Recurrent primary sclerosing cholangitis after orthotopic liver transplantation - Is chronic rejection part of the disease process?

Background. The possibility of primary sclerosing cholangitis (PSC) recurre nce after liver transplantation has been debated. The aim of this study is to examine whether recurrent PSC and chronic rejection (CR) are different e xpressions of the same disease process, Methods. One hundred consecutive patients receiving 118 grafts for the diag nosis of PSC were! reviewed and placed into three groups: group A, recurren t disease, as evidenced by cholangiographic and pathologic findings with ra diographic arterial flow to the liver (n=18; 15.7%); group B, those who dev eloped CR (n=15; 13.0%); and group C, all others (n=82;17.3%). Cholangiogra ms and histopathologic specimens were examined in a blinded fashion, Results. Demographic factors were similar, except for age, with a significa ntly younger age and more episodes of rejection in groups A and B (P<0.03), Group A had a higher incidence of cytomegalovirus hepatitis (P=0.008), Fiv e-year graft survivals for A, HE, and C were 64.6%, 33.3%, and 76.1%, respe ctively (P=0.0001), 5-year patient survivals were 76.2%, 66.7%, and 89.1%, respectively (P=0.0001), and repeat transplantation rates were 27.8%, 46.7% , and 8.5%, respectively (P=0.005), Radiographically, 90% of cholangiograms in patients with recurrent disease showed at least multiple intrahepatic s trictures. Histopathologically, patients with recurrent disease and CR shar ed many features. Conclusions. We have described a high incidence of recurrent PSC and CR in patients who received transplants for PSC. Histopathologic analysis suggest s that CR and recurrent PSC could represent a spectrum of indistinguishable disease. However, the distinct difference in clinical outcome, as evidence d by an increased repeat transplantation rate and lower graft and patient s urvival in the CR group, clearly suggests that they are two distinct entiti es that require very different treatment strategies.