Encapsulating peritoneal sclerosis

Encapsulating Peritoneal Sclerosis (EPS) is a rare and severe complication associated with long-term peritoneal dialysis (PD). It is characterized by the formation of a thick fibrous membrane encapsulating the intestines, which can lead to intestinal obstruction and severe nutritional and metabolic disturbances. The exact cause of EPS is not fully understood, but it is believed to be related to the prolonged exposure to PD fluids, which can induce inflammatory and fibrotic changes in the peritoneum.

Etiology and Pathogenesis

The development of EPS is thought to be multifactorial, involving both patient-related and treatment-related factors. Long duration of PD, the use of certain PD solutions, recurrent episodes of peritonitis, and genetic predisposition are among the factors implicated in the pathogenesis of EPS. The condition is characterized by progressive fibrosis of the peritoneum, leading to the encapsulation of the intestines and restriction of their movement.

Clinical Features

EPS can present with a wide range of symptoms, which may include abdominal pain, nausea, vomiting, anorexia, weight loss, and altered bowel habits. In advanced cases, patients may develop signs of intestinal obstruction. Due to the nonspecific nature of these symptoms, the diagnosis of EPS can be challenging and requires a high index of suspicion.

Diagnosis

The diagnosis of EPS is primarily based on clinical presentation and imaging findings. Computed tomography (CT) scans of the abdomen are particularly useful in identifying the characteristic features of EPS, such as bowel encapsulation and calcification of the peritoneum. In some cases, a biopsy of the peritoneum may be necessary to confirm the diagnosis and exclude other causes of peritoneal fibrosis.

Treatment

The treatment of EPS is complex and may involve medical and surgical interventions. Medical treatment focuses on managing symptoms and includes the use of anti-inflammatory and immunosuppressive medications. Nutritional support is also critical in the management of patients with EPS. In cases where there is intestinal obstruction or severe complications, surgical intervention to remove the fibrous capsule may be necessary. However, surgery carries a high risk of morbidity and mortality.

Prognosis

The prognosis of EPS is poor, with high rates of morbidity and mortality. Early diagnosis and intervention are crucial in improving outcomes. Transitioning to hemodialysis and avoiding the use of certain PD solutions may help reduce the risk of developing EPS in patients undergoing long-term PD.

Prevention

Preventive strategies for EPS include minimizing the duration of PD treatment, using biocompatible PD solutions, and prompt treatment of peritonitis episodes. Regular monitoring of patients on long-term PD for signs of peritoneal membrane changes is also recommended.

NIH genetic and rare disease info

• NIH info on Encapsulating peritoneal sclerosis

Encapsulating peritoneal sclerosis is a rare disease.