title: A combination of corticosteroid, sirolimus, and intradialytic parenteral nutrition in encapsulating peritoneal sclerosis: Case report and literature review.
abstract: Encapsulating peritoneal sclerosis (EPS) is a rare complication of peritoneal dialysis that carries a high morbidity and mortality. Although its pathogenesis is still not clear, the "two hit theory" suggests that long term deterioration of the peritoneum combined with intraperitoneal inflammation is needed in the pathogenesis of EPS. To date, there is no proven effective therapy with an absence of randomized controlled trials. Individual case reports and small case series have reported on the use of tamoxifen and corticosteroids for medical management of EPS. Here, we present the first case of EPS treated successfully with a combination of sirolimus, low dose corticosteroid and intradialytic parenteral nutrition. A critical review of the relevant literature on this subject is also presented to determine the best approach.
authors.affiliation: Internal medicine, Universidade Estadual Paulista Julio de Mesquita Filho-UNESP, Botucatu, SP, Brazil.
authors.forename: Alexandre
authors.initials: A
authors.lastname: Minetto Brabo
authors.affiliation: Internal medicine, Universidade Estadual Paulista Julio de Mesquita Filho-UNESP, Botucatu, SP, Brazil.
authors.forename: Nayrana
authors.initials: N
authors.lastname: Soares Do Carmo Reis
authors.affiliation: Internal medicine, Universidade Estadual Paulista Julio de Mesquita Filho-UNESP, Botucatu, SP, Brazil.
authors.forename: Pasqual
authors.initials: P
authors.lastname: Barretti
authors.affiliation: Internal medicine, Universidade Estadual Paulista Julio de Mesquita Filho-UNESP, Botucatu, SP, Brazil.
authors.forename: Daniela
authors.initials: D
authors.lastname: Ponce
mesh_terms: D000293:Adolescent; D000305:Adrenal Cortex Hormones / Q000494:pharmacology / Q000627:therapeutic use*; D000900:Anti-Bacterial Agents / Q000494:pharmacology / Q000627:therapeutic use*; D005260:Female; D006801:Humans; D010288:Parenteral Nutrition / Q000379:methods*; D056627:Peritoneal Fibrosis / Q000188:drug therapy* / Q000473:pathology; D020123:Sirolimus / Q000494:pharmacology / Q000627:therapeutic use*
chemical_list: D000305:Adrenal Cortex Hormones; D000900:Anti-Bacterial Agents; D020123:Sirolimus
keywords: Peritoneal dialysis; encapsulating peritoneal sclerosis; intradialytic parenteral nutrition; sirolimus