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Saudi Journal of Kidney Diseases and Transplantation
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Table of Contents   
CASE REPORT  
Year : 2013  |  Volume : 24  |  Issue : 6  |  Page : 1203-1206
Encapsulating peritoneal sclerosis


1 Nephrology Department of Teaching Hospital, Aristide LE DANTEC, Dakar, Senegal
2 Gastroenterology Department of Teaching Hospital, Aristide LE DANTEC, Dakar, Senegal
3 Surgery Department of Teaching Hospital, Aristide LE DANTEC, Dakar, Senegal

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Date of Web Publication13-Nov-2013
 

   Abstract 

Encapsulating peritoneal sclerosis (EPS) is a rare but potentially lethal complication of peritoneal dialysis (PD). Peritoneal tuberculosis is considered an etiologic factor. We report a case of EPS in a 40-year-old man who was switched to hemodialysis because of peritoneal tuberculosis after 2 years of PD. Because of the persistence of gastrointestinal symptoms and cachexia, laparoscopic exploration was performed, which revealed an important thickening of the peritoneal membrane sheathing the intestinal loops. Accordingly, a diagnosis of EPS was made. Anti-tuberculosis treatment associated with a low dose of corticosteroids stabilized the disease.

How to cite this article:
Cisse M M, Dia D, Seck S M, Cisse M, Ka E F, Gueye S, Tall A O, Niang A, Diouf B. Encapsulating peritoneal sclerosis. Saudi J Kidney Dis Transpl 2013;24:1203-6

How to cite this URL:
Cisse M M, Dia D, Seck S M, Cisse M, Ka E F, Gueye S, Tall A O, Niang A, Diouf B. Encapsulating peritoneal sclerosis. Saudi J Kidney Dis Transpl [serial online] 2013 [cited 2019 Oct 20];24:1203-6. Available from: http://www.sjkdt.org/text.asp?2013/24/6/1203/121291

   Introduction Top


Encapsulating peritoneal sclerosis (EPS) is a diffuse peritoneal sclerosis of the visceral and parietal peritoneum. EPS is a potentially lethal complication of peritoneal dialysis (PD). [1] Sclerosis usually extends into the peritoneal cavity, resulting in an encasement of the small bowel that limits normal motility and, at the end, forms a "cocoon." EPS typically occurs in a patient on long-standing PD with demonstrated ultrafiltration failure and high peritoneal membrane transport status. It has been described in patients on both continuous and intermittent PD regimens, and many factors have been incriminated in its pathogenesis, especially bio-incompatible PD fluids and peritoneal infections such as tuberculosis. [2],[3],[4] The clinical presentation usually combines features of acute or sub-acute intestinal obstruction with an inflammatory syndrome and severe weight loss. Imaging techniques, particularly computerized tomography (CT) scanning, can help in the diagnosis of this disease, but are insufficiently sensitive. The diagnosis of EPS is confirmed by pathology. [2] The treatment principles included cessation of PD and switch to hemodialysis (HD), digestive tract rest and appropriate nutritional support. [3] Low-dose corticosteroid therapy or Tamoxifen can be added, but surgery is often necessary to release the intestinal loops. [2]

We report one case of EPS after 2 years of PD and a transfer to HD because of peritoneal tuberculosis.


   Case Report Top


A 40-year-old man was admitted for uncontrollable vomiting, loss of appetite, abdominal pain, ascites and severe weight loss. He was followed-up for 2 years at an outpatient PD clinic as an end-stage renal disease patient secondary to nephron-angiosclerosis. He was started on continuous ambulatory PD in April 2008 after insertion of a double-cuff catheter and glucose 1.36% and 2.27% solutions were used. In February 2009, he was switched to automated PD after loss of residual renal function and hyperpermeable peritoneum on the peritoneal equilibration test.

In December 2009, the patient presented with abdominal pain, vomiting, cloudy fluid and presence of Mycobacterium tuberculosis in the peritoneal fluid, confirming peritoneal tuberculosis. The treatment consisted of rifampicin, isoniazid, ethambutol and pyrazinamide adapted to renal function. The patient was switched from PD to HD.

Two months later, the patient presented with severe weight loss, abdominal pain, ascites and uncontrollable vomiting. Persistent peritoneal tuberculosis or EPS was suspected. Blood investigations revealed white blood cells 8900/mm 3 , neutrophiles 72.6%, lymphocytes 12.9%, hemoglobin 10.2 g/dL, platelet count 314,000/mm 3 , C-reactive protein (CRP) 165 mg/L, total protein 58 g/L, K+ 4 mmol/L, Na+ 135 mmol/L and VIH 1 and 2 negative. Peritoneal fluid investigations revealed red blood cells 10,000/mm 3 , white blood cells 150/mm 3 with 50% of neutrophils and 35% of lymphocytes.

The abdominal and chest X-ray were normal. CT scanning was not performed. Upper digestive endoscopy showed severe peptide esophagitis grade D. Coelioscopic exploration followed by laparotomy disclosed important cloudy fluid ascites with an important peritoneal thickening [Figure 1] and a voluminous cocoon sheathing the intestinal loops [Figure 2].
Figure 1: Cloudy fluid ascitis.

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Figure 2: Encapsulating peritoneal sclerosis with cocoon.

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Pathology showed acute inflammatory infiltrates, fibrous and thick wall without granuloma or Koch bacillus.

The diagnosis of EPS was confirmed and the patient received oral corticosteroids (prednisone 0.5 mg/kg/day) in combination with the ongoing tuberculosis treatment (bitherapy with rifampicin and isoniazid after 2 months of quadritherapy). Five months later, the clinical and biological evolution was favorable, with cessation of vomiting and normalization of CRP.


   Discussion Top


A PD program was launched in Senegal in March 2004 as an affordable alternative to HD and, since then, 60 patients have been treated.

However, this renal replacement therapy is associated with many complications of which EPS is the most severe. [1] The prevalence of EPS varies between 0.6 and 10%, and is significantly associated with PD duration, particularly more than 5 years; [5],[6] our patient developed EPS after only 2 years of PD. However, Mounia et al described an onset of EPS after 7 months of PD in a 57-year-old patient. [3]

The occurrence of uncontrollable vomiting with important weight loss suggested peritoneal tuberculosis after 2 months of well-tolerated anti-tuberculosis treatment, but the absence of peritoneal granulations and acid fast bacilli in the bacteriological and histopathological exams excluded this diagnosis.

In a patient with a past history of PD, the occurrence of vomiting or features of acute or sub-acute intestinal obstruction associated with a non-specific inflammatory syndrome and severe weight loss is highly suggestive of EPS. [3] In our case, abdominal X-ray was not contributive (absence of gas - fluid levels and calcifications) and CT scanning was not performed. EPS was confirmed by histopathology after laparotomy, which is still the best diagnostic method to confirm EPS by showing peritoneal thickening that sheaths the intestinal loops to form a cocoon. [2],[4] Histopathology examination found a paucity of cellular inflammatory infiltrates and absence of tuberculosis granulomas.

EPS is considered an abnormal reaction of the peritoneum to prolonged exposure to foreign bodies. [2] There are a number of factors, PD-related and non PD-related, that predispose to the development of EPS. Factors related to PD included duration of PD ≥5 years, use of hypertonic solutions and peritoneal infection (mainly Mycobacterium tuberculosis, Staphylococcus sp. and Streptococcus sp.). Factors not related to PD include the use of beta-blockers (Prac-tolol), ovarian or gastric tumors, autoimmune diseases, hepatic cirrhosis [3],[7] and eosinophilic peritonitis. [8] In our case, two etiologic factors were identified: PD history and peritoneal tuberculosis. These two factors may probably have a synergistic action and explain the early onset of EPS.

The treatment of EPS includes PD arrest, digestive tract rest with parenteral nutrition and low-dose corticosteroid therapy. [3] Immunosuppressive therapy with agents such as azathioprin, cyclosporine [6] or tamoxifen [9] has also been proposed. Surgery treatment is difficult and is associated with a high risk of colic perforation and sepsis. [6] Parenteral nutrition was not available for our patient (very high cost). However, corticosteroid therapy and switch to HD permitted survival at 5 months.

We conclude that EPS is a rare but potentially lethal complication of PD. Many factors are involved in its pathogenesis, including duration of PD and peritoneal tuberculosis. These two factors were found in our case, which probably explains the early onset of EPS in our patient. [12]

 
   References Top

1.Gupta SK, Venkataseshan VS, Churg J. Mesangiocapillary glomerulonephritis in Down's syndrome. Am J Nephrol 1991;11:112-7.  Back to cited text no. 1
    
2.Ozkaya O, Paksu MS, Bek K, et al. Renal amyloidosis due to pulmonary tuberculosis in a patient with Down syndrome. Eur J Pediatr 2006;165:134-5.  Back to cited text no. 2
    
3.Takemura T, Yoshioka K, Akano N, et al. Immunotactoid glomerulopathy in a child with Down syndrome. Pediatr Nephrol 1993;7:86-8.  Back to cited text no. 3
    
4.Robson WL, Leung AK, Woodman RC, Trevenen CL. Anti-neutrophil cytoplasmic antibody associated glomerulonephritis in a patient with Down's syndrome. Pediatr Nephrol 1995;9:204-5.  Back to cited text no. 4
    
5.Haseyama T, Imai H, Komatsuda A, et al. Proteinase-3- antineutrophil cytoplasmic antibody (PR3-ANCA) positive crescentic glomerulonephritis in a patient with Down's syndrome and infectious endocarditis. Nephrol Dial Transplant 1998;13:2142-6.  Back to cited text no. 5
    
6.Schwab M, Boswald M, Ludwig K, Wittekind C, Waldherr R, Ruder H. A patient with Down's syndrome and anti-neutrophilic cytoplasmic antibody-positive vasculitis. Pediatr Nephrol 1996;10:249-51.  Back to cited text no. 6
    
7.Baqi N, Tejani A, Sullivan EK. Renal transplantation in Down's syndrome: A report of the North American Pediatric Renal Transplant Cooperative Study. Pediatr Transplant 1998;2: 211-5.  Back to cited text no. 7
    
8.Birk PE, Burke BA, Vernier RL. Glomerulonephritis in children with Down syndrome. Pediatr Nephrol 1996;10:549.  Back to cited text no. 8
    
9.Lo A, Brown HG, Fivush BA, Neu AM, Racusen LC. Renal disease in Down's Syndrome: Autopsy study with emphasis on glomerular lesions. Am J Kidney Dis 1998;31:329-35.  Back to cited text no. 9
    
10.Glasson EJ, Sullivan SG, Hussain R, et al. Change in the survival profile of people with Down syndrome. Implications for genetic counseling. Journal of Trisomy 2004;11.  Back to cited text no. 10
    
11.Yoshikawa N, Ijima K, Shimomura M, Nakamura H, Ito H. IgG-associated primary glomerulonephritis in children. Clin Nephrol 1994;42: 281-7.  Back to cited text no. 11
    
12.Assadi FK. IgG-associated mesangial glomerulonephritis in a patient with Down syndrome. Med Sci Monit 2004;10:CS54-6.  Back to cited text no. 12
    

Top
Correspondence Address:
M M Cisse
Assistant, Nephrology Department of Teaching Hospital, Aristide LE DANTEC, Dakar
Senegal
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DOI: 10.4103/1319-2442.121291

PMID: 24231485

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