What is the best treatment approach for a patient with encapsulating peritoneal sclerosis, likely due to long-term peritoneal dialysis for chronic kidney disease or end-stage renal disease?

Encapsulating Peritoneal Sclerosis: Treatment Approach

The primary treatment for encapsulating peritoneal sclerosis (EPS) requires immediate cessation of peritoneal dialysis with transition to hemodialysis, combined with corticosteroids for the inflammatory stage and tamoxifen for the fibrotic stage, with surgical enterolysis reserved for refractory cases causing bowel obstruction. 1, 2

Immediate Management: Discontinue Peritoneal Dialysis

• Transfer the patient from peritoneal dialysis to hemodialysis immediately upon diagnosis or strong clinical suspicion of EPS 3, 4
• EPS represents a formal indication for switching from continuous ambulatory peritoneal dialysis (CAPD) to hemodialysis, as it causes technique failure in approximately 16% of patients 3
• The mortality rate approaches 50% at one year after diagnosis, making prompt intervention critical 1

Medical Management: Stage-Based Approach

Inflammatory Stage Treatment

• Corticosteroids (prednisolone) are the treatment of choice during the inflammatory stage 1, 4
• Peritoneal lavage combined with prednisolone administration has been reported effective in preventing or stopping EPS progression 4

Fibrotic Stage Treatment

• Tamoxifen therapy should be initiated for patients in the fibrotic stage 1, 2
• In a single-center series, 10 patients received tamoxifen treatment with apparent benefit, though distinguishing between inflammatory and fibrotic stages can be difficult in practice 2
• Many clinicians use both corticosteroids and tamoxifen concurrently given the challenge of definitively staging the disease 1

Nutritional Support

• Aggressive nutritional support is essential, frequently requiring total parenteral nutrition (TPN) 1, 5
• EPS causes recurrent small bowel obstructions that compromise oral intake and intestinal function 1
• PEG-J placement may be considered for enteral nutrition and gastric decompression in patients with persistent symptoms 5

Surgical Intervention: Reserved for Specific Indications

• Surgery (peritonectomy and enterolysis) should be reserved for patients who fail conservative medical therapy and have persistent bowel obstruction 1, 5
• Total enterolysis to remove the fibrous capsule from the bowel is indicated when bowel obstruction has occurred 4
• Surgical management is time-consuming, high-risk, and should only be performed at institutions with surgical expertise in this area 1
• One case series demonstrated successful outcomes with exploratory laparotomy, extensive lysis of adhesions, and bowel diversion in patients with deteriorating quality of life 5

Risk Stratification and Prevention

Duration-Based Risk

• The prevalence of EPS increases dramatically with peritoneal dialysis duration: 0.9% for <5 years, 3.8% for 5-10 years, and 11.5% for >10 years 4
• Consider elective transition to hemodialysis for patients approaching 5 years on peritoneal dialysis, particularly those developing high-transport status 4

Monitoring Strategy

• Careful monitoring with CT scans of the peritoneal membrane should be performed in patients beyond 5 years of peritoneal dialysis 2
• Early catheter removal should be considered in patients with peritoneal thickening on imaging 2

Common Pitfalls to Avoid

• Do not delay diagnosis waiting for definitive imaging findings—CT abdomen can be suggestive but many patients require exploratory laparotomy for diagnosis 1
• Recognize that peritonitis rates may not differ between EPS patients and the general peritoneal dialysis population, so absence of frequent peritonitis does not exclude EPS 2
• Avoid continuing peritoneal dialysis once EPS is suspected or diagnosed, as this perpetuates the inflammatory insult 4, 1
• Do not pursue surgical intervention prematurely—surgery carries significant morbidity and should only be considered after medical management fails 1

Post-Transplant Considerations

• EPS can present years after kidney transplantation, even after cessation of peritoneal dialysis 6
• Patients who undergo kidney transplantation after peritoneal dialysis remain at risk and require ongoing surveillance 5, 6
• Immunosuppressive medications should be continued in transplant recipients with EPS, with tamoxifen added as definitive medical management 6