Dr. Jeyarajah et al., Recurrent primary sclerosing cholangitis after orthotopic liver transplantation - Is chronic rejection part of the disease process?, TRANSPLANT, 66(10), 1998, pp. 1300-1306
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.