Encapsulating Peritoneal Sclerosis Presenting with Recurrent Ascites and Tamoxifen: Case Reports and Review of the Literature

Abstract

there is no comment

Encapsulating peritoneal sclerosis (EPS) is a rare but serious complication of peritoneal dialysis (PD). The morbidity and mortality of EPS is extremely high.[1] We report two PD patients with EPS presenting with recurrent ascites and their successful treatment with tamoxifen. Literature review was performed to find the current evidence for the use of tamoxifen in EPS.

CASE 1

A 36-year-old female with end stage renal disease (ESRD) secondary to hypertensive nephrosclerosis started continuous ambulatory peritoneal dialysis (CAPD) in 1995 until she received a renal transplant in 1996. She recommenced CAPD after the transplant failed in 2000. Subsequently, she was switched to automated peritoneal dialysis. She underwent graft nephrectomy in 2001 for chronic rejection. In 2004, a peritoneal equilibrium test (PET) demonstrated high transporter status. The following year, she developed two episodes of severe Serratia Marcescens peritonitis separated by three months. The PD cannula was removed two days after the second peritonitis episode and thereafter she commenced hemodialysis. In June 2006, she presented with severe abdominal pain, vomiting, and ascites, and over the next two months had multiple admissions for recurrent ascites that required repeated drainage. The ascitic fluid initially was reported to be sterile; however, subsequent drainage revealed pseudomonas, and she was treated with intravenous gentamicin. In August 2006, she had a diagnostic laparotomy performed, which revealed an intra-abdominal mass of small bowel with an epithelialized, pigmented cavity and plaques adhering to the small bowel (Howse N, personal communication). This was biopsied and showed loose fibroblastic tissue with vascular stroma and fibrinous material. The patient remained symptomatic of partial bowel obstruction. Over three months, her body weight fell from a baseline of 65 kg to 51.8 kg and serum albumin dropped from 41 g/L to 20 g/L. Total parenteral nutrition and tamoxifen 20 mg once daily (OD) were commenced in September. Her gastrointestinal symptoms improved quickly within two weeks, and her body weight and serum albumin increased to 53.6 kg and 42 g/L, respectively. The patient continued to do well at six-month follow-up.

CASE 2

A 38-year-old female with ESRD secondary to crescentic glomerulonephritis started on hemodialysis in 1982 until her first transplant in 1984, after her graft failed in 1989. She commenced on CAPD until her second renal transplant in 1990. This second graft failed in 1999, and she went back to CAPD until 2005. In 2004, PET demonstrated high transporter status. During these six years, she had four episodes of Staphylococcus Aureus peritonitis and switched to hemodialysis after the fourth episode. Six months later, she presented with abdominal pain and ascites. CT abdomen confirmed a thickened and calcified peritoneum and localized collection, which was drained. Five months later, she presented with worsening ascites. Further drainage was undertaken under ultrasound guidance and peritoneal biopsy was performed. Ascitic fluid remained sterile. The biopsy showed reactive peritoneal fibrosis and neovascularization. She was started on tamoxifen 20 mg OD in September 2006. Her gastrointestinal symptoms resolved within two weeks, and she received a successful renal transplant three months later. At six‐month follow-up, the patient remained well.

DISCUSSION

Encapsulating peritoneal sclerosis (EPS) is a rare but life-threatening complication of peritoneal dialysis. While there are a number of treatments that have been proposed, there is currently no standard therapy for EPS.[2] Ideally, early detection would prevent the complications of EPS and allow patients to switch dialysis modality and receive total parenteral nutrition sooner, in an attempt to retard disease progression. This early detection, however, requires a high index of suspicion for EPS. Clinical and radiological findings can be used,[1–4] but others have confirmed the diagnosis of EPS from laparotomy findings and peritoneal biopsy,[2] such as in these two cases. Tamoxifen has been used successfully in the treatment of fibrosing diseases such as retroperitoneal fibrosis, sclerosing cervicitis, fibrosing mediastinitis, and rapidly growing desmoid tumors.[5],[6] Tamoxifen also appears to upregulate transforming growth factor-beta 1, which has a stimulating effect on metalloproteinase-9. Because metalloproteinase-9 degrades type IV and denatured collagen, the upregulation of transforming growth factor-beta 1 might favor mesothelial healing by facilitating the removal of denatured collagen.[6]

In the literature, three authors have described using tamoxifen and steroids in the treatment of EPS.[8–10] The first case of EPS treated with tamoxifen alone was reported by Turner et al.[7] A few other case reports have also reported success using this drug.[3],[6],[7] Others have advocated empirical treatment of tamoxifen to prevent the development of EPS in those patients with high risk factors (long-term [i.e., > 5 years] PD, those with high transporter status, loss of ultrafiltration or radiological changes[4]), but this requires further investigation.

However, our cases are unique, as both patients presented with recurrent ascites and were treated successfully with tamoxifen. No other cause for the ascites was found. Tamoxifen 20 mg OD was used as the lone drug therapy and resulted in the quick resolution of gastrointestinal symptoms, therefore providing further support to the usefulness of tamoxifen in this potentially life-threatening condition. Steroid was not used as there were concerns of it worsening the infected ascites. We suggest that tamoxifen alone can be used in EPS presenting with recurrent ascites, abdominal pain, and subacute abdominal obstruction.