Update on potential medical treatments for encapsulating peritoneal sclerosis; human and experimental data

Abstract

Encapsulating peritoneal sclerosis (EPS) is an infrequent but serious complication of peritoneal dialysis (PD). The pathogenesis is unknown but speculation is ongoing. The current management of EPS focuses on prevention and treatment of the inflammatory and fibrotic changes at the level of the peritoneal membrane with immunosuppressive and antifibrotic agents, respectively. This article reviews the currently available human and animal data on potential agents to prevent and/or treat EPS. We propose a strategy for early diagnose EPS in an attempt to avoid the development of the full-blown and potentially life-threatening clinical syndrome of EPS. Future research should focus on studying potential prophylactic and therapeutic agents in humans in large, multicenter, randomized trials but also on early detection of EPS in the inflammatory phase by means of biomarkers and the establishment of a composite EPS score.

Long-Term Success with Adhesiolysis in Post-Transplant Encapsulating Peritoneal Sclerosis: A Retrospective Case Series of 4 Patients and Review of the Literature

“…Our cases were already on 5 mg per day prednisolone treatment as part of their immunosuppressive regimens. Because these agents have antiinflammatory properties, the reduced doses of corticosteroids in recent maintenance immunosuppressive regimens have been suggested as contributing factors to the increased number of post-transplant cases (9). Hence, we increased the prednisolone doses to 40 mg per day with the intent of tapering the dose.…”

Itraconazole Attenuates Peritoneal Fibrosis Through Its Effect on the Sonic Hedgehog Signaling Pathway in Mice

“…Due to issues with drug accessibility and safety, there are only a few options in the treatment of peritoneal fibrosis in PD patients, including tamoxifen and other immunosuppressive drugs [31]. Therefore, itraconazole may be a useful treatment option for peritoneal fibrosis.…”

Cocoon Syndrome: A Rare Entity

“…In patients with mild symptoms, conservative management including bowel rest, nasogastric tube decompression and nutritional support may be considered the most appropriate therapeutic option [4], as a medical treatment also included the use of drugs such as tamoxifen, steroids, colchicine, and azathioprine [2,5]. In Figure 2.…”