Peritoneal Sclerosis

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Peritoneal sclerosis and fibrosis include a broad spectrum of histopathological changes of the peritoneal membrane. The main macroscopic and microscopic findings include: thickening and sclerosis of the membrane with or without adhesions, dense and fibrous tissue, loss of mesothelial cells, deposition permeated with chronic inflammatory infiltrate, and in its most severe manifestation (encapsulated peritoneal sclerosis) encasement of the bowel by a new membrane resembling a cocoon.

Among the postulated etiologies are the uremic milieu, acetate containing PDF, recurrent peritonitis, plasticizers, formaldehyde, pyrogens/endotoxin, multiple abdominal surgeries, clorhexidine, hypertonic and acidic PDF and inflammatory or foreign body response to the peritoneal catheter. No single factor has been incriminated in the pathogenesis of peritoneal sclerosis, suggesting a probable multifactorial etiology.

The possible pathogenetic mechanisms are loss of normal cellular structure, loss of plasminogen activation from damaged mesothelial cells, impaired fibrinolysis, fibrosis, elevated levels of type I and III procollagen propeptide and the “plasma leak to response hypothesis”¹. The latter postulates plasma extravasation due to increased transport, accumulation of plasma components over the peritoneal surface, followed by migration of cellular components into the plasma and fibrin gel to form a de novo biomembrane.

The incidence is relatively low, estimated at 1.5 – 4.6 cases per 1,000 patient year², and the geographical distribution somewhat ununiform, with the highest reported prevalence in Japan³.

The diagnosis is based on the clinical features (UF loss, reduced solute transport, nausea, vomiting, bowel obstruction, progressive inanition, overhydration, ascites and hemoperitoneum); blood testing (increased C-reactive protein and TGF_ß1 and decreased hemoglobin, serum albumin, CA-125 levels); plain abdominal x-rays, contrast studies, CT scan or ultrasound; laparoscopy; and biopsy¹.

Preventive measures recommended for encapsulated peritoneal sclerosis are:

Intraperitoneal heparin use in case of fibrin production
Avoidance of persistent hemoperitoneum
Reduction of peritonitis rates
Avoidance of hypertonic glucose solutions
Use of more biocompatible dialysis solutions
Avoidance of beta blockers
Peritoneal rest for at least one month in patients with type I UFF

Although the definitive treatment has not been characterized, the following have been suggested based on limited clinical experience:

Avoidance of surgical intervention if conservative measures are successful
Palliative surgery for obstruction and necrosis of the bowel
Discontinuation of peritoneal dialysis
Prednisone, azathioprine or tamoxifen^4-7

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References:

Nakayama M. The plasma leak-to-response hypothesis: A working hypothesis on the pathogenesis of encapsulating peritoneal sclerosis after long-term peritoneal dialysis treatment. Perit Dial Int 25 (Suppl 4):S71-S76, 2005
Yokota S, Kumano K, Sakai T. Prognosis for patients with sclerosing encapsulating peritonitis following CAPD. Adv Perit Dial 13:221-223, 1997
Special issue on encapsulating peritoneal sclerosis in Japan in Perit Dial Int 25 (Suppl 2), 2005
Mori Y, Matsuo S, Sutoh H, Toriyama T, Kawahara H, Hotta N. A case of a dialysis patient with sclerosing peritonitis successfully treated with corticosteroid therapy alone. Am J Kidney Dis 30:275-278, 1997
Diaz-Buxo JA. Peritoneal sclerosis in a woman on continuous cyclic peritoneal dialysis. Semin Dial 5:317-320, 1992
del Peso G, Bajo MA, Gil F, Aguilera A, Ros S, Costero O, Castro MJ, Selgas R. Clinical experience with tamoxifen in peritoneal fibrosing syndromes. Adv Perit Dial 19, 32-35, 2003
Eltoum MA, Wright S, Atchley J, Mason JC. Four consecutive cases of peritoneal dialysis-related encapsulating sclerosis treated successfully with tamoxifen. Perit Dial Int 26:203-206, 2006