Primary sclerosing cholangitis (PSC) is a rare progressive cholestatic liver disease with presumed autoimmune etiology. It is characterized by inflammation and fibrosis of the intrahepatic and extrahepatic bile ducts. There is a strong association between PSC and inflammatory bowel diseases (IBDs), particularly ulcerative colitis(UC). The history of inflammatory bowel disease (IBD) after liver transplantation (LT) for PSC has been reported as variable. Even though in majority of the cases, IBD symptoms do not change or improve, there also cases of deterioriation reported. Approximately one third of patients with PSC may develop de nova IBD 10 years after LT. Several studies have focused on the course of IBD after LT. These studies found conflicting results. There is no data of our country in this subject. Our aim is to describe the natural history of IBD and PSC after liver transplantion in our patients.Materials and Methods
There were six patients transplanted for PSC who survived more than 12 months. Ulcerative colitis was diagnosed in four of the six(67 %) patients before transplantation. Colonoscopy and biopsy were performed before and after transplantation for all patients. Patients were followed for an average 56 (24-96) months. All received tacrolimus and prednisone with or without azathioprine as maintenance immunosuppression.Results
A total of 6 PSC patients were included in this study, 3 males and 3 females. The mean age at LT was 33.6 years (range, 18-43 y). Four of 6 patients with PSC had UC before liver transplantation. After LT, 2 patients (50%) had quiescent disease and were receiving no additional medications other than standard immunosuppression. Two patients (50%) had severe flares which could not be controlled by oral and IV prednisone orazathioprine and undergone total colectomy. Multifocal dysplasia was detected in colectomy specimens in 1 of the 2 cases. De novo IBD was diagnosed in 2 patients. PSC recurred after an average of 8 years in 5 of 6 patients. Two of those patients developed de novo UC at the same time. Four of the patients with recurrent PSC had active UC.Conclusions
Preexisting ulcerative colitis often has an aggressive course, while de novo ulcerative colitis may develop in patients transplanted for primary sclerosing cholangitis. An increased risk of colorectal cancer is present also after LT in IBD patients with primary sclerosing cholangitis. Regarding the higher clinical complexity of this subgroup of IBD patients, the management of IBD after LT requires close coordination between transplant surgeon, hepatologists and IBD experts.