have been suggested to prevent recurrence, such as suturing intestine to intestine, and using medication management with steroids or tamoxifen might decrease risk.
Optimizing nutrition and hydration is critical for patients with EPS. Those opting for surgery are at a high risk of refeeding syndrome, a potentially fatal condition that can occur when someone who is malnourished starts eating again, so robust nutritional support is vital before and after surgery. It’s been found that patients who underwent surgery had better results when total parenteral nutrition – a method of feeding someone intravenously – was used. However, not all patients with EPS gain the same benefits from this nutrition method, and patients with chronic EPS can benefit from close monitoring and increased oral intake.
What else can Encapsulating Peritoneal Sclerosis be?
A disease that could be confused with encapsulating peritoneal sclerosis (EPS) is peritoneal encapsulation. This is an uncommon condition where a thin extra layer surrounds parts of the small intestine. This creates an additional layer around the intestine, which usually doesn’t cause any symptoms and is found by chance. However, it can also lead to symptoms similar to that of a small intestine blockage. This condition is different from EPS because peritoneal encapsulation is a birth defect, not a condition that develops from chronic inflammation.
Because lots of the symptoms of EPS are generic problems related to the digestive system, it can be mistaken for a wide range of other conditions, such as:
- Small bowel obstruction
- Gastroparesis
- Irritable bowel syndrome
- Pancreatic adenocarcinoma
- Peptic ulcer disease
- Pancreatitis
- Crohn’s disease
- Retroperitoneal fibrosis
- Tumors
- Cancer
- Hernias
- Peritonitis
What to expect with Encapsulating Peritoneal Sclerosis
A study from Taiwan looked at patients from three different medical centers. These patients were split into two groups, based on how severe their encapsulating peritoneal sclerosis (EPS) was. EPS is a condition where the tissue lining the inside of the abdomen becomes thick and hard. In severe cases, there were signs and symptoms of serious complications like hard-to-manage blockages, poor blood supply to the gut, severe infection (or sepsis), and situations that required surgery.
The study found that the overall mortality rate of EPS was 35%. It was even higher, as much as 74%, in patients who had severe EPS. EPS is a condition with a high likelihood of causing other serious health problems. The rate of people dying from it can be as high as 50% within a year of being diagnosed.
Possible Complications When Diagnosed with Encapsulating Peritoneal Sclerosis
There can be complications from both the medicines taken and the operation. The medicine called Tamoxifen can increase the risk of certain types of cancer like endometrial adenocarcinoma and uterine sarcoma, as well as conditions like pulmonary embolism (a blockage in the lungs), and stroke in female patients. In contrast, Prednisone can cause several side effects such as increased blood pressure, high blood sugar levels, changes in potassium and sodium balance, fluid retention, mood swings, and physical symptoms resembling Cushing syndrome, which is a condition that occurs from exposure to high cortisol levels for a long time.
Operations can bring their own set of issues. These may include too much bleeding, accidental damage to the intestines, surgical site infection, deep vein thrombosis, a condition known as atelectasis that causes lung tissue to collapse, and sepsis, which is a life-threatening infection.
Common Complications:
- Increased risk of certain cancers (for Tamoxifen)
- Pulmonary embolism and stroke (for Tamoxifen)
- High blood pressure and blood sugar levels (for Prednisone)
- Mood changes (for Prednisone)
- Excessive surgical bleeding
- Inadvertent bowel injury during surgery
- Surgical site infection
- Deep vein thrombosis
- Atelectasis
- Sepsis
Many of these issues happen after surgery, so it’s worth considering preventive measures. These could include techniques to prevent blood clots, antibiotics before surgery, careful handling of bladder catheters, promoting good lung function, and gradually reintroducing food as tolerated.
Preventing Encapsulating Peritoneal Sclerosis
It’s very important for patients to understand their health condition and treatment options. By understanding your health, you’ll be better equipped to make decisions about your care. This involves knowing the risks and benefits of each treatment, and any possible complications. The key to this is good communication between you and your healthcare team.
One communication method the healthcare team might use is the ‘teach-back’ method. This is where you’ll be asked to repeat back what you’ve been told, to check you’ve understood it fully. This often involves answering any questions you have and explaining everything in a way that avoids confusing medical terms. This way, if you’re considering a procedure like peritoneal dialysis, you will understand its benefits and risks. In particular, you need to be aware of the increased risk of a rare complication called Encapsulating Peritoneal Sclerosis (EPS).
With good education about this potential complication from dialysis, you’ll know what signs and symptoms to look out for, which can help to detect EPS early. Learning about your condition can reduce the risk of a delayed diagnosis and ensure you get the right treatment. This applies not only to the disease but to treatment options as well. You should also understand the pros and cons of using drugs like steroids and tamoxifen, as well as surgery. This will help you make an informed decision about your treatment.
