Sclerosing Encapsulating Peritonitis in an Anti-HCV-Positive Patient on Chronic Ambulatory Peritoneal Dialysis

Sclerosing encapsulating peritonitis (SEP) is a clinical syndrome associated with ileus symptoms and irreversible sclerosis of the peritoneal membrane. The diagnosis of the disease is usually made at laparotomy, but findings on CT scans and ultrasounds of the peritoneum have been used to support diagnoses.[1–3]

We report an anti-HCV-positive case of SEP, presenting with severe abdominal symptoms, malnutrition ascites, and blood-stained dialysate. A 33-year-old man developed end-stage renal disease secondary to unknown origin ten years ago. He had been HCV-positive for ten years and was taking pegile interferon for one to two years. He had been maintained on continuous ambulatory peritoneal dialysis (CAPD) for 10 years. In the months before admission, peritoneal dialysis was becoming more difficult because of a decline in ultrafiltration. Three months previously, the PD catheter was removed and hemodialysis therapy was commenced. He was hospitalized with a 48 h history of vomiting and abdominal pain. On examination, he had a fever of 39.1˚C, dehydration, abdominal tenderness, distention, and rebound. Despite vancomycin and ceftazidime, he remained ill. The patient was suffering distention and spontanea fistulization in his abdomen, and a peritoneal dialysis catheter was replaced to improve drainage. The catheter was removed after three months. Computed tomography of the abdomen showed a markedly distended stomach and proximal small bowel loops clustered in the right side of the abdomen, thickening of the bowel wall, and extensive, irregular calcification of the parietal peritoneal membrane. Laparotomy confirmed the diagnosis of SEP.

The entire mass of bowel loops was separated in two distinct compartments by a sclerotic, tanned, and wrinkled covering. Oral prednisolone at a dose of 1 mg/kg daily, azathioprine 100 mg/gün, and tamoxifen 10 mg/day were commenced. Within 72 h, his temperature became normal; CRP fell from 210 mg/L to 40 mg/L within one week; and his anorexia had resolved. Prednisolone was reduced to 0.3 mg/kg daily. After 12 days, he was discharged with complete resolution of symptoms. Prednisolone therapy was slowly tapered and discontinued after 12 months. Azathioprine and tamoxifen therapy discontinued after 12 months. Sixteen months later, he remains well on regular hemodialysis therapy with AST: 25 mg/dL, ALT: 32 mg/dL, CRP: 11 mg/L, and albumin: 3.5 mg/dL.

There is no agreement on whether the treatment of choice is surgical, or conservative therapy. These include the use of immunosuppression, corticosteroids with parenteral nutrition, tamoxifen, and surgical debridement. In the presence of intestinal obstruction and potential bowel necrosis, surgical intervention is warranted. Tamoxifen, a nonsteroidal anti-oestrogen drug, has been successfully used in the treatment of fibrosclerotic disorders. Transforming growth factor b1 (TGF-b1) has a stimulatory effect on metalloproteinases-2 and -9 (MMP2 and MMP9). Because MMP9 degrades type IV and denaturized collagens, TGF-b1, the production of which is stimulated by tamoxifen, might favor mesothelial healing by facilitating the removal of denaturated collagen.[4],[5] Prednisolone, azathioprine, and tamoxifen combination did not have any negative effect on liver function and HCV status. This is the first SEP in an anti-HCV-positive patient on CAPD in western literature. In conclusion, SEP is no more a fatal complication if peritoneal dialysis treatment is interrupted promptly and immunosuppressive agents are administered.