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Saudi Journal of Kidney Diseases and Transplantation
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Year : 2014  |  Volume : 25  |  Issue : 2  |  Page : 419-422
Encapsulating peritoneal sclerosis in a patient with Alport's syndrome on long-term peritoneal dialysis

1 Nephrology Clinic, Dr. Lutfi Kırdar Kartal Trainig Hospital, Kartal, Istanbul, Turkey
2 Surgery Clinic, Dr. Lutfi Kırdar Kartal Trainig Hospital, Kartal, Istanbul, Turkey

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Date of Web Publication11-Mar-2014

How to cite this article:
Bahcebasi ZB, Akarsu O, Yildirim M, Kucuk H. Encapsulating peritoneal sclerosis in a patient with Alport's syndrome on long-term peritoneal dialysis. Saudi J Kidney Dis Transpl 2014;25:419-22

How to cite this URL:
Bahcebasi ZB, Akarsu O, Yildirim M, Kucuk H. Encapsulating peritoneal sclerosis in a patient with Alport's syndrome on long-term peritoneal dialysis. Saudi J Kidney Dis Transpl [serial online] 2014 [cited 2022 Dec 4];25:419-22. Available from: https://www.sjkdt.org/text.asp?2014/25/2/419/128607
To the Editor,

Encapsulating peritoneal sclerosis (EPS) is a rare, serious complication in patients on long-term peritoneal dialysis (PD). It was first des­cribed by Ghandi et al in 1980. [1] Malnutrition and bowel obstruction are the associated com­plications, and the mortality rate is very high. [2] The most important risk factors for EPS in­clude recurrent peritonitis, long-term PD and use of hypertonic glucose solutions. Cortico­steroids and tamoxifen are used for the medi­cal treatment of EPS. [3]

The patient, a 35-year-old man with end-stage renal disease due to Alport's syndrome, had been on treatment with PD for the pre­ceding 10 years. He presented with an episode of peritonitis and treatment with antibiotics was not sufficiently effective. High C-reactive protein level (100 mg/dL), malnutrition and abdominal discomfort persisted. The perito­neal fluid was exudative and cultures including that for tuberculosis and fungus remained negative. Ultrasound (US) of the abdomen showed large, cystic and septated masses between the liver and the diaphragm with ascites [Figure 1]. Computed tomography (CT) of the abdo­men revealed large loculated fluid collections between the bowel loops and multiple cas­eations of the peritoneum [Figure 2]. Surgical exploration showed thickened fibrous perito­neum covering the bowel walls [Figure 3]. These findings were compatible with the diagnosis of EPS.
Figure 1: Ultrasound images showing large, cystic and septated masses between the liver and the diaphragm with ascites.

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Figure 2: Computed tomography of the abdomen showing large loculated fluid collections between the bowel loops and multiple calfications of the peritoneum

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Figure 3: Intra-operative photograph showing thickened fibrous peritoneum covering the bowel walls.

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Glucocorticoid treatment was contrindicated because the patient had a femoral head osteo­necrosis. Treatment with tamoxifen was star­ted (30 mg daily). Mode of renal replacement therapy was changed to intermittent hemo­dialysis.

Two months later, the patient presented with alternating constipation and diarrhea and severe anorexia. US showed intra-peritoneal echogenic strands with fluid collection. The patient was subjected to surgical viscerolysis with pa­renteral hyperalimentation; antibiotics were administered and the dose of tamoxifen was aug­mented to 40 mg/day. His symptoms improved gradually.

EPS is rare and life-threatening complication of PD with a mortality rate of 24-54%. [4],[5] Encapsulation of the bowel causes progressive obstruction. [6] Exposure of the peritoneal mem­brane to bio-incompatible solutions, recurrent persistent peritonitis, exposure to pro-fibrotic immunosuppressive drugs and genetic predis­position are the factors that are responsible for the development of EPS. [6],[7]

Genetic variations of the tumor growth factor (TGF)-pi gene may make some people high producers, who cannot turn off the fibrotic process. [8] Sampimon et al speculated that Alport's syndrome predisposes to the develop­ment of EPS. [9] Alport's syndrome is a rare disorder of collagen IV, but whether it affects the basement membranes of peritoneal tissue and vessels is unknown. EPS is associated with an increase in interstitial and endothelial col­lagen IV deposits. In the current case, the need for a second surgical exploration and viscerolysis may support this hypotesis. [9]

In our case, the diagnosis of EPS was based on the presence of findings suggestive of intes­tinal obstruction on abdominal CT and pre­sence of malnutrition. Vomiting, abdominal pain, ascites, diarrhea, abdominal distention, ano­rexia and weight loss may all be associated with EPS. [10]

The findings of EPS on CT are peritoneal calcification, bowel wall thickening, peritoneal thickening, loculated fluid collections or tethered bowel loops. [10] In the current case, both US and CT results indicated EPS. [11],[12]

The suggested medical treatment of EPS in­cludes glucocorticoids, mycophenolate mofetil or antifibrotic agents such as tamoxifen or col­chicine. [3],[13] Corticosteroids ameliorate the peri­toneal fibrosis and inflammation and decrease the synthesis of fibroblast growth factor. [2] Tamoxifen has been effective in peritoneal and also retroperitoneal fibrosis. [14],[15] In 1999, the first patient successfully treated with tamo­xifen showed a gradual amelioration and ex­pressive reduction in the thickness of peri­toneal and intestinal loops. [16] Tamoxifen may decrease TGF-beta levels and, thus, may cause an antifibrotic action. The beneficial effect of tamoxifen may be augmented by combining it with corticosteroids. [17] The daily dose of tamo­xifen has been suggested as 10-40 mg, although the duration of treatment is still unclear. [18]

When intestinal obstruction sets in, bowel rest, total parenteral nutrition, tamoxifen, ste­roids and/or other immunosuppressive agents should be started. For severe and persistent intestinal obstruction, surgical enterolysis is needed. [19] In the past, surgical treatment for EPS was contraindicated. Although successful outcomes have been reported after surgical intervention, prognosis after surgery has usually been poor. If symptoms of ileus remain despite proper medical treatment, surgery should be considered. [2] If bowel obstruction persists, laparotomy and enterolysis should be per­formed to obtain a complete cure. As in the current case, with appropriate medical and sur­gical treatment and close follow-up, EPS may not be a fatal complication of PD. [13]

In conclusion, EPS may cause bowel obstruc­tion and malnutrition as a rare but severe complication of long-term PD. Alport's syn­drome may facilitate its development. CT scan and US are useful to confirm the diagnosis of EPS along with the presence of signs and symptoms of intestinal obstruction and malnu­trition.

   References Top

1.Gandhi VC, Humayun HM, Ing TS, et al. Scle­rotic thickening of the peritoneal membrane in maintenance peritoneal dialysis patients. Arch Intern Med 1980;140:1201-3.  Back to cited text no. 1
2.Nakamoto H. Encapsulating peritoneal sclerosis- a clinician's approach to diagnosis and medical treatment. Perit Dial Int 2005;25 Suppl 4:S30-8.  Back to cited text no. 2
3.Balasubramaniam G, Brown EA, Davenport A, et al. The Pan Thames EPS study: Treatment and outcomes of encapsulating peritoneal scle­rosis. Nephrol Dial Transplant 2009;24:3209-15.  Back to cited text no. 3
4.Trigka K, Dousdampanis P, Chu M, et al. Oreopoulos. Encapsulating peritoneal sclero-sis: A single-center experience and review of the literature. Int Urol Nephrol 2011;43:519-26.  Back to cited text no. 4
5.Yılmaz R, Arıcı M. Periton diyalizinin uzun dönem komplikasyonları. Türk Nefroloji Diyaliz ve Transplantasyon Dergisi 2007;16 Suppl 2: 62-7.  Back to cited text no. 5
6.Augustine T, Brown PW, Davies SD, Summers AM, Wilkie ME. Encapsulating peritoneal sclerosis: Clinical significance and implications. Nephron Clin Pract 2009;111:149-54.  Back to cited text no. 6
7.Chin AI, Yeun JY. Encapsulating peritoneal sclerosis: An unpredictable and devastating complication of peritoneal dialysis. Am J Kidney Dis 2006;47:697-712.  Back to cited text no. 7
8.Summer AM. Encapsulating Peritoneal Sclerosis- Have we found the perpetrator? Perit Dial Int 2009;29:499-501.  Back to cited text no. 8
9.Sampimon DE, Vlijm A, Phoa SS, Krediet RT, Struijk DG. Encapsulating peritoneal sclerosis in a peritoneal dialysis patient using biocom­patible fluids only: Is Alport Syndrome a risk factor?. Perit Dial Int 2010;30:240-2.  Back to cited text no. 9
10.Kawaguchi Y, Kawanishi H, Mujais S, Topley N, Oreopoulos DG. Encapsulating peritoneal sclerosis: Definition, etiology, diagnosis, and treatment. International Society for Peritoneal Dialysis Ad Hoc Committee on Ultrafiltration Management in Peritoneal Dialysis. Perit Dial Int 2000;20 Suppl 4:43-55.  Back to cited text no. 10
11.Vlijm A, Stoker J, Bipat S, et al. Computed Tomographıc findings characteristic for encap-sulating peritoneal sclerosis: A case-control study. Perit Dial Int 2009;29:517-22.  Back to cited text no. 11
12.Brown EA. Computed Tomographic scanning and diagnosis of encapsulating peritoneal sclerosis. Perit Dial Int 2009;29:502-4.  Back to cited text no. 12
13.Lo WK, Kawanishi H. Encapsulating perito­neal sclerosis-medical and surgical treatment. Perit Dial Int 2009;29 Suppl 2:S211-4.  Back to cited text no. 13
14.Evrenkaya TR, Atasoyu EM, Unver S, Basekim C, Baloglu H, Tulbek MY. Cortico­steroid and tamoxifen therapy in sclerosing encapsulating peritonitis in a patient on continuous ambulatory peritoneal dialysis. Nephrol Dial Transplant 2004;19:2423-4.  Back to cited text no. 14
15.Guest S. Tamoxifen therapy for encapsulating peritoneal sclerosis: Mechanism of action and update on clinical experience. Perit Dial Int 2009;29:252-5.  Back to cited text no. 15
16.Allaria PM, Giangrande A, Gandini E, Pisoni IB. Continuous ambulatory peritoneal dialysis and sclerosing encapsulating peritonitis: Tamo­xifen as a new therapeutic agent? J Nephrol 1999;12:395-7.  Back to cited text no. 16
17.Eltoum MA, Wright S, Atchley J, Mason JC. Four consecutive cases of peritoneal dialysis-related encapsulating peritoneal sclerosis treated successfully with Tamoxifen. Perit Dial Int 2006;26:203-6.  Back to cited text no. 17
18.Wong CF. Clinical experience with tamoxifen in encapsulating peritoneal sclerosis. Perit Dial Int 2006;26:183-4.  Back to cited text no. 18
19.Kawanishi H, Watanabe H, Moriishi M, Tsuchiya S. Successful surgical management of encapsulating peritoneal sclerosis. Perit Dial Int 2005;25 Suppl 4:39-47.  Back to cited text no. 19

Correspondence Address:
Hasan Kucuk
Surgery Clinic, Dr. Lutfi Kırdar Kartal Trainig Hospital, Kartal, Istanbul
Zerrin Bicik Bahcebasi
Nephrology Clinic, Dr. Lutfi Kırdar Kartal Trainig Hospital, Kartal, Istanbul
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Source of Support: None, Conflict of Interest: None

DOI: 10.4103/1319-2442.128607

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  [Figure 1], [Figure 2], [Figure 3]


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