ABSTRACT:Encapsulating peritoneal sclerosis (EPS) is a rare complication of peritoneal dialysis (PD) that carries a high morbidity and mortality. The 'two hit theory' suggests that long term deterioration of the peritoneum combined with intraperitoneal inflammation is needed in the pathogenesis of EPS. For unclear reasons, post transplantation EPS is being increasingly reported in patients previously on PD. To date, there is no proven effective therapy with an absence of randomised controlled trials. Individual case reports and small case series have reported on the use of tamoxifen and corticosteroids for medical management of EPS. The use of everolimus has been reported in a single case, and never in the setting of renal transplantation. Here, we present the first case of post-transplant encapsulating peritoneal sclerosis treated successfully with a combination of everolimus, tamoxifen, low dose corticosteroid and surgery.
CASE REPORTA 37-year-old man of Vietnamese background presented to our hospital in March 2009 for deceased donor renal transplantation.End-stage renal failure was secondary to hepatitis C-related mesangioproliferative glomerulonephritis with cryoglobulinaemia. He had been on automated peritoneal dialysis for over 6 years with a combination of dextrose based peritoneal dialysis solutions. There had been no previous episodes of peritoneal dialysis-related peritonitis. A preceding peritoneal equilibration test showed that he was a high average transporter. In the year prior to transplant he had lost all residual renal function, and had signs of peritoneal membrane failure. At the time of being called for transplantation, he was hypertensive to 250/110 mmHg and was fluid overloaded in excess of 5 L, as a consequence of gradual ultrafiltration failure. Biochemically he was under-dialysed with a urea of 28 mmol/L and creatinine 1180 μmol/L. Hypertension had been complicated by severe left ventricular hypertrophy, diastolic dysfunction and moderate pulmonary hypertension. Other comorbidities were renal osteodystrophy and renal anaemia. Previous liver biopsies and his hepatitis C viral loads by polymerase chain reaction suggested that this disease was quiescent with no evidence of cirrhosis.The donor was a 46-year-old, brain dead man. There was a 5/6 HLA mismatch with a cold ischemic time of 15.5 hours. Serology showed cytomegalovirus donor and recipient positivity. Transplantation was planned with 'standard' induction therapy including basiliximab, methylprednisone, tacrolimus and mycophenolate mofetil. Standard prophylactic agents including valganciclovir, trimethoprim/sulfamethoxazole, pantoprazole and nystatin were also commenced. Hypertension was aggressively managed prior to transplant.The transplant surgery was complicated by donor kidney core biopsy-related haematuria and subscapular bleeding with blood pressure instability. Because of the likelihood of need for dialysis after transplant surgery, the surgeon opted to leave the Tenckoff catheter in situ. Dialysis was not required. However, re...