EPS is a rare complication of PD that is characterized :
intraperitoneal inflammation
Fibrosis
EPS causes ultrafiltration failure and bowel obstruction and is associated with significant morbidity and a high mortality.
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M tor inhibitors for management of encapsulating peritoneal sclerosis
1. mTOR inhibitors for management of
encapsulating peritoneal sclerosis
Dr Nahid Rahimzadeh
Ped Nephrologist
Rasoul-e-Akram hospital (IUMS)
2. Introduction
ā¢ EPS is a rare complication of PD that is characterized :
ā¢ intraperitoneal inflammation
ā¢ Fibrosis
ā¢ EPS causes ultrafiltration failure and bowel obstruction and is associated
with significant morbidity and a high mortality.
3. ā¢ Signs of peritoneal ļ¬brosis are detected in 50% to 80% of patients within
one to two years on PD.
ā¢ The frequency of EPS varies across the globe between 0.5% to 7.3% , but
may be as high as 17.2% in patients undergoing PD for 15 or more years.
ā¢ The clinical presentation and diagnosis of EPS differ among centers,
countries, and over time.
ā¢ EPS may even develop after PD discontinuation, i.e., in patients who have
switched to hemodialysis (HD) or have undergone kidney transplantation
4. ā¢ The risk of EPS may be higher among transplant recipients who used to be on
peritoneal dialysis compared with patients who are still on peritoneal dialysis
and have not received a transplant.
ā¢ The reasons for the transplantation-associated risk are not known.
ā¢ The increased risk may be related to:
ā¢ the cessation of dialysis
ā¢ calcineurin inhibitors
ā¢ EPS is not a contraindication to transplantation
5. ā¢ PostTx-EPS occurs in patients with a previous history of PD in the ļ¬rst to
second post-transplantation year.
ā¢ At the present time almost 50 % of all EPS cases are PostTx-EPS.
7. Risk Factors
ā¢ Time on peritoneal dialysis
ā¢ Severe peritonitis
ā¢ Specific dialysate fluids
ā¢ Specific drugs
ā¢ Disinfectants (e.g., chlorhexidine, povidone iodine)
ā¢ Decreased ultrafiltration and increased solute transport
8. Clinical Features
ā¢ Early(inflammatory) :
ā¢ Anorexia, nausea, diarrhea, and intermittent abdominal pain.
ā¢ The physical exam is often unrevealing in early stages, may show evidence of volume
overload including weight gain and edema
ā¢ Late(ileus):
ā¢ Ileus and/or peritoneal adhesions, symptoms of intermittent obstruction( including
severe cramping, abdominal pain, constipation, and vomiting).
ā¢ The exam may reveal an abdominal mass
9. Laboratory findings
ā¢ There are no routine laboratory findings that suggest EPS.
ā¢ The peritoneal dialysis fluid may show WBCs
ā¢ Serial peritoneal equilibration tests may show a trend toward increased solute transport and
decreased ultrafiltration
ā¢ Radiographic findings
ā¢ CT scan shows peritoneal calcification, bowel thickening, tethering, and dilatation.
ā¢ Bowel tethering and peritoneal calcification are the most specific findings
10. Diagnosis of EPS
ā¢ Gastrointestinal obstruction with severe abdominal pain
ā¢ Nausea and vomiting
ā¢ Weight loss
ā¢ The transporter status is high in peritoneal equilibration tests
12. Treatment
ā¢ The treatment of EPS is still controversial but is most often proposed to be
a combination of:
ā¢ Cessation of PD
ā¢ Use of immunosuppressive
ā¢ Antifibrotic agents
ā¢ Nutritional support
21. In conclusion
ā¢ The risk of EPS development is very high in patients with a history of more than 5 years of
PD treatment, frequent peritonitis episodes and alteration of PD efļ¬cacy.
ā¢ In all forms, EPS is a serious, sometimes life-threatening condition.
ā¢ If not accurately and early diagnosed and treated, EPS can severely affect the patientsā
survival.
ā¢ The use of mTORi in the EPS classical form must be carefully evaluated, taking into
consideration the possibility of adverse effects in patients who do not need
immunosuppressive treatment.
22. ā¢ The best medical treatment for PostTxEPS is still controversial.
ā¢ mTORi may represent a useful option in PostTx-EPS treatment.
ā¢ Post-transplantation immunosuppressive protocol based on mTORi, MMF
and steroids, avoiding or reducing the proļ¬brotic drugs (CNI), in order to
prevent PostTx-EPS development