Four consecutive cases of peritoneal dialysis-related encapsulating peritoneal sclerosis treated successfully with tamoxifen

Abstract

Sclerosing peritonitis is a rare complication of peritoneal dialysis (PD). In encapsulating peritoneal sclerosis (EPS), the most severe form of the disease, the intestine is entrapped in a fibrous tissue, causing intestinal obstruction. Patients are typically seriously ill, with evidence of infection and requirement for parenteral nutrition. A mortality rate of 73% has been reported. There is no established medical treatment and surgery has offered variable results. Our unit provides renal replacement therapy for a population of about 2 million. The prevalent population of PD patients averages 110. The cumulative PD population since January 1993 is 643, with an EPS prevalence of 0.6%. Influenced by the first case reported by Allaria in 1999 suggesting benefit of tamoxifen in treating EPS, we have treated with tamoxifen the four consecutive cases of EPS that have presented since 1999. All 4 patients have survived and recovered intestinal function. ALL showed prior evidence of peritoneal dysfunction with ultrafiltration failure and were characterized by long duration of PD therapy rather than multiple episodes of peritonitis. We conclude that tamoxifen is a highly promising therapy in EPS, hitherto a usually fatal condition. This description of its efficacy in acutely ill patients with EPS complements its possible prophylactic use in patients with the earlier and milder disease, sclerosing peritonitis. A high index of clinical suspicion for sclerosing peritonitis is desirable, perhaps facilitated by routine screening of at-risk patients.