Recurrent sclerosing cholangitis or ischemic bile duct lesions—A diagnostic challenge?

Abstract

A47-year-old male diagnosed with ulcerative colitis in 1985 and primary sclerosing cholangitis in 1990 was referred for orthotopic liver transplantation in 2001. He was accepted and underwent transplantation in June 2001. He experienced one acute, steroid-sensitive rejection during the first month. A hepatic artery stenosis (Fig. 1) diagnosed 30 days following transplantation was treated twice with percutaneous transluminal angioplasty and subsequently with insertion of a stent due to restenosis. The arterial blood supply was normal thereafter. A magnetic resonance cholangiography performed in December 2001 showed no strictures in the bile tree. During the next 6 to 8 months the patient had repetitive episodes with fever, increased bilirubin, pale stools, and dark urine. His symptoms responded to antibiotic treatment. During this period he had fluctuating aminotransferases, which responded to increased orally administered corticosteroids. Since August 2002, magnetic resonance cholangiography has shown multiple biliary strictures (Fig. 2). In March 2004 a liver biopsy (Fig. 3) was highly suggestive of recurrent disease. The patient suffers from recurrent, bacterial cholangitis and is currently being evaluated for retransplantation. Angiography of hepatic artery demonstrating stenosis (arrow). September 2001, 1 month following transplantation. Magnetic resonance cholangiography demonstrating multiple, intrahepatic strictures. January 2004, 2 years and 7 months following transplantation. Liver biopsy demonstrating inflammation, portal enlargement and periductal, concentric fibrosis. March 2004, 2 years and 9 months following transplantation. Both his magnetic resonance cholangiography and biopsy are strongly suggestive of recurrent sclerosing cholangitis, but according to the Mayo clinic criteria1 the arterial problem excludes him from being classified as such. This emphasizes the problem of diagnosing recurrent sclerosing cholangitis, and we believe it contributes to an underestimation of the true risk of disease recurrence. The distinction between recurrent disease and ischemic bile duct lesions is of importance when evaluating the patient for a retransplantation and possibly also for the selection of immunosupression following retransplantation.