A Case of Idiopathic Encapsulating Peritoneal Sclerosis with an Unrecognized Cause

Itatani, Yoshiro; Park, Taebum; Kawamoto, Kazuyuki; Ito, Tadashi; Ogasawara, Kunio

doi:10.5833/jjgs.42.311

Cited by 1 publication

(1 citation statement)

References 15 publications

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“…Encapsulating peritoneal sclerosis (EPS) is a rare clinical syndrome characterized by an inflammatory fibrocollagenous membrane encasing the small and large intestines, resulting in recurrent intestinal obstruction (1)(2)(3). EPS often occurs as refractory ascites retention due to chronic peritonitis (2)(3)(4)(5)(6). Eltringham et al first reported EPS as a side effect of βblockers in 1977 (7); however, most EPS cases have been described as secondary to peritoneal dialysis (1-3) since this phenomenon was first described by Gandhi et al in 1980 (8).…”

Section: Introductionmentioning

confidence: 99%

Encapsulating Peritoneal Sclerosis in Systemic Lupus Erythematosus, Rheumatoid Arthritis, and Systemic Sclerosis

et al. 2023

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We encountered a 57-year-old Japanese woman with encapsulating peritoneal sclerosis (EPS) in systemic lupus erythematosus (SLE), rheumatoid arthritis (RA), and systemic sclerosis. The patient was admitted to our hospital because of ascites retention. Administration of tocilizumab, an anti-interleukin-6 (IL-6) receptor antibody, for her RA reduced the refractory ascites remarkably; however, she developed sudden acute gastrointestinal bleeding and died a year later. On autopsy, sclerotic thickening of the peritoneum showed diffuse infiltration of podoplanin-positive fibroblast-like cells, and a diagnosis of EPS was made. EPS rarely occurs in SLE, and tocilizumab may be a new treatment candidate for EPS.

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